morphine and Intestinal-Obstruction

Cystic fibrosis is an autosomal-recessive defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene located on chromosome 7 that affects 1 in 2500 live White births. Defects in the gene lead to abnormally thick secretions causing chronic obstruction in the respiratory and gastrointestinal tracts. Common gastrointestinal pathology in children with cystic fibrosis includes meconium ileus in infancy and distal intestinal obstruction syndrome in childhood and exocrine pancreatic insufficiency, constipation, and rectal prolapse. This article describes the presentation, diagnosis, and management of these conditions in patients with cystic fibrosis, from birth to adulthood.

Topics: Adult; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Meconium; Meconium Ileus

This report summarizes the clinical characteristics of intractable anemia as part of the clinical presentation of Hirschsprung's disease (HD) and aims to strengthen clinicians' ability to recognize early signs of HD.. An 11-year-old boy with a 6-year history of intractable anemia, low hemoglobin level (55 g/L), poor response to oral iron supplementation and blood transfusion, and difficulty with defecation was diagnosed with HD. A 19-month-old boy with a 3-month history of intractable anemia, low hemoglobin level (64 g/L), poor response to oral iron supplementation and blood transfusion, delayed meconium passage, and history of intestinal obstruction was also diagnosed with HD. Both patients underwent surgery, after which anemia was corrected effectively in both cases. Two more cases of intractable anemia as the chief complaint and diagnoses of HD over different durations since the onset of anemia (ranging from 1.7 years to 21 years) were identified in a literature search. Both patients underwent surgery, after which anemia was corrected.. Intractable anemia as part of the clinical presentation of HD is extremely rare. Detailed inquiries of medical histories and physical examinations are key to early diagnoses and preventing misdiagnoses. Anemia in HD patients may primarily be caused by impaired iron absorption due to HD.

Topics: Child; Hirschsprung Disease; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Physical Examination

The purpose of this study was to critically appraise and compare the diagnostic performance of imaging modalities that are used for the diagnosis of upper and lower gastrointestinal (GI) tract obstruction in neonates and infants.. A focused clinical question was constructed and the literature was searched using the patient, intervention, comparison, outcome method comparing radiography, upper GI contrast study, and ultrasound in the detection of upper GI tract obstruction such as duodenal atresia and stenosis, jejunal and ileal atresia, and malrotation and volvulus. The same methods were used to compare radiography and contrast enema in the detection of lower GI tract obstruction such as meconium plug syndrome, meconium ileus, Hirschsprung disease, and imperforate anus. Retrieved articles were appraised and assigned a level of evidence based on the Oxford University Centre for Evidence-Based Medicine hierarchy of validity for diagnostic studies.. There were no sensitivities/specificities available for the imaging diagnosis of duodenal atresia or stenosis, jejunal or ileal atresias, meconium plug, and meconium ileus or for the use of cross-table lateral radiography for the diagnosis of rectal pouch distance from skin in imperforate anus. The retrieved sensitivity for the detection of malrotation on upper GI contrast study is 96%, and the sensitivity for the diagnosis of midgut volvulus on upper GI contrast study is 79%. The retrieved sensitivity and specificity for the detection of malrotation with volvulus on ultrasound were 89% and 92%, respectively. The retrieved sensitivity and specificity for the detection of Hirschsprung disease on contrast enema were 70% and 83%, respectively. The retrieved sensitivity of invertogram for the diagnosis of rectal pouch distance from skin in imperforate anus is 27%. The retrieved sensitivities of perineal ultrasound and colostography for the diagnosis of rectal pouch distance from skin in imperforate anus were 86% and 100%, respectively.. There is limited evidence for the imaging diagnosis of duodenal atresia and stenosis, jejunal and ileal atresias, meconium plug, meconium ileus, and imperforate anus, with recommended practice based mainly on low-quality evidence or expert opinion. The available evidence supports the use of upper GI contrast study for the diagnosis of malrotation and volvulus, with ultrasound as an adjunct to diagnosis. Contrast enema is useful in the investigation of suspected Hirschsprung disease, but a negative study does not outrule the condition. Colostography is the investigation of choice for the work-up of infants with complex anorectal malformations before definitive surgical repair.

Topics: Comparative Effectiveness Research; Contrast Media; Duodenal Obstruction; Humans; Ileus; Infant; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Intestinal Volvulus; Meconium; Radiography, Abdominal; Sensitivity and Specificity; Ultrasonography

The article reviews advances in gastrointestinal aspects of cystic fibrosis (CF) published in the literature over the past year, and highlights new and interesting research.. Animal models can be used to understand the pathophysiology of gastrointestinal complications in CF. The CF mouse is useful for studying distal intestinal obstruction, dysmotility and dysbiosis, and the CF pig model has helped us better understand meconium ileus and pancreatic and hepatobiliary secretory problems. Studies in humans help elucidate the evolution of pancreatic insufficiency, how reflux may lead to lung disease, problems with intestinal dysmotility, mechanisms leading to pancreatitis and the increased prevalence of gastrointestinal cancer. Biomarkers are shedding light on CF-related liver disease. Rectal biopsies can help in diagnosis and in studying new drugs for CF.. Gastrointestinal complications of CF are likely to be seen with increasing frequency as patients with CF lead longer lives. CF animal models and modern research techniques are providing new insights into extrapulmonary complications. CF clinicians should be familiar with diagnosis and management of common gastrointestinal complications and should build bridges with specialists so that referrals can be made when needed.

Topics: Animals; Cystic Fibrosis; Digestive System Diseases; Disease Models, Animal; Dysbiosis; Gastrointestinal Diseases; Humans; Ileus; Intestinal Obstruction; Liver Diseases; Meconium; Mice; Pancreatic Diseases; Prognosis; Rectum; Swine

Meconium obstruction of prematurity is an entity primarily affecting very low birthweight or extremely low birthweight babies causing low intestinal obstruction. Its presence may at best delay establishment of enteral feeding and compromise nutrition and at worst lead to mechanical obstruction requiring surgery or to intestinal perforation. There are considerable challenges in the recognition, diagnosis and management of this condition. Awareness of the disease and understanding of its pathogenesis may lead to early detection of affected babies and allow proactive measures to decrease the associated morbidity and mortality.

Topics: Early Diagnosis; Humans; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Infant, Very Low Birth Weight; Intestinal Obstruction; Meconium

Meconium ileus at birth, distal intestinal obstruction syndrome (DIOS), and constipation are an interrelated group of intestinal obstruction syndromes with a variable severity of obstruction that occurs in cystic fibrosis patients. Long-term follow-up studies show that today meconium ileus is not a risk factor for impaired nutritional status, pulmonary function, or survival. DIOS and constipation are frequently seen in cystic fibrosis patients, especially later in life; genetic, dietary, and other associations have been explored. Diagnosis of DIOS is based on suggestive symptoms, with a right lower quadrant mass confirmed on abdominal radiography, whereas symptoms of constipation are milder and of longer standing. In DIOS, early aggressive laxative treatment with oral laxatives (polyethylene glycol) or intestinal lavage with balanced osmotic electrolyte solution and rehydration is required, which now makes the need for surgical interventions rare. Constipation can generally be well controlled with polyethylene glycol maintenance treatment.

Topics: Constipation; Cystic Fibrosis; Humans; Ileus; Intestinal Obstruction; Meconium; Risk Factors

Topics: Child; Cystic Fibrosis; Digestive System Surgical Procedures; Humans; Intestinal Obstruction; Meconium

Cystic fibrosis (CF) is an inherited disorder that presents as a multisystem disease with meconium ileus being the presenting symptom in 20% of patients. Approximately half of these patients present with complicated meconium ileus mandating early surgical intervention, potentially resulting in short gut syndrome. Although liver transplantation in children with CF has been described, this is the first report of a combined liver and small bowel transplant in a recipient with CF. A 7-month-old boy with CF presented with short bowel syndrome following extensive small bowel resection for meconium ileus and progressive cholestatic liver failure from intravenous hyperalimentation. He underwent combined liver and small intestinal transplant. He was discharged home three weeks post-transplant on enteral feeds with supplemental intravenous fluid. He has had routine protocol small bowel allograft biopsies with no documented rejection episodes. He has been treated for minor respiratory infections without major sequelae. Improvements in pulmonary therapy have impacted on the survival in the CF population to the point where the need for multiorgan transplantation will be increased in the future. Extrapolating from the excellent experience of liver transplantation in children with CF, early liver and small intestinal multivisceral transplantation, if indicated, can be performed safely in children with CF.

Topics: Cystic Fibrosis; Humans; Infant; Intestinal Obstruction; Intestine, Small; Liver Failure; Liver Transplantation; Male; Meconium; Short Bowel Syndrome

We report a case of a 5-day-old infant male who presented with abdominal distention and inability to pass meconium. Barium enema revealed microcolon, and at surgery the etiology was found to be multiple proximal ileal atresia.

Topics: Barium Sulfate; Colon; Diagnosis, Differential; Enema; Humans; Ileal Diseases; Ileum; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Radiography

More than 1,000 mutations have been identified in the cystic fibrosis (CF) transmembrane regulator (CFTR) disease gene. The impact of these mutations on the protein and the wide spectrum of CF phenotypes prompted a series of Genotype-Phenotype correlation studies. The CFTR genotype is invariably correlated with pancreatic status-in about 85% of cases with pancreatic insufficiency and in about 15% of cases with pancreatic sufficiency. The correlations between the CFTR genotype and pulmonary, liver, and gastrointestinal expression are debatable. The heterogeneous phenotype in CF patients bearing the same genotype or homozygotes for nonsense mutations implicated environmental and/or genetic factors in the disease. However, the discordant phenotype observed in CF siblings argued against a major role of environmental factors and suggested that genes other than CFTR modulate the CF phenotype. A locus that modulates gastrointestinal expression was identified in mice and subsequently in humans. By analyzing nine CF patients discordant for meconium ileus we were able to show that this locus had a dominant effect. Moreover, in a collaborative study we found a higher rate of polymorphisms in beta-defensin genes 1 and 2 in CF patients and in controls. In another multicenter study mutations in alpha-1 antitrypsin (A1AT) and mannose binding lectin genes were found to be independent risk factors for liver disease in CF patients. The body of evidence available suggests that the variegated CF phenotype results from complex interactions between numerous gene products.

Topics: Animals; Codon, Nonsense; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epistasis, Genetic; Gene Expression Regulation; Genetic Heterogeneity; Genotype; Humans; Intestinal Obstruction; Liver; Lung; Meconium; Mice; Mice, Inbred CFTR; Multifactorial Inheritance; Mutation; Organ Specificity; Pancreas; Phenotype; Polymorphism, Genetic

Retained meconium resulting in intestinal obstruction in infants with very low birth weight (VLBW) may cause significant morbidity and even mortality. Immature intestinal neuromuscular function, abnormal meconium composition, and maternal magnesium sulfate therapy results in delay of first meconium passage. This "normal" passage of first meconium may not occur until 1 week of life or later. A spectrum of conditions results from this delayed passage. At the most serious end of this continuum is intestinal obstruction. These patients present with abdominal distension, emesis, and reduced stool frequency. Early diagnosis of this condition using plain abdominal radiographs and contrast enemas where appropriate is imperative. Contrast enemas may be both diagnostic and therapeutic. Most patients do not need surgical intervention. Operation is indicated when enemas fail to relieve the obstruction or perforation occurs. Prompt diagnosis and appropriate management results in a good outcome. Meconium obstruction in VLBW infants does not appear to be associated with cystic fibrosis or Hirschsprung's disease.

Topics: Humans; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Infant, Very Low Birth Weight; Intestinal Obstruction; Intestinal Perforation; Meconium

Intestinal obstruction in the newborn infant may be due to a variety of conditions, including atresia and stenosis, annular pancreas, malrotation, duplication cyst, meconium ileus, meconium plug syndrome and neonatal small left colon syndrome, and Hirschsprung's disease. Neonates with unrecognised intestinal obstruction deteriorate rapidly, show an increase of associated morbidity and appropriate surgical treatment becomes more hazardous. Early diagnosis depends largely on the prompt detection of obstructive manifestations by the clinician and the subsequent accurate interpretation of radiographic findings by the radiologist. Plain film of the abdomen is often helpful in determining the level of obstruction and usually dictates, together with clinical symptoms, the choice of the contrast study firstly to perform. In this article we will review the clinical and radiological signs of different pathological conditions causing intestinal obstruction in the newborn.

Topics: Colon; Constriction, Pathologic; Contrast Media; Cysts; Hirschsprung Disease; Humans; Infant, Newborn; Intestinal Atresia; Intestinal Diseases; Intestinal Obstruction; Intestines; Meconium; Pancreas; Radiography; Syndrome; Torsion Abnormality

Timely passage of the first stool is a hallmark of the well-being of the newborn infant. Failure of a full-term newborn to pass meconium in the first 24 hours may signal intestinal obstruction. Lower intestinal obstruction may be associated with disorders such as Hirschsprung's disease, anorectal malformations, meconium plug syndrome, small left colon syndrome, hypoganglionosis, neuronal intestinal dysplasia and megacystis-microcolon-intestinal hypoperistalsis syndrome. Radiologic studies are usually required to make the diagnosis. In addition, specific tests such as pelvic magnetic resonance imaging, anorectal manometry and rectal biopsy are helpful in the evaluation of newborns with failure to pass meconium.

Topics: Anal Canal; Colonic Diseases; Congenital Abnormalities; Diagnosis, Differential; Hirschsprung Disease; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Rectum; Syndrome

Topics: Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Intestinal Obstruction; Meconium; Mice; Mutation; Pancreatic Diseases

Diseases of the small colon are numerous and they can affect horses of any age. Diseases can be simple obstructions, nonstrangulating obstructions, strangulating obstructions, and congenital defects. American Miniature Horses appear prone to luminal obstruction with impacted intestinal contents, and some ischemic diseases are more common in postpartum mares. Enterotomy and enterectomy have a high success rate in the small colon, provided the affected portion can be exteriorized. The beginning and terminal portions of the small colon can be involved in many diseases and are not readily accessible from standard abdominal approaches. The prognosis is generally more favorable than has been proposed.

Topics: Animals; Animals, Newborn; Bezoars; Colon; Colonic Diseases; Female; Horse Diseases; Horses; Infarction; Intestinal Atresia; Intestinal Obstruction; Meconium; Obstetric Labor Complications; Pregnancy; Prognosis; Rupture

Imaging plays a major role in most neonatal gastrointestinal emergencies. The role may vary from helping to establish a diagnosis, to the evaluation of associated abnormalities, to surgical planning, or to therapy for some conditions like meconium ileus or meconium plug syndrome. Plain radiographs and ultrasound serve a primary imaging modalities with bowel contrast examinations, CT scan, and MR imaging playing roles in more complex cases.

Topics: Abdomen, Acute; Diagnostic Imaging; Digestive System Abnormalities; Digestive System Surgical Procedures; Emergencies; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Meconium ileus represents a functional disorder mainly detected during the second trimester of gestation. The diagnosis can be recognized by bowel hyperechogenicity or by the pathologist after a systematic examination. It must lead to etiologic diagnosis and specific investigations according to the history. Even in absence of family history, cystic fibrosis must be considered with pancreatic study and with frozen fetal material in order to be able to search for the most common mutations. Meconium ileus can also be associated with infection, fetal blood swallowing, chromosomal aberrations and multiple fetal abnormalities. Only good investigations for specific diagnosis can lead to a genetic counsel and a good follow up of the next gestation.

Topics: Cystic Fibrosis; Digestive System Abnormalities; Humans; Intestinal Obstruction; Meconium

Many different pathophysiologic conditions can affect the neonatal intestinal tract and ultimately require surgical intervention. The symptomatology is primarily that of bowel dysfunction and obstruction. A thorough understanding of these disease processes is necessary of the managing physician. With this knowledge, a systematic course of resuscitation, evaluation, and treatment can be instituted, usually with predictably gratifying results for patient, family, and physician alike.

Topics: Duodenal Obstruction; Enterocolitis, Pseudomembranous; Fluid Therapy; Hirschsprung Disease; Humans; Ileum; Infant, Newborn; Intestinal Atresia; Intestinal Diseases; Intestinal Obstruction; Jejunum; Meconium; Resuscitation

We present a case of meconium peritonitis which was associated with a short bowel and complicated by progressive bowel distension and difficulty in making a definitive diagnosis of cystic fibrosis. Treatment was by bowel resection and an ileostomy (and later bowel anastomosis), followed by parenteral nutrition which was complicated by hepatitis. The literature is reviewed and management dilemmas and options are discussed.

Topics: Adult; Cystic Fibrosis; Female; Fetal Diseases; Humans; Ileostomy; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Karyotyping; Male; Meconium; Parenteral Nutrition; Peritonitis; Polyhydramnios; Pregnancy; Ultrasonography, Prenatal

Meconium ileus is a manifestation of intestinal and pancreatic dysfunction that results in the accumulation of a sticky and inspissated intraluminal meconium, which in most cases results from the autosomal recessive disease cystic fibrosis. Both nonoperative and operative therapies are effective in relieving this small-bowel obstruction; in the past, although less so today, a successful nonoperative treatment was associated with a more favorable outcome. Once the meconium ileus is relieved successfully, and the diagnosis of cystic fibrosis is established, the treatment for the intestinal manifestations of the disease focuses on enzyme replacement to augment patient nutritional status. Simultaneously, the treatment of the life-threatening pulmonary disease focuses on mucous retention and chronic infection in the lungs. Future therapies for patients with cystic fibrosis include lung transplantation, pharmacologic manipulation of the epithelial cell abnormality, and gene transfer therapy into the respiratory epithelium.

Topics: Cystic Fibrosis; Diagnosis, Differential; Humans; Infant, Newborn; Intestinal Obstruction; Meconium

A survey is given of the pathophysiology of the main alterations in the small intestine of cystic fibrosis patients. Special attention is paid to the understanding of meconium ileus in the fetus and the newborn, the repercussion of duodenal acidity on the duodenal mucosa and the intraduodenal digestion, and primary and secondary biochemical alterations in the secretory-digestive-absorptive function of the small-intestinal mucosa. The meconium equivalent syndrome and its connection with the atypical course of intussusception and appendiceal perforation with silent pelvic abscess are also discussed.

Topics: Cystic Fibrosis; Humans; Intestinal Obstruction; Intestine, Small; Meconium

Topics: Ductus Arteriosus, Patent; Humans; Ileal Diseases; Infant, Newborn; Infant, Premature; Intestinal Obstruction; Male; Meconium; Postoperative Complications; Radiography

Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Lung; Meconium; Patient Care Team; Radiography, Abdominal; Radiology; Technology, Radiologic

Topics: Child, Preschool; Cystic Fibrosis; Female; Genetic Carrier Screening; Genetic Counseling; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Minerals; Nutritional Physiological Phenomena; Pancreatic Extracts; Pregnancy; Respiratory Tract Diseases; Respiratory Tract Infections; Vitamins

It has long been recognized that the patient with chronic lung disease, malnutrition, vitamin K deficiency or electrolyte and blood gas disturbances presents a special problem for the surgeon. Patients with cystic fibrosis have all of these abnormalities. Until recently, the patient with cystic fibrosis was exclusively the domain of the pediatrician. Today, with the increasing number of patients over the age of 20 years, internists and surgeons have an increasingly important role in the care of these patients. In addition, since women with cystic fibrosis are capable of bearing children, the need for genetic, obstetric and gynecologic counseling is becoming more apparent. Although the patient with cystic fibrosis is generally at a much greater risk for the complications of surgical treatment than the normal patient, experience in the management of these patients has greatly improved the outlook. However, in most instances, surgical management of the patient with cystic fibrosis represents palliation, since these patients usually die of the medical complications of their disease. Further controlled studies are needed to fully evaluate the role of surgical treatment in the sequelas of cystic fibrosis.

Topics: Adolescent; Adult; Biliary Tract Diseases; Child; Child, Preschool; Cystic Fibrosis; Female; Hemoptysis; Humans; Hypertension, Portal; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Middle Aged; Mucocele; Nasal Polyps; Pneumothorax; Risk; Surgical Procedures, Operative

Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Lung Diseases; Mass Screening; Meconium; Prognosis

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Mass Screening; Meconium

Topics: Bile Acids and Salts; Blood Proteins; Cystic Fibrosis; Glycoproteins; Hemoglobins; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Steroids

Topics: Abdomen, Acute; Abdominal Muscles; Angiography; Biliary Tract; Bladder Exstrophy; Enterocolitis, Pseudomembranous; Gastroesophageal Reflux; Gastrointestinal Diseases; Hernia, Diaphragmatic; Hernia, Umbilical; Humans; Infant Nutritional Physiological Phenomena; Infant, Newborn; Infant, Newborn, Diseases; Intensive Care Units; Intestinal Atresia; Intestinal Obstruction; Meconium; Monitoring, Physiologic; Radionuclide Imaging; Ultrasonography

Topics: Abnormalities, Multiple; Colonic Diseases; Digestive System Abnormalities; Duodenal Obstruction; Esophageal Atresia; Female; Hernia; Humans; Ileum; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Intestines; Jejunum; Male; Meconium; Megacolon; Mesenteric Cyst; Polyhydramnios; Pregnancy; Pylorus; Radiography; Vitelline Duct

Topics: Adolescent; Adult; Appetite; Child; Child, Preschool; Cystic Fibrosis; Diabetes Complications; Female; Growth Disorders; Humans; Hypertension, Portal; Infant; Infant, Newborn; Intestinal Diseases; Intestinal Obstruction; Intussusception; Lactose Intolerance; Liver Cirrhosis; Male; Meconium; Pancreas; Pancreatitis; Rectal Prolapse

Topics: Child, Preschool; Colonic Diseases; Cystic Fibrosis; Female; Humans; Ileum; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestine, Small; Jejunum; Meconium; Methods

Topics: Abdomen; Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Fatty Liver; Feces; Humans; Infant; Intestinal Absorption; Intestinal Diseases; Intestinal Mucosa; Intestinal Obstruction; Intestines; Lactose Intolerance; Meconium; Metabolism, Inborn Errors; Pain; Pancreas; Prognosis; Rectal Prolapse

Topics: Adolescent; Adult; Autopsy; Child; Child, Preschool; Cystic Fibrosis; Diabetes Mellitus, Type 1; Female; Humans; Infant; Infant, Newborn; Infertility, Male; Intestinal Obstruction; Liver; Liver Cirrhosis, Biliary; Lung; Male; Meconium; Pancreas; Pregnancy; Sweat

Topics: Acetylcysteine; Adolescent; Autopsy; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Hydrogen Peroxide; Ileum; Infant; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Intestine, Small; Male; Meconium; Pancreatin; Peritonitis; Prognosis; Radiography, Abdominal; Therapeutic Irrigation; Vitamin K Deficiency

Topics: Birth Weight; Diagnosis, Differential; Humans; Infant Food; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Megacolon; Therapeutic Irrigation

Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Lung Diseases; Meconium; Mucoproteins; Pancreas; Prognosis; Sweat

Topics: Anal Canal; Congenital Abnormalities; Cystic Fibrosis; Duodenal Obstruction; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Meconium; Megacolon; Pancreas; Rectum

Meconium retention is associated with feeding intolerance. Trials using glycerol and Gastrografin to expedite the evacuation of meconium have failed to generate clinically valid results for efficacy and safety.. We assessed the feasibility of aggressive meconium evacuation with saline rectal washout (RW) in very-low-birth-weight infants to reduce the time it took them to reach full enteral feeds.. We conducted an open-label, pilot, randomized controlled trial (RCT) (birth weight stratified, i.e., to 750-999 g and 1,000-1,500 g) of early aggressive meconium evacuation with twice-daily normal saline RW compared to conventional management with glycerin suppositories (GS), until full enteral feeds (110 mL/kg/day) were reached. Primary outcome was time to reach full enteral feeds. Safety, process, and secondary efficacy outcomes were also evaluated.. Sixty-one infants were randomized, 28 to RW and 33 to GS. The process and feasibility outcomes were met. RW was found to be safe; none of the RW-randomized infants developed necrotizing enterocolitis (≥ stage II) or complications secondary to RW. Evidence of efficacy was supported: in the 750-999 g stratum (n = 15), the median time to full enteral feeds was shorter with RW (11.0 days, 95% CI: 10.4-11.6) than with GS (15.6 days, 95% CI: 13.0-18.2) by a reduction of 4.6 days (p = 0.027). In the 1,000-1,500 g stratum (n = 46), there was no evidence of benefit: RW 10.2 days (95% CI 8.3-12.1) and GS 10.1 days (95% CI 9.3-10.9, p = 0.304).. Our protocol was feasible and an adequately powered RCT is required to confirm the findings of this trial.

Topics: Adult; Birth Weight; Enema; Enteral Nutrition; Feasibility Studies; Female; Gestational Age; Glycerol; Humans; Infant, Newborn; Infant, Premature; Infant, Very Low Birth Weight; Intensive Care Units, Neonatal; Intestinal Obstruction; Male; Meconium; Pilot Projects; Singapore; Sodium Chloride; Suppositories; Therapeutic Irrigation; Time Factors; Treatment Outcome

We hypothesized that small volume enemas accelerate meconium evacuation in very low birth weight (VLBW) infants. In a randomized controlled trial, VLBW infants (n = 81) received either repeated daily small volume enemas if complete spontaneous meconium passage failed within 24 h or no intervention. Small volume enemas did not accelerate complete meconium evacuation, which occurred after 6.0 to 9.6 (95% CI) d in the intervention group and after 7.7 to 11.0 (95% CI) d in the control group. No adverse events were observed. Daily administration of small volume enemas had no effect on total meconium evacuation defined by the time of last meconium passage.

Topics: Defecation; Enema; Humans; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Infant, Very Low Birth Weight; Intestinal Obstruction; Meconium; Time Factors

Although newborn screening for cystic fibrosis (CF) is widely advocated, hard evidence in its favor is difficult to obtain, partly because of a dramatically improved life expectancy. Between 1985--1989 infants, born in Wales and the West Midlands were randomized to newborn CF screening by heel-prick immunoreactive trypsin (IRT) measurement or diagnosis by clinical presentation. Eligible children with CF who died in the first 5 years of life were identified from the local pediatricians and from the National UK CF Survey. In all, 230,076 infants were randomized to be screened, while 234,510 were unscreened. One hundred seventy-six CF children were identified, of whom 7 died in the first 5 years of life, 3 having presented with meconium ileus. Median age of diagnosis in the screened group was 8 weeks. On an intention to treat analysis, all 4 nonmeconium ileus-related deaths occurred in the unscreened group (Fisher's exact test, P < 0.05). However, the clinical presentation of 2 of these infants led to them being diagnosed prior to 8 weeks, i.e., earlier than would have been likely by screening. In conclusion, newborn screening has the potential to decrease infant CF deaths, but if it is to be successful, identification and treatment must occur as soon as possible after birth.

Topics: Cystic Fibrosis; False Negative Reactions; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Neonatal Screening; Risk Factors; Trypsin

Meconium ileus can be difficult to distinguish from ileal atresia on plain radiographs and on contrast enema. Both show a microcolon in the face of a small bowel obstruction. The treatment of the two is very different. Meconium ileus obstruction may be relieved medically by contrast enema; ileal atresia requires prompt surgical intervention. This study was made to determine if abdominal ultrasonography might be helpful in distinguishing between these two entities. Abdominal ultrasonograms from the past 10 years of all patients with these two diseases who were studied with preoperative ultrasonography at Arkansas Children's Hospital were reviewed. Six of 16 patients with meconium ileus had preoperative ultrasonograms. All six patients with meconium ileus had multiple loops of bowel filled with very echogenic thick meconium. Four of 22 patients with ileal atresia had preoperative ultrasonograms. These four patients with ileal atresia had dilated loops of bowel filled with fluid and air. None had a dilated bowel filled with thick echogenic contents. Preoperative abdominal ultrasonography is proposed as a simple method for distinguishing between these two disease entities with very different treatment plans.

Topics: Diagnosis, Differential; Humans; Ileal Diseases; Ileum; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Meconium; Ultrasonography

Phasing of heterozygous alleles is critical for interpretation of

Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Meconium Ileus; Trypsin; Trypsinogen

We present a case of an infant born to a mother with COVID-19, who at 24 hours of life was treated with a glycerin suppository for failure to pass meconium and went on to develop bilious emesis and abdominal distention as feeding continued over the next several hours. After a barium enema identified the distal obstruction, the pediatric surgical team used rectal irrigation to remove a large meconium plug, which mimicked the appearance of the descending colon on plain film, in a case of small left colon syndrome. Although intestinal obstruction in the newborn is rare, it is imperative that it is promptly diagnosed and treated appropriately to avoid negative outcomes; which, even in perhaps the mildest form of functional distal obstruction, meconium plug syndrome, can lead to an impressive clinical illness with risk of intestinal perforation and subsequent meconium peritonitis if the obstruction is not relieved.

Topics: Child; COVID-19; Cystic Fibrosis; Female; Fetal Diseases; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Vomiting

Topics: Cystic Fibrosis; Female; Fetal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Meconium

Magnesium sulphate (MgSO. An analytical retrospective study was conducted on <32 weeks preterm babies admitted to a tertiary-level hospital (January 2016-December 2017). Epidemiological, prenatal and postnatal data on the outcomes were obtained, analysed and compared in both groups (exposed to MgSO. This study found no correlations between MgSO

Topics: Female; Gestational Age; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Magnesium Sulfate; Meconium; Pregnancy; Retrospective Studies

Meconium-related ileus in very low birth weight infants can lead to increased morbidity or mortality and prolonged hospitalization without prompt diagnosis and treatment. This study primarily aimed to identify the incidence of and factors associated with meconium-related ileus and secondarily sought to investigate clinical and growth outcomes after water-soluble contrast media (Gastrografin) enema.. We retrospectively reviewed medical records of very low birth weight infants born between February 2009 and March 2019 in the neonatal intensive care unit of a single medical center. Perinatal factors, clinical outcomes, and growth outcomes were compared between the group with meconium-related ileus that received Gastrografin enema and the control group.. Twenty-four (6.9%) patients were diagnosed with meconium-related ileus among 347 very low birth weight infants. All achieved successful evacuation of meconium with an average of 2.8 (range: 1-8) Gastrografin enema attempts without procedure-related complications. Initiation of Gastrografin enema was performed at mean 7.0 days (range: 2-16) after birth. Incidences of moderate to severe bronchopulmonary dysplasia were higher and the duration of mechanical ventilation and need for oxygen were longer in the meconium-related ileus group (P = 0.039, 0.046, 0.048, respectively). Meconium-related ileus infants took more time to start enteral feeding and the nothing per oral time was longer (P = 0.001 and 0.018, respectively). However, time to achieve full enteral feeding and Z-scores for weight and height at 37 weeks and at 6 months corrected age did not differ between the two groups.. Gastrografin enema in very low birth weight infants with meconium-related ileus was an effective and safe medical management. Following Gastrografin enema, very low birth weight infants with meconium-related ileus achieved similar subsequent feeding progress and similar growth levels as the control groups without meconium-related ileus.

Topics: Diatrizoate Meglumine; Enema; Humans; Ileus; Infant, Newborn; Infant, Very Low Birth Weight; Intestinal Obstruction; Meconium; Retrospective Studies

Anastomotic or perianastomotic ulcers present with symptoms such as chronic anaemia and occult bleeding as long-term complications of bowel resection performed in infancy.. Herein, we describe a 15-year-old girl with a history of surgery for meconium obstruction without mucoviscidosis in infancy who was hospitalized with chief complaints of presyncope and convulsions. Seven hours after admission, she developed melena and went into shock. An emergency laparotomy was performed, and a Dieulafoy lesion was detected near the site of ileal anastomosis from the surgery that had been performed during infancy.. Although overt massive lower gastrointestinal bleeding necessitating emergency care is rare in the long term after infant bowel resection, Dieulafoy lesions can cause serious bleeding, requiring rapid life-saving haemostatic procedures.

Topics: Adolescent; Anastomosis, Surgical; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Ulcer

Topics: Humans; Ileus; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Intestine, Small; Meconium

This is a commentary on the manuscript titled "Early Management of Meconium Ileus in Infants with Cystic Fibrosis: A Prospective Population Cohort Study" by Long A-M, et al.

Topics: Cohort Studies; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Meconium Ileus; Prospective Studies

Contemporary early outcome data of meconium Ileus (MI) in cystic fibrosis (CF) are lacking on a population level. We describe these and explore factors associated with successful non-operative management.. A prospective population-cohort study using an established surveillance system (BAPS-CASS) was conducted October 2012-September 2014. Live-born infants with bowel-obstruction from inspissated meconium in the terminal ileum and CF were reported. Data are described as median (interquartile range, IQR).. 56 infants were identified. 14/56(25%) had primary laparotomy (13/23 complicated MI, 1/33 simple), the remainder underwent contrast enema. Twelve, (12/33 (36%) with simple MI) achieved decompression. 8/12 (67%) who decompressed had >1 enema vs 3/20 (15%) with simple MI who had laparotomy after enema. The number of enemas per infant (1-4), contrast agents and their concentration, were highly variable. Enterostomy was formed at 24/44(55%) of laparotomies. In infants with simple MI, time to full enteral feeds was 6 (2-10) days in those decompressing with enema vs 15 (9-19) days with laparotomy after enema. Case fatality was 4% (95% CI 0.4-12%). Two infants, both preterm died, both in the second month after birth.. Infants with simple MI achieving successful enema decompression were more likely to have had repeat enemas than those who proceeded to laparotomy. Successful non-operative management was associated with a shorter time to full feeds. The early management of infants with MI is highly variable and not standardised across the UK and Ireland.

Topics: Cohort Studies; Cystic Fibrosis; Enema; Humans; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Meconium Ileus; Prospective Studies

therapeutic strategy for meconium-related ileus (MRI) in very-low-birth-weight infants (VLBWs) has not been established. This study aims to clarify the optimum therapeutic strategy for MRI in VLBWs.. MRI was defined as delayed meconium excretion and microcolon on contrast enema with Gastrografin (diatrizoate acid). Forty-two infants with MRI were treated at our institution between 2009 and 2019, and are reviewed here. They were classified into two groups: in group A (n=21), Gastrografin regurgitated into the dilated intestine during the first or second round of Gastrografin enema (GaE), while in group B (N = 21), Gastrografin did not regurgitate. Laparotomy was indicated if the intestine was perforated, or if abdominal distention was not relieved by two rounds of GaE.. in group A, meconium was excreted in all cases within 24 h after GaE, and no cases required laparotomy. In group B, twelve cases (57%) underwent laparotomy (P < 0.01), six cases in this group (29%), showed free air on X-ray images (P < 0.01). The median hospital stay in groups A and B were 89.0 and 136.5 days, respectively (P < 0.05). Overall mortality was 2.4%.. early therapeutic diagnosis by GaE followed by early surgery is suggested as the optimum strategy for MRI in VLBWs.

Topics: Diatrizoate Meglumine; Enema; Humans; Ileus; Infant, Newborn; Infant, Very Low Birth Weight; Intestinal Obstruction; Meconium; Meconium Ileus

Thus far, few reports have described the rare, non-obstructive type of fetal diffuse bowel dilatation. We describe such a case in the fetus of a 31-year-old Pakistani pregnant woman, gravida 3, para 2. A series of ultrasonographic examinations in the third trimester showed a "honeycomb" appearance of fetal d iffuse dilated bowel loops, a mildly enlarged stomach, and mild polyhydramnios. Magnetic resonance imaging further revealed fluid-filled dilated bowel loops extending to the colon and rectum. The male neonate was born at 36 weeks and had marked abdominal distension but did not show signs of mechanical bowel obstruction. He passed a profuse amount of liquid with meconium at 4 h of life. Thereafter, his distended abdomen and bowel dilatation subsided, and he became asymptomatic within a week of life. Taken together with previous case reports, among infants who show the "honeycomb" sign in utero, there definitely exists a subset with a favorable outcome and an unknown etiology. This case alerts physicians who are responsible for perinatal care to the fact that careful assessment is required for a newborn when the "honeycomb" sign is observed via fetal imaging. Without evidence of mechanical bowel obstruction, alternative etiologies should be sought to avoid unnecessary laparotomy.

Topics: Adult; Dilatation; Female; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Magnetic Resonance Imaging; Male; Meconium; Pregnancy; Prenatal Diagnosis

Topics: Humans; Ileostomy; Infant, Extremely Low Birth Weight; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Male; Meconium; Parenteral Nutrition; Treatment Outcome

This is a case of a male patient who had a post-lung transplant complication given to distal intestinal obstruction by meconium. He was managed with laxative irrigation though enteroscope directly in the ileum with immediate resolution, without complications, avoiding surgical management. No similar cases have been reported.

Topics: Endoscopy, Gastrointestinal; Humans; Ileum; Infant, Newborn; Intestinal Obstruction; Lung Transplantation; Male; Meconium

Topics: Child; Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Meconium Ileus

Topics: Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Vomiting

A subcapsular hematoma of the liver is often found during autopsy in stillborn infants rather than clinically. It is usually asymptomatic unless ruptured; thus, the diagnosis is often delayed or missed. Rupture of a subcapsular hematoma in a premature neonate causes massive intraabdominal hemorrhage, which is associated with high mortality. Thus, early recognition and treatment to avoid rupture are imperative. We describe a case of life-threatening hemorrhage from a subcapsular hematoma of the liver during emergent laparotomy for mechanical obstruction in an 860 g premature neonate and discuss the appropriate preoperative preparation and anesthetic management for this case.

Topics: Blood Loss, Surgical; Emergencies; Fatal Outcome; Heart Arrest; Hematoma; Hemostasis, Surgical; Humans; Infant, Extremely Low Birth Weight; Infant, Extremely Premature; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Intraoperative Complications; Laparotomy; Liver Diseases; Male; Meconium; Rupture, Spontaneous

Topics: Abdomen; Brain Diseases; Cerebral Ventricles; Diagnosis, Differential; Enema; Female; Humans; Infant, Newborn; Intestinal Obstruction; Meconium

Meconium plug syndrome (MPS) is associated with Hirschsprung disease (HD) in 13-38% of cases. This study sought to assess institutional variation in utilization of rectal biopsy in children with MPS and the likelihood of diagnosing HD in this population.. Patients with MPS on contrast enema in the first 30 days of life from the Pediatric Health Information System database in 2016-2017 were included. Institutional rates of rectal biopsies performed during the initial admission were calculated and then used to predict institutional rates of early HD diagnoses using Poisson regression.. Of 373 newborns with MPS, 106 (28.4%) underwent early rectal biopsy, of whom 43 (40.5%) had HD. Fifty-seven (15.3%) were ultimately diagnosed with HD. Eight (14%) of these patients had a delayed diagnosis. HD rates between institutions did not differ significantly (range 0-50%, p=0.52), but usage of early rectal biopsy did (range 0-80%, p=0.03). Each additional early biopsy increased the early HD diagnosis rate by 35% (β=0.30, 95% CI 0.15-0.45, p<0.0001).. The incidence of HD is increased in children with MPS. There is significant hospital variability in the utilization of early rectal biopsy, and opportunity exists to standardize practice.. Study of Diagnostic test Level of Evidence: Level III.

Topics: Biopsy; Fetal Diseases; Hirschsprung Disease; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Retrospective Studies

Topics: Diagnosis, Differential; Female; Hirschsprung Disease; Humans; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Meconium; Vomiting

Meconium obstruction (MO) in extremely low birth weight (ELBW) infants is a challenging disease to treat. We performed ultrasound-guided hydrostatic enema on six ELBW infants diagnosed with MO. We consider this procedure to be safe and effective, and recommend it as a treatment for MO in ELBW infants.

Topics: Enema; Female; Humans; Infant, Extremely Low Birth Weight; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Ultrasonography, Interventional

Green-stained amniotic fluid does not always indicate that meconium was passed in utero.. We report the case of a 2280-g Hispanic preterm female born at 32 weeks of gestation with congenital jejunal atresia. The amniotic fluid was greenish stained; the initial impression was meconium-stained amniotic fluid. Postnatal findings revealed no meconium in her rectum. The content of her first stool appeared sticky and white.. In the absence of meconium in the rectum, the pediatrician should consider the possibility that the greenish amniotic fluid is not meconium stained, but rather stained with bile due to the fetus vomiting in utero secondary to intestinal obstruction.

Topics: Adult; Amniotic Fluid; Bile; Diagnosis, Differential; Female; Fetal Diseases; Gestational Age; Humans; Infant, Newborn; Infant, Premature; Intestinal Atresia; Intestinal Obstruction; Meconium; Pregnancy; Pregnancy Complications; Vomiting

Topics: Amniotic Fluid; Female; Humans; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Meconium; Pregnancy; Pregnancy Complications; Radiography, Abdominal; Vomiting

This is a single-center case series about the rare condition of volvulus without malposition and/or malrotation (VWM) in preterm babies. We focus on diagnostic difficulties, and our results should help to distinguish VWM as a distinct entity different from classical volvulus and segmental volvulus.. Medical chart review of infants with VWM from 2003-2012 was used.. A total of 15 patients were identified. All of them had volvulus in the absence of intestinal malposition or other associated intestinal pathologies. All patients were born prematurely. Emergency laparotomy was necessary in all 15 patients. Two groups were identified. Group 1 includes four patients with typical signs of meconium obstruction of prematurity (MOP). Small bowel resection was only necessary in one of these four patients, all survived without residual intestinal lesions. Group 2 consists of 11 patients without signs of MOP-small bowel resection and temporary enterostomy were necessary in all these children. Four patients presented with pneumatosis intestinalis on the abdominal plain film, suggesting necrotizing enterocolitis. Although two infants died, the survivors showed complete recovery.. VWM is a distinct disease of prematurity. When associated with MOP, VWM has a favorable outcome of treatment. In contrast, VWM occurring in the absence of signs of meconium obstruction requires small bowel resection. VWM primarily affects the top of the midgut (ileum). Because of absent malposition, presentation of VWM may be uncharacteristic. Pneumatosis intestinalis in advanced VWM may lead to diagnostic difficulties and a delay in treatment.

Topics: Databases, Factual; Diagnosis, Differential; Digestive System Surgical Procedures; Enterostomy; Gestational Age; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature; Intestinal Obstruction; Intestinal Volvulus; Intestine, Small; Meconium

This study aimed to assess the therapeutic results of ultrasound (US)-guided water-soluble contrast enema in very low birth weight (VLBW) preterm infants (<1,500 g) with meconium obstruction and to study factors that affect therapeutic results.. This study included a total of 33 consecutive VLBW infants with clinically diagnosed meconium obstruction underwent US-guided water-soluble contrast enema, from April 2007 to March 2014. Patients were classified into two groups based on to procedure outcome: the success group (evacuation of the meconium plug resolution followed by improved bowel distention within 2 days of the procedure, without additional interventions), and the failure group (the contrast enema failed to relieve the obstruction, or other procedure-related complications occurred). Patient- and mother-related clinical factors and procedure-related factors were compared between both groups.. Overall success rate was 54.5%, with 18 successful (M:F=10:8), and 15 failure (M:F=7:8) cases. When compared with the failure group, the success group patients showed statistically significant older gestational age (29(+1) vs. 27 weeks; p=0.028), larger birth weight (1023.1g vs. 790.3g; p=0.048), and higher body weight on the day of the procedure (1036.2g vs. 801.6g, p=0.049). However, no statistically significant differences were seen between other patient and maternal factors. Among the procedure-related factors, retrial of contrast injection during the procedure was associated with significantly higher success than the single trial (p=0.027). The presence of refluxed contrast into the distal ileum was the statistically significant predictor for success of the procedure (p=0.038). There were three cases of bowel perforation (9.1% per person).. US-guided water-soluble contrast enema in VLBW infants with meconium obstruction showed a 54.5% success rate and a 9.1% perforation rate per person. Among the procedure-related factors, retrial of contrast injection during the procedure and the presence of refluxed contrast into the distal ileum were related to the success of the procedure.

Topics: Acetylcysteine; Birth Weight; Cathartics; Contrast Media; Diatrizoate Meglumine; Enema; Expectorants; Female; Gestational Age; Humans; Ileal Diseases; Infant, Newborn; Infant, Premature; Infant, Very Low Birth Weight; Intestinal Obstruction; Intestinal Perforation; Iothalamic Acid; Male; Meconium; Radiography; Retreatment; Sodium Chloride; Time Factors; Treatment Outcome; Ultrasonography, Interventional

Citrulline is a non-protein amino acid synthesized in the enterocytes of the small bowel. Recent studies have reported that plasma citrulline levels correlate with functional enterocyte mass.. This study aimed to determine the normal dried blood spot (DBS) citrulline levels and to determine the existence of a correlation between citrulline levels and meconium obstruction of prematurity (MOP).. A retrospective cohort study was performed involving 285 infants born at less than 32weeks gestation who were admitted to the neonatal intensive care unit between Oct 2009 and Aug 2014.. We analyzed the DBS citrulline levels, which are routinely measured via newborn screening at 7days following birth, using liquid chromatography-MS/MS. We investigated the relationship between DBS citrulline levels and clinical parameters such as gestational age (GA), body measurements at birth, gender, or the presence or absence of either necrotizing enterocolitis or MOP.. A total of 229 infants with a median GA of 29.6weeks and a median birth weight of 1160g were included. DBS citrulline levels were not associated with GA, body measurements at birth or gender. DBS citrulline levels were significantly decreased when patients presented with MOP (p=0.037).. Early DBS citrulline levels were not associated with either GA or body measurements at birth but were reduced among preterm infants with MOP compared with the control infants. These results may be indicative of abnormal fetal intestinal development and reduced functional enterocyte mass among preterm infants with MOP.

Topics: Citrulline; Enterocolitis, Necrotizing; Enterocytes; Female; Gestational Age; Humans; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Intestinal Obstruction; Male; Meconium; Neonatal Screening; Retrospective Studies

The purpose of this study was to evaluate outcomes of the surgical management for meconium ileus (MI) and Distal Intestinal Obstruction Syndrome (DIOS) in Cystic Fibrosis (CF).. Children born between 1990 and 2010 were identified using a regional CF database. Retrospective case note analysis was performed. Outcome measures for MI were mortality, relaparotomy rate, length of stay (LOS), time on parental nutrition (TP), and time to full feeds (TFF). Outcome measures for DIOS were: age of onset, number of episodes, and need for laparotomy.. Seventy-five of 376 neonates presented with MI. Fifty-four (92%) required laparotomy. Contrast enema decompression was attempted in nineteen. There were no post-operative deaths. Thirty-nine (72%) neonates with MI were managed with stomas. LOS was longer in those managed with stomas (p=0.001) and in complex MI (p=0.002). Thirty-five patients were treated for DIOS. Twenty-five patients were managed with gastrograffin. Ten patients underwent surgical management of DIOS. Overall, MI did not predispose to later development of DIOS. There was a significantly greater incidence of laparotomy for DIOS in children who had MI.. The proportion of neonates with complex meconium ileus was high (49%) and may explain the infrequent utilisation of radiological decompression. Complex MI or management with stomas both significantly increase LOS. Re-laparotomy rate is high (22%) in MI irrespective of the type of management. DIOS is not a benign condition, particularly when the child has had previous abdominal surgery. Early referral to a surgical team is essential in these children.

Topics: Contrast Media; Cystic Fibrosis; Diatrizoate Meglumine; Digestive System Surgical Procedures; Enema; Female; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Length of Stay; Male; Meconium; Reoperation; Retrospective Studies; Surgical Stomas; Treatment Outcome

To document causes of colic in equine neonates, evaluate clinical features of neonates managed medically versus surgically, determine short- and long-term survival rates for neonates with specific medical and surgical lesions, and assess ability of patients to achieve intended use.. Retrospective case series.. 137 client-owned equine neonates (< 30 days old) with a history of colic or signs of colic within 1 hour after hospital admission examined between 2000 and 2010.. Signalment, history, results of physical examination, laboratory data, ancillary diagnostic tests, details of treatment, primary diagnosis, concurrent diseases and short-term survival rate were obtained from the medical records. Long-term follow-up information was obtained through phone survey.. 137 neonates with colic were included. The majority (122 [89%]) of neonates were managed medically. The 3 most common diagnoses associated with colic were enterocolitis (37 [27%]), meconium-associated colic (27 [20%]), and transient medical colic (26 [19%]). The most common reason for surgery was small intestinal strangulating obstruction, and these neonates were more likely to have severe, continuous pain and were less responsive to analgesics. Concurrent diseases were common (87 [64%]) but did not significantly impact survival rate. Short-term survival rate was not significantly different between medically (75%) and surgically (73%) managed neonates. Long-term survival rate was excellent (66/71 [93%]) for horses that survived to hospital discharge. Most neonates surviving to maturity were used as intended (49/59 [83%]).. Most neonates examined for signs of colic can be managed medically. Short-term survival rate in medically and surgically treated neonates was good. Long-term survival rate of foals discharged from the hospital was excellent, with most achieving intended use.

Topics: Animals; Animals, Newborn; Colic; Enterocolitis; Female; Horse Diseases; Horses; Intestinal Obstruction; Male; Meconium; Retrospective Studies

Meconium obstruction (MO) of prematurity can result in increased morbidity or mortality and prolonged hospitalization if not diagnosed and treated appropriately.. The aims of our study were to identify the incidence and risk factors associated with MO and to review the treatment outcomes.. A retrospective analysis was undertaken of 101 extremely low birth weight infants (ELBWIs) who were born between January 1, 2007, and April 1, 2009, at Seoul National University Hospital. Prenatal and neonatal factors were compared between the MO and control groups. The treatment outcomes were also reviewed.. Twenty-two (22%) patients were diagnosed as having MO. Eighteen of these patients (82%) had prenatal risk factors for MO. Respiratory distress syndrome was more prevalent in the MO group than in the control group (p = 0.001). Overall, 17 of the 22 patients (77%) were relieved with medical treatment and the rest underwent ileostomy. The times to full enteral feeding did not differ between the medically treated group and the control group. However, the surgically treated group required more time to achieve full enteral feeding, and some patients had persistent gastrointestinal problems.. MO is not a rare condition in ELBWIs, and the majority of ELBWIs have prenatal risk factors. Medical management was effective, and medically manageable cases had good prognoses for subsequent feeding, whereas some surgically managed cases had persistent gastrointestinal problems.

Topics: Adult; Comorbidity; Ductus Arteriosus, Patent; Female; Gestational Age; Humans; Ileostomy; Infant, Extremely Low Birth Weight; Infant, Newborn; Intensive Care Units, Neonatal; Intestinal Obstruction; Male; Meconium; Placental Insufficiency; Pregnancy; Republic of Korea; Respiratory Distress Syndrome, Newborn; Retrospective Studies; Sex Ratio; Ultrasonography, Prenatal

The significance of meconium plug syndrome (MPS) is unclear but has been associated with Hirschsprung's disease and magnesium tocolysis. We reviewed our experience to attempt to identify any potential association with these conditions and to review our outcomes.. Using the International Classification of Diseases, Ninth revision, code for meconium obstruction, patient charts were identified during the 1998-2008 period. A total of 61 cases of MPS were found, after excluding 7 of meconium ileus. Data regarding the hospital course and outcomes were collected and analyzed.. Approximately 30% of patients had spontaneous resolution of the meconium plug without any treatment. Of those patients requiring treatment, contrast barium enema was used, with 97% success. Only 2 patients required surgical intervention owing to worsening distension and subsequent peritonitis. When we stratified the patients according to gestational age of >36 and <36 wk, contrast barium enemas were performed 2.2 ± 1.8 versus 8.6 ± 7.8 d after birth (P = 0.003), respectively, and the lower gestational age patients had a longer length of stay. Contrast barium enema was still successful in 94% of patients with a gestational age of <36 wk. Magnesium tocolysis was noted in 16% of the cases, and Hirschsprung's disease was only found in 3.2% of patients.. Patients with MPS have excellent outcomes, independent of gestational age. Contrast barium enema remains the initial diagnostic and treatment of choice for patients with MPS. Also, although previous reports have shown a link between magnesium tocolysis and Hirschsprung's disease with MPS, our experience suggests otherwise.

Topics: Barium Sulfate; Cystic Fibrosis; Enema; Female; Hirschsprung Disease; Humans; Ileal Diseases; Incidence; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Retrospective Studies; Syndrome; Treatment Outcome

Variants associated with meconium ileus in cystic fibrosis were identified in 3,763 affected individuals by genome-wide association study (GWAS). Five SNPs at two loci near SLC6A14 at Xq23-24 (minimum P = 1.28 × 10(-12) at rs3788766) and SLC26A9 at 1q32.1 (minimum P = 9.88 × 10(-9) at rs4077468) accounted for ~5% of phenotypic variability and were replicated in an independent sample of affected individuals (n = 2,372; P = 0.001 and 0.0001, respectively). By incorporating the knowledge that disease-causing mutations in CFTR alter electrolyte and fluid flux across surface epithelium into a hypothesis-driven GWAS (GWAS-HD), we identified associations with the same SNPs in SLC6A14 and SLC26A9 and established evidence for the involvement of SNPs in a third solute carrier gene, SLC9A3. In addition, GWAS-HD provided evidence of association between meconium ileus and multiple genes encoding constituents of the apical plasma membrane where CFTR resides (P = 0.0002; testing of 155 apical membrane genes jointly and in replication, P = 0.022). These findings suggest that modulating activities of apical membrane constituents could complement current therapeutic paradigms for cystic fibrosis.

Topics: Amino Acid Transport Systems; Amino Acid Transport Systems, Neutral; Antiporters; Cell Membrane; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genetic Predisposition to Disease; Genome-Wide Association Study; Genotype; Humans; Ileus; Intestinal Obstruction; Meconium; Polymorphism, Single Nucleotide; Sodium-Hydrogen Exchanger 3; Sodium-Hydrogen Exchangers; Sulfate Transporters

Meconium ileus, intestinal obstruction in the newborn, is caused in most cases by CFTR mutations modulated by yet-unidentified modifier genes. We now show that in two unrelated consanguineous Bedouin kindreds, an autosomal-recessive phenotype of meconium ileus that is not associated with cystic fibrosis (CF) is caused by different homozygous mutations in GUCY2C, leading to a dramatic reduction or fully abrogating the enzymatic activity of the encoded guanlyl cyclase 2C. GUCY2C is a transmembrane receptor whose extracellular domain is activated by either the endogenous ligands, guanylin and related peptide uroguanylin, or by an external ligand, Escherichia coli (E. coli) heat-stable enterotoxin STa. GUCY2C is expressed in the human intestine, and the encoded protein activates the CFTR protein through local generation of cGMP. Thus, GUCY2C is a likely candidate modifier of the meconium ileus phenotype in CF. Because GUCY2C heterozygous and homozygous mutant mice are resistant to E. coli STa enterotoxin-induced diarrhea, it is plausible that GUCY2C mutations in the desert-dwelling Bedouin kindred are of selective advantage.

Topics: Amino Acid Sequence; Animals; Bacterial Toxins; Cyclic GMP; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diarrhea; Down-Regulation; Enterotoxins; Escherichia coli Proteins; Female; Gastrointestinal Hormones; Genes, Modifier; HEK293 Cells; Heterozygote; Humans; Intestinal Mucosa; Intestinal Obstruction; Male; Meconium; Mice; Molecular Sequence Data; Mutation; Natriuretic Peptides; Pedigree; Phenotype; Receptors, Enterotoxin; Receptors, Guanylate Cyclase-Coupled; Receptors, Peptide

Topics: Cystic Fibrosis; Diabetes Mellitus, Type 1; Fatal Outcome; Female; Humans; Ileal Diseases; Intestinal Obstruction; Intestines; Meconium; Radiography; Young Adult

Meconium obstruction without cystic fibrosis in low-birthweight neonates is a distinct clinical entity. We aimed to determine what therapeutic strategies work best in very-low-birthweight neonates with meconium obstruction of the small bowel under varied clinical conditions caused by the associated diseases of prematurity.. Medical records of very-low-birthweight neonates with meconium obstruction of the small bowel treated from 1998 to 2008 were retrospectively reviewed. Pre- and postnatal data, treatments, and clinical outcomes were assessed.. Nine patients with perinatal complications were identified. Mean gestational age and birthweight were 26.9 weeks and 863 g, respectively. Abdominal distension developed from 1 to 7 days of life. Five patients were initially treated with Gastrografin enema, three of whom had successful outcomes. Two hemodynamically unstable patients failed to respond to Gastrografin treatment; they ultimately died of sepsis. The remaining four without Gastrografin treatment underwent enterostomy to resolve the obstructions with good results.. Gastrografin and surgical treatments should be appropriately selected based on the underlying pathologies of meconium obstruction of the small bowel. Therapeutic Gastrografin enema is effective, safe and repeatable; however, it is not recommended for hemodynamically unstable patients. Surgical intervention is reserved for those who develop rapid abdominal distension that risks perforation.

Topics: Contrast Media; Diagnosis, Differential; Diatrizoate Meglumine; Digestive System Surgical Procedures; Female; Humans; Infant, Newborn; Infant, Very Low Birth Weight; Intestinal Obstruction; Intestine, Small; Male; Meconium; Practice Guidelines as Topic; Radiography, Abdominal

We report our experience of managing eight babies who presented with neonatal intestinal obstruction and whose rectal biopsies showed severely immature ganglion cells.. Neonatal unit records were reviewed to detect patients with suspected Hirschsprung's disease or functional intestinal obstruction. Those with intestinal atresia, anorectal malformation, malrotation, cystic fibrosis and prematurity were excluded.. We identified 73 patients born at term. Twenty-seven did not need a rectal biopsy. Twenty-one had biopsy proven Hirschsprung's disease, while 17 had a normal rectal biopsy. Eight patients, all of whom presented with severe abdominal distension, showed immature ganglion cells. Seven had failed to pass meconium after birth. X-rays in all patients showed distended loops of bowel. Two neonates underwent an emergency laparotomy and a stoma. A repeat biopsy at 3 months showed maturation of ganglion cells and the stoma was reversed. Rectal biopsy was repeated in two other patients 2-9 months after the first biopsy and showed mature ganglion cells. At follow-up, one patient still suffers from severe constipation. Seven are asymptomatic now, including the two patients who needed a stoma.. Immature ganglion cells on rectal biopsy may be an indicator of transient functional immaturity of the intestine.

Topics: Cell Nucleus; Female; Ganglion Cysts; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Rectum; Retrospective Studies; Surgical Stomas

Delayed meconium passage, typical of premature newborns, is a predisposing condition for bowel perforation with a significant risk of morbidity and mortality.. A retrospective study was undertaken to verify the entity of the disease, assess the average time to meconium passage in a neonatal population of very low birth weight (VLBW) infants, and identify associated risk factors.. The time of first stool passage was studied in 110 VLBW infants (weighing less than 1500 g at birth). Their perinatal features, clinical course, and treatment were reviewed and studied retrospectively.. Delayed meconium passage was recorded in 81% of this group. Patent ductus arteriosus, mechanical ventilation and uteroplacental insufficiency were significantly associated with delayed passage. An inverse relationship between gestational age, birth weight and meconium passage was found. Bowel perforation occurred in 4.5% of this neonatal population with a mortality of 50%.. In very low birth weight infants delay in the passage of the first stool is common. Perforation in these patients may represent a fatal event, and procedures such as daily rectal enemas, which can prevent this complication, must be applied.

Topics: Defecation; Female; Follow-Up Studies; Gestational Age; Humans; Infant, Newborn; Infant, Premature, Diseases; Infant, Very Low Birth Weight; Intestinal Obstruction; Male; Meconium; Retrospective Studies

Various definitions for distal intestinal obstruction syndrome (DIOS), meconium ileus equivalent, and constipation in patients with cystic fibrosis (CF) are used. However, an unequivocal definition for DIOS, meconium ileus equivalent, and constipation is preferred. The aims of this study were, therefore, to seek consensus on the definitions for DIOS and constipation in patients with CF and to determine the incidence, characteristics, and treatment of DIOS in a cohort of paediatric patients with CF.. During the 2005 European Society for Paediatric Gastroenterology, Hepatology, and Nutrition meeting in Porto a group of paediatric gastroenterologists discussed the definition of DIOS and constipation in CF. Subsequently, all patients younger than or equal to 18 years with complete DIOS according to the definition agreed upon and diagnosed during the years 2001 to 2005 in 8 CF centres were studied.. Distal intestinal obstruction syndrome was defined as an acute complete or incomplete faecal obstruction in the ileocaecum, whereas constipation was defined as gradual faecal impaction of the total colon. Fifty-one episodes of DIOS in 39 patients were recorded, giving an overall incidence of 6.2 (95% confidence interval, 4.4-7.9) episodes per 1000 patient-years. Of the 39 patients with DIOS, 20% experienced a relapse, 92% were pancreatic insufficient, 44% had a history of meconium ileus at birth, and 82% had a severe genotype. Conservative treatment was effective in 49 of 51 DIOS episodes (96%).. The European Society for Paediatric Gastroenterology, Hepatology, and Nutrition CF Working Group definitions of DIOS and constipation in CF are specific and make a clear distinction between these 2 entities. The incidence of DIOS in the present study was considerably higher than reported previously.

Topics: Constipation; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; Genotype; Humans; Ileal Diseases; Ileus; Incidence; Intestinal Obstruction; Meconium; Multicenter Studies as Topic

Topics: Constipation; Cystic Fibrosis; Genotype; Humans; Ileal Diseases; Intestinal Obstruction; Meconium; Multicenter Studies as Topic; Mutation

Topics: Humans; Infant, Newborn; Intestinal Obstruction; Intestinal Volvulus; Intestine, Small; Meconium; Peritonitis; Radiography

Meconium obstruction of prematurity (MO) often occurs in extremely low-birth weight (ELBW) infants, and its treatment is quite a challenge for neonatologists. We attempted to establish a method of primary treatment for MO of prematurity in ELBW infants.. An iopamidol enema with 50 cm H(2)O static pressure was performed as the primary treatment. This procedure is safe and effective and we recommend this as the first treatment for MO in ELBW infants.. The procedure was performed 50 times in 23 infants and no complications occurred. Out of 23 patients, 20 (88%) improved, but the other 3 did not. In the failure group, the procedure was performed on a significantly later date and the mortality rate was higher (12.5 vs. 67%).. This procedure is safe and effective. We recommend this as the first treatment for MO in ELBW infants.

Topics: Contrast Media; Enema; Female; Humans; Infant, Extremely Low Birth Weight; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Intestinal Obstruction; Iopamidol; Male; Meconium; Retrospective Studies; Treatment Outcome

Meconium peritonitis (MP) is a rare prenatal condition that leads to substantial neonatal morbidity and mortality.. All patients between 1998 and 2006 referred for prenatal diagnosis were retrospectively analyzed for diagnosis of MP. Prenatal ultrasound findings were compared with postnatal etiology, intraoperative findings, and postnatal outcome of the patients.. Antenatal MP was diagnosed in 14 fetuses between 18 and 38 weeks' gestation. The prenatal diagnosis of MP was confirmed by clinical and radiological findings in 8 (62%) of 13 infants born alive. All patients were delivered preterm between 33 and 36 weeks' gestation by cesarean section. Urgent neonatal surgery for treatment of bowel obstruction was required in all eight infants. Prenatal ultrasound diagnosis of bowel dilatation was the only variable found to be associated with the need for subsequent surgical intervention (P=0.02).. Clinical outcome of MP diagnosed antenatally can be either mild or severe form. The underlying cause of severe MP is heterogeneous and neonatal surgery was always required.

Topics: Dilatation, Pathologic; Female; Fetal Diseases; Gestational Age; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Peritonitis; Pregnancy; Retrospective Studies; Treatment Outcome; Ultrasonography, Prenatal

The aim of this report is to determine clinical characteristics and outcome of Cystic Fibrosis (CF).. Cases of CF managed at Infantile Medicine A Department in Children's Hospital of Tunis during 13 years (1994-2006) were reviewed.. 16 children had CF. They were 8 males and 8 females. 13 patients were consanguineous and four had similar familial cases. The mean age at diagnosis was 19 months (10 days, 13 years). 3/4 of patients were symptomatic within the first trimester of life. Revealing symptoms were: obstructive bronchopathy associated to chronic diarrhea (n=6), edema-anemia-hypotrophy-hypoproteinemia syndrome (n=3), meconium ileus (n=4), bronchiectasis (n=2) and chronic diarrhea (n=1). The diagnosis was confirmed by sweat test and genotypic data. The F508 del was the most frequent mutation (54%). Clinical outcome was characterized by the occurrence of respiratory and nutritional complications: acute respiratory failure (n=6), chronic respiratory failure (n=3), chronic pseudomonas aeruginosa infection (n=6) at a medium age of 3.8 years, recurrent haemoptysis (n=2), pleural effusion (n=2), a malnutrition (n =10) and diabetes associated to puberty delay in one patient. Seven patients died at mean age of 4.4 years (6 months, 17.3 years). Among surviving patients, six had no compromised nutritional status or lung function. Prenatal diagnosis was performed in three families.. CF is characterized by earliest onset and severity of symptoms. Therapeutic insufficiency is the main cause of precocious complications and poor prognosis in our series.

Topics: Adolescent; Bronchiectasis; Child; Child, Preschool; Chronic Disease; Consanguinity; Cystic Fibrosis; Diarrhea; Female; Genotype; Hospitals, Pediatric; Humans; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Mutation; Nutritional Status; Prognosis; Respiratory Insufficiency; Retrospective Studies; Survival Analysis; Sweat

Topics: Female; Follow-Up Studies; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium

Topics: Adult; Contrast Media; Female; Fetal Diseases; Hernia; Herniorrhaphy; Humans; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Meconium; Movement; Peritonitis; Pregnancy; Pregnancy Trimester, Third; Ultrasonography, Prenatal; Young Adult

The significance of meconium plug syndrome is dependent on the underlying diagnosis. The incidence of pathologic finding, particularly Hirschsprung's disease, contributing to the presence of these plugs, has been debated. However, there are little recent data in the literature. Therefore, we reviewed our experience with meconium plugs as a cause of abdominal distension to evaluate the associated conditions and incidence of Hirschsprung's disease.. We reviewed the records of newborns with meconium plugs found in the distal colon on contrast enema from 1994 to 2007. Demographics, radiologic findings, histologic findings, operative findings, and clinical courses were reviewed.. During the study period, 77 patients were identified. Mean gestational age was 37.4 weeks and birth weight, 2977 g. Hirschsprung's disease was found in 10 patients (13%). One had ultrashort segment disease and another had total colonic aganglionosis. Maternal diabetes was identified in 6 patients. No patients were diagnosed with cystic fibrosis, meconium ileus, malrotation, or intestinal atresia.. Meconium plugs found on contrast enema are associated with a 13% incidence of Hirschsprung's disease in our experience. Although all patients with plugs and persistent abnormal stooling patterns should prompt a rectal biopsy and genetic probe, the incidence of Hirschsprung's and cystic fibrosis may not be as high as previously reported.

Topics: Comorbidity; Female; Gestational Age; Hirschsprung Disease; Humans; Ileus; Incidence; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Length of Stay; Male; Meconium; Pregnancy; Pregnancy in Diabetics; Retrospective Studies; Syndrome

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium

The initial clinical presentation and radiographic finding of microcolon in children with long-segment intestinal aganglionosis involving the entire colon, ileum and sometimes the jejunum can mimic meconium ileus. This makes the diagnosis difficult for the radiologist and surgeon.. To document and describe the clinical and radiographic findings in children with long-segment intestinal aganglionosis who are initially thought to have meconium ileus.. We reviewed the cases of six neonates with long-segment intestinal aganglionosis presenting as meconium ileus at our institutions between 1978 and 2002. We examined the clinical presentation and the radiographic, surgical, and pathologic findings. In addition, 17 cases from the literature were identified and are included in the discussion.. A total of 23 cases were reviewed. Right lower quadrant intraluminal calcifications were noted on abdominal radiographs in all six neonates of our series and were described in 13 of the 17 neonates reported in the literature. Similarly, a microcolon was present in five of the six neonates of our series and in 14 of 16 historical neonates (one not reported).. In a neonate with small-bowel obstruction and a microcolon, the presence of right lower quadrant intraluminal calcifications should raise the suspicion of long-segment intestinal aganglionosis even if the operative findings are typical of meconium ileus and a biopsy should be performed.

Topics: Biopsy; Calcinosis; Colon; Diagnosis, Differential; Hirschsprung Disease; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Radiography; Rectum; Retrospective Studies

This matched case-control study compared the nutritional and the pulmonary long-term outcomes of cystic fibrosis (CF) patients presenting a history of meconium ileus (MI) with early-diagnosed symptomatic CF without MI (non-MI).. Twenty-six patients with CF treated for MI between 1980 and 1997 have been matched for sex, birth date, and earliest CF symptomatic diagnosis for the children with non-MI CF. Clinical characteristics, genotype and complications were evaluated as well as the progression of the CF disease from infancy to 15 years old by nutritional status (z score weight, z score height), pulmonary function tests (PFTs) (FVC and FEV1), and Pseudomonas aeruginosa acquisition.. Median duration of the follow-up was 12.5 years (range, 10-17 years). Genotype identification showed no significant difference. Further on, the rate of complications and the occurrence of chronic P. aeruginosa colonization did not differ. At age of 15 years (n = 13), nutritional status and PFTs did not demonstrate any significant difference.. These results suggest that adequate initial nutritional and medical management of MI allows further similar nutritional status and PFTs compared with other early-diagnosed symptomatic CF patients. In this study, MI did not represent an additional risk factor for the patient's life.

Topics: Adolescent; Case-Control Studies; Cathartics; Child; Child, Preschool; Cohort Studies; Cystic Fibrosis; Diatrizoate Meglumine; Digestive System Surgical Procedures; Enema; Female; Humans; Ileus; Infant; Intestinal Obstruction; Male; Meconium; Nutrition Assessment; Pseudomonas aeruginosa; Pseudomonas Infections; Respiratory Function Tests; Treatment Outcome

The authors present a case of cystic fibrosis in a newborn. The first symptom of cystic fibrosis in this case was meconium ileus. They describe correct diagnostic method of ileus in newborn, consecutive operation and last but not least they show necessity of follow-up investigations to verification of cystic fibrosis.

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium

Topics: Diagnosis, Differential; Female; Fetal Diseases; Humans; Ileal Diseases; Infant, Newborn; Intestinal Obstruction; Magnetic Resonance Imaging; Male; Meconium; Pregnancy

Meconium ileus equivalent is one of the lesser-known manifestations of cystic fibrosis. It manifests as distant small bowel obstruction caused by meconium-like stool plugs and occurs mostly in adult patients. With the improved overall survival of patients with cystic fibrosis, general surgeons may encounter this condition more often in the future. We treated a 19-year-old woman with cystic fibrosis who presented with complete distal small bowel obstruction. Medical therapy with Gastrografin and N-acetylcysteine failed to resolve the obstruction. At surgery, a meconium-like plug in the distal ileum was manually pushed into the colon with subsequent relief of symptoms. Meconium ileus equivalent should be considered and treated in cystic fibrosis patients presenting with small bowel obstruction.

Topics: Adult; Cystic Fibrosis; Female; Humans; Intestinal Obstruction; Intestine, Small; Meconium

We reviewed the final diagnosis and incidence of bowel pathology in neonates presenting with large bowel obstruction that was relieved by the passage of meconium plugs. A retrospective case-note review was undertaken of all patients with a discharge diagnosis of meconium plug syndrome (MPS), meconium ileus (MI), Hirschsprung's disease (HD), or small left colon syndrome (SLCS) from January 1996 to April 2002. Of 21 patients with meconium plug obstruction, eight (38%) had HD, nine had MPS, four had SLCS, and none had MI. However, there was considerable clinical and radiological overlap between MPS and SLCS, suggesting that these terms are imprecise. We found a much higher incidence of HD in babies presenting with meconium plug obstruction than has previously been reported. Overlap between MPS and SLCS suggests that these are not specific diagnoses and that current terminology needs to be changed. All babies with meconium plug obstruction should have HD and cystic fibrosis (CF) excluded.

Topics: Colonic Diseases; Humans; Incidence; Infant, Newborn; Intestinal Obstruction; Meconium; Retrospective Studies

Guidelines for diagnosis and therapy of meconium obstruction in extremely low birth weight neonates are still not well established.. All low-birth-weight infants presenting with meconium obstruction over a 5-year period were reviewed retrospectively. Patients with meconium plug syndrome or cystic fibrosis were excluded.. Seven patients were identified. Average birth weight and gestational age were 874 g and 27.7 weeks, respectively. All were products of high-risk pregnancies, and 6 (86%) were delivered by cesarean section. All patients presented with distended abdomens without peritonitis. Abdominal films showed multiple distended intestinal loops without air-fluid levels in all cases. Three patients (43%) had contrast enemas, all showing microcolon. Nonoperative therapy, consisting of rectal irrigations and N-acetylcysteine per orogastric tube, succeeded in 3 patients who were obstructed for less than 10 days and failed in 1 patient obstructed for 12 days. Four patients, all obstructed for more than 10 days, underwent enterotomy and lavage (2), bowel resection and ileostomy (1), and initial peritoneal drainage for perforation followed by bowel resection (1). All patients survived with intact bowel function.. Extremely low-birth-weight infants with meconium obstruction can be diagnosed based on their typical clinical and plain radiographic characteristics, without need for a contrast enema. Nonoperative treatment is successful early in the course of the obstruction. In the absence of an intestinal complication, simple enterotomy and meconium evacuation effectively treats long-standing obstruction.

Topics: Algorithms; Enema; Humans; Ileal Diseases; Ileum; Infant, Newborn; Infant, Premature, Diseases; Infant, Very Low Birth Weight; Intestinal Obstruction; Meconium; Radiography; Retrospective Studies

Meconium obstruction of prematurity is a distinct clinical condition that occurs in very low birth weight infants, predisposing them to intestinal perforation and a prolonged hospitalization if not diagnosed and treated promptly. We report a series of 21 infants, including 2 detailed case reports, whose clinical course is indicative of meconium obstruction of prematurity. Specific risk factors are identified along with descriptions of clinical and radiologic findings, disease course, treatment, and outcome. Meconium obstruction of prematurity was more common in infants with a maternal history of pregnancy-induced or chronic hypertension, suggesting the possibility of decreased intestinal perfusion prenatally. Inspissated meconium was located most frequently in the distal ileum, making this disease process difficult to treat. Gastrografin enemas were safe, diagnostic, and therapeutic. Delay in diagnosis and treatment was associated with perforation and delay in institution of enteral feeds.

Topics: Diagnostic Errors; Enema; Humans; Ileus; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Infant, Small for Gestational Age; Infant, Very Low Birth Weight; Intestinal Obstruction; Intestinal Perforation; Male; Meconium; Radiography; Risk Factors

About one-half of patients with meconium ileus (MI) present with a complication such as volvulus, atresia, meconium peritonitis or giant cystic meconium peritonitis. The treatment of these complications requires surgery. However, the preoperative diagnosis of complicated MI is difficult. We describe two neonates with complicated small-bowel obstruction, one with MI related to cystic fibrosis and the other not related to cystic fibrosis. In both, contrast enema depicted a spiral appearance of the distal small bowel, which at surgery proved to be the result of volvulus associated with antenatal bowel perforation. This appearance of the small bowel on contrast enema in this clinical setting has not been previously described. The recognition of this spiral appearance of the distal small bowel suggests the need for surgery.

Topics: Contrast Media; Cystic Fibrosis; Enema; Female; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Intestinal Volvulus; Intestine, Small; Meconium; Radiography, Abdominal; Ultrasonography, Interventional

Two newborn girls presented with congenital small-bowel atresia; in one case a high intestinal obstruction had been demonstrated by prenatal echography, while in the other case there were feeding problems and a failure to produce meconium. In both infants, the postoperative period was complicated by feeding problems, malabsorption and insufficient growth. Cystic fibrosis (CF) was then diagnosed in both patients. After modification of the diet, both showed rapid growth to a normal weight. The prevalence of CF in children with congenital small-bowel atresia is 6-13%, which is considerably higher than in a normal population. There is still no good explanation for this finding, but it is likely that CF contributes to the development of small-bowel atresia. In view of the high prevalence of CF in children with small-bowel atresia, children with congenital small-bowel atresia should be examined for CF.

Topics: Cystic Fibrosis; Female; Humans; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Intestine, Small; Meconium; Postoperative Complications; Treatment Outcome

Topics: Acetylcysteine; Humans; Infant, Newborn; Infant, Very Low Birth Weight; Intestinal Obstruction; Intestinal Perforation; Intussusception; Meconium; Risk Factors

To determine the incidence of cystic fibrosis (CF) in neonates with intestinal obstruction (NIO) secondary to meconium ileus (MI), jejunoileal atresia (JA), meconium plug syndrome (MPS), volvulus (V), and meconium peritonitis (MP) and analyze the correlation of ultrasonographic (US) signs with CF in NIO with a prenatal diagnosis of intestinal anomaly, a prospective analysis of different types of NIO from 1990 to 1998 was undertaken. Immunoreactive trypsin measurement, genetic studies, and sweat tests were performed to confirm or rule out CF. Cases with prenatal diagnosis were analyzed for gestational age, dilated bowel, ascites, hyperechoic bowel, and calcifications. Of 80 neonates, 19 (24%) had CF: 2/33 (6%) JA, 6/14 (43%) MPS, 1/14 (7.1%) MP, 10/10 (100%) MI, and 0/9 V. Thirty (37.5%) had a prenatal diagnosis of an intestinal anomaly. The overall incidence of CF in NIO with a prenatal diagnosis of intestinal anomaly was 4/30 (13%), or 333 times the estimated risk of CF in the general population. A hyperechoic pattern with dilated bowel was associated with higher specificity for CF: 3/3 cases (100%), followed by hyperechoic bowel with ascites: 3/4 cases (75%). All babies with any type of NIO should thus be screened for CF. Prenatal screening for CF should be indicated in all pregnancies with US patterns of specific intestinal disorders.

Topics: Cystic Fibrosis; Female; Fetal Diseases; Humans; Incidence; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Pregnancy; Prenatal Diagnosis; Prospective Studies

Fetal small bowel obstruction is usually diagnosed by sonography in the late second or early third trimester. We report two such cases of different etiology: a case of meconium ileus due to cystic fibrosis and a case of intestinal atresia. The only sonographic finding that allowed differentiation between them was the echogenic bowel in the case of cystic fibrosis.

Topics: Adult; Cystic Fibrosis; Fatal Outcome; Female; Fetal Diseases; Gestational Age; Humans; Intestinal Atresia; Intestinal Obstruction; Intestine, Small; Meconium; Pregnancy; Ultrasonography, Prenatal

The objective was to evaluate the incidence of gastrointestinal abnormalities amongst those fetuses with antenatally diagnosed echogenic bowel (EB).. A retrospective review of all cases delivered from April 2002 to March 2003 with antenatally diagnosed EB was conducted. This was defined as bowel that appeared as echogenic as (if not greater than) the iliac bone on a real-time image. The postnatal outcomes with regard to gastrointestinal abnormalities, till their discharge, were noted.. Of the 13,941 patients delivered, there were 70 cases with antenatally diagnosed EB, giving an incidence of 70/13,941 or 0.50%. Of these, 6 defaulted follow-up and 1 had a mid-trimester termination of pregnancy at 21 weeks' gestation for social reasons. Of the remaining 63 cases with EB, 2 were stillbirths at 31 weeks and 35 weeks of gestation, respectively. Three fetuses (3/63 or 4.76%) were diagnosed with gastrointestinal abnormalities. Meconium plug syndrome was diagnosed postnatally in 2 cases, of which, 1 resolved with conservative management while the other required an emergency laparotomy. Intestinal atresia was diagnosed in the postmortem of one of the stillbirths. There was evidence of intrauterine growth retardation (IUGR) in both the stillbirth and the fetus that had required laparotomy. None of the 3 fetuses exhibited clinical features of aneuploidy.. As the quoted background risk for gastrointestinal pathology is 0.23%, an increased incidence (4.76%) is observed in those fetuses found to have antenatal EB. It is possible that the presence of IUGR is associated with a worse prognosis. Further prospective studies are needed to verify this association.

Topics: Abnormalities, Multiple; Adult; Female; Fetal Diseases; Fetal Growth Retardation; Humans; Incidence; Infant, Newborn; Intestinal Obstruction; Intestines; Maternal Age; Meconium; Parity; Pregnancy; Pregnancy Outcome; Pregnancy Trimester, Second; Pregnancy, High-Risk; Prenatal Care; Prognosis; Risk Factors; Singapore; Ultrasonography, Prenatal

Topics: Cystic Fibrosis; Diagnosis, Differential; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Physical Examination; Risk Assessment; Scrotum; Sensitivity and Specificity; Vas Deferens

Topics: Adult; Amniotic Fluid; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Analysis; Female; Gastrointestinal Diseases; Genetic Carrier Screening; Heterozygote; Humans; Intestinal Obstruction; Intestines; Jews; Meconium; Mutation; Ultrasonography, Prenatal

We present two cases of fetal meconium peritonitis in a single and twin pregnancy, respectively. The first case diagnosis was made at 30 weeks and was confirmed after delivery of the twins by cesarean section at 37 weeks. The second case diagnosis was made at 31 week and was confirmed at 37 weeks. Meconium peritonitis is a rare prenatal complication that results from intrauterine perforation of small bowel with spillage of sterile meconium into peritoneal cavity. We now report two cases of meconium peritonitis diagnosed at 30 and 31 weeks gestation.

Topics: Adult; Diagnosis, Differential; Female; Fertilization in Vitro; Fetal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Jejunal Diseases; Male; Meconium; Peritonitis; Pregnancy; Pregnancy Trimester, Third; Pregnancy, Multiple; Twins; Ultrasonography, Prenatal

Although its pathogenesis remains still unknown, fibrosing colonopathy (FCP) is considered to be the result of prolonged treatment by high doses of pancreatic enzyme preparations, in a small proportion of patients who present with cystic fibrosis (CF). We present the case of a newborn with meconium ileus (treated by conservative measures), in which, at the age of 3 weeks, the features of intestinal obstruction made necessary the removal of 15 cm of the proximal large intestine. Macroscopical and especially microscopical appearances typical for FCP were found, despite the absence of any enzymatic treatment. These findings raised the suspicion of CF, which was confirmed 4 weeks later at necropsy by the presence of characteristic pancreatic lesions. This case and another similar report in the literature suggest that the mechanism of FCP must be linked with the disease itself, at least in some patients. Thus, for us, FCP is not a "closed subject" and we sustain the importance of continuing studies, which will shed light on its etiopathogenesis.

Topics: Colon; Colonic Diseases; Cystic Fibrosis; Dietary Supplements; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Pancreatin

The etiology of meconium obstruction without cystic fibrosis is unclear. Interstitial cells of Cajal (ICC) function as pacemakers in gut motility and may play a role in the pathophysiology of the disease.. The ICC were examined by immunohistochemical staining with anti-c-kit antibody in the bowel walls of 6 neonates who had meconium obstruction without cystic fibrosis, and the results were compared with specimens from normal neonates (n = 2).. Six patients underwent ileostomy between 2 and 15 days after birth, and 5 of them presented with microcolon. Ganglion cells were present in the ileum and colon. Whereas ICC were evenly distributed in the control specimens, they were not seen at the time of ileostomy in the colons of 2 patients, and the other 4 showed scanty distribution in muscle layers. However, ileum showed normal distribution of ICC in all patients. The ileostomies were closed between 39 and 104 days of age, and the ICC distribution was changed to a normal pattern in the colons of all 6 patients. Their bowel movements were restored to normal after closure.. The findings of this study suggest that delayed maturity of ICC may be a cause of meconium obstruction without cystic fibrosis.

Topics: Coiled Bodies; Colonic Diseases; Female; Gastrointestinal Motility; Humans; Ileal Diseases; Ileostomy; Immunohistochemistry; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium

A female neonate developed abdominal distension with vomiting. She was suffering from meconium ileus and cystic fibrosis.

Topics: Abdomen; Cystic Fibrosis; Female; Humans; Ileal Diseases; Ileum; Infant, Newborn; Intestinal Obstruction; Meconium; Vomiting

The objective of the present study was to determine the possible prognostic factors which may explain the difference in the survival of patients with cystic fibrosis (CF) with and without meconium ileus. Over a period of 20 years, 127 patients with CF, whose diagnosis was confirmed by typical clinical characteristics and altered sweat chloride levels, were studied retrospectively. The patients were divided into two groups: group 1 consisted of patients who presented CF and meconium ileus (N = 9), and group 2 consisted of patients with CF without meconium ileus (N = 118). The characteristics studied were based on data obtained upon admission of the patients using a specific protocol. Demographic, clinical, nutritional and laboratory data were obtained. The genotype was determined in 106 patients by PCR. Survival was analyzed using the Kaplan-Meier method. The median follow-up period was 44 months. A statistically significant difference was observed between the groups studied regarding the following variables: age at diagnosis and weight and height z scores. The presence of meconium ileus was associated with an earlier diagnosis; these patients had greater deficits in height and weight at the time of diagnosis and at the end of the study. The estimated probability of survival for patients with CF without meconium ileus was 62 +/- 14% and for those with meconium ileus 32 +/- 18%. Patients with CF and meconium ileus presented a poor nutritional status at diagnosis and a lower survival rate compared to the general CF population.

Topics: Chi-Square Distribution; Cystic Fibrosis; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Prognosis; Retrospective Studies; Survival Rate

The aim of this study was to develop new nonoperative strategies for the management of meconium ileus in an attempt to improve on the current unacceptable failure rate.. Mice were constipated with subcutaneous morphine injection. Each then received an enema solution (perflubron, surfactant, Tween-80, Gastrografin, Golytely, DNase, N-Acetylcysteine, Viokase, or normal saline). After the enema solution was administered, stool output was quantitated. Histologic examination of the intestines was performed on a second group of mice that also received enemas. Finally, viscosity measurements were taken of human meconium at baseline and after variable incubation periods with each test solution.. For relieving constipation in vivo, Gastrografin enema was most efficacious. All agents were equally benign to the intestinal mucosa. In vitro, only 4% N-Acetylcysteine and perflubron were less effective at decreasing meconium viscosity than normal saline at T = 0 hours, with N-Acetylcysteine producing greater reduction in viscosity than normal saline at T = 6 hours.. Our results show that surfactant and Gastrografin are the most effective for the in vivo relief of constipation. This is accomplished without mucosal damage. These agents also significantly reduce viscosity in vitro, and we speculate that they may be beneficial in relieving constipation caused by meconium ileus in cystic fibrosis patients.

Topics: Animals; Constipation; Diatrizoate Meglumine; Enema; Humans; In Vitro Techniques; Infant, Newborn; Intestinal Obstruction; Intestines; Male; Meconium; Mice; Viscosity

The incidence of cystic fibrosis (CF) is very rare in Japanese, while it is frequent in Caucasians. Here we report on three Japanese cases of CF. One of the patients had consanguineous parents. All three patients initially developed meconium ileus, and hepatobiliary and pancreatic changes became more severe as age increased. The DeltaF508 mutation, the most frequent mutation associated with CF in Caucasians, was not found in these patients. To evaluate the relationship between the severity of hepatic lesions and a history of meconium ileus, we examined hepatic lesions in the present three cases, and we reviewed 22 Japanese autopsied cases of CF in the literature. No correlation was found between the incidence of biliary cirrhosis and a history of meconium ileus, because the cases with meconium ileus showed a high mortality during the neonatal period, before biliary cirrhosis developed. The high incidence of meconium ileus in Japanese CF patients may relate to a clinically severe phenotype and reflect a different genetic background between Caucasians and Japanese.

Topics: Adolescent; Autopsy; Child, Preschool; Consanguinity; Cystic Fibrosis; Fatal Outcome; Female; Humans; Infant; Intestinal Obstruction; Japan; Liver; Liver Cirrhosis, Biliary; Male; Meconium; Pancreas; United States

We report a case of in utero paracentesis of ascites in a fetus with meconium peritonitis due to volvulus at 34 weeks which resulted in the correction of an abnormal fetal heart rate pattern and enabled vaginal delivery by preventing abdominal dystocia. The intrauterine intervention also helped to establish the diagnosis and potentially reduced the respiratory compromise after birth.

Topics: Adult; Arrhythmias, Cardiac; Diagnosis, Differential; Female; Fetal Diseases; Fetal Monitoring; Heart Rate, Fetal; Humans; Ileal Diseases; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Paracentesis; Peritonitis; Pregnancy; Pregnancy Trimester, Third; Prenatal Diagnosis; Twins

We report on a female preterm infant with hepatic failure and neonatal tissue siderosis of hemochromatotic type diagnosed by using both histochemistry and atomic absorption spectroscopy. The infant presented with meconium ileus, signs of rapidly progressive hepatic failure, and hyperferritinemia (7132 ng/ml). Despite surgery and intensive care the infant died 32 days after birth. Postmortem examination showed a wrinkled liver with extensive collapse of the hepatic architecture and regenerating nodules as well as hepatic and extrahepatic iron accumulation of hemochromatotic type, sparing the reticuloendothelial system. Atomic absorption spectroscopy confirmed an increase in the iron content of various organs: liver, heart, pancreas, oral salivary gland, kidney, and adrenal gland. The increase in the iron content of various organs was determined by comparing the analysis of the propositus with those of 5 gestationally age-related preterm infants who had died in the intensive care unit: 2 died of meconium aspiration syndrome, the other 3 of hyaline membrane disease, bronchopulmonary dysplasia, and immaturity, respectively. We also compared the analysis of 15 fetuses having a a condition predisposing to iron accumulation (trisomy 21, trisomy 18, cytomegalovirus, amnion infection syndrome, Rhesus- and ABO-incompatibility, congenital hemolysis, anti-phospholipid syndrome, congenital heart disease). Delta F508, the most frequent mutation seen in cystic fibrosis patients, was excluded by gene sequencing. Different noxae causing iron accumulation in the neonatal period have led to the statement that neonatal hemochromatosis may collect different etiologies, such as metabolic disorders, infections, chromosomal aberrations, and immunological disorders. In this study, we report the singular evidence of neonatal iron accumulation of hemochromatotic type in an infant presenting with meconium ileus and propose a classification of the neonatal disorders associated with iron accumulation.

Topics: DNA; DNA Mutational Analysis; Fatal Outcome; Female; Hemochromatosis; Histocytochemistry; Humans; Infant, Newborn; Intestinal Obstruction; Iron; Liver Failure; Meconium; Prussian Blue Reaction; Siderosis; Spectrophotometry, Atomic

The present study was designed to compare the clinical course of children diagnosed with cystic fibrosis (CF) in infancy due to the presence of meconium ileus (MI) with children diagnosed by way of a newborn screening programme (non-MI). A matched case-control study design was used. Matching was performed on the basis of sex and date of birth. All children born in New South Wales, Australia after 1980 and who had attended the CF clinic at The Children's Hospital at Westmead since diagnosis were included as possible cases or controls. Parameters pertaining to the clinical course were compared in 39 matched pairs. MI children had a significantly worse pulmonary status. The forced expiratory volume in one second was 16.3 +/- 5.2% higher (p<0.001, n=21 pairs) and the forced vital capacity value 10.5 +/- 4.7%, higher (p<0.05, n=21 pairs) in non-MI children. The difference between the pairs (18.6 +/- 4.4 MI and 20.5 +/- 3.4 non-MI) in the Shwachman chest radiograph score was statistically significant (p<0.05, n=39 pairs). There were no significant differences in any other assessed parameters, such as height, weight, the presence of liver function abnormalities, the frequency of hospitalization or airway microbial colonization. Meconium ileus may be an early indication of a more severe phenotype of cystic fibrosis. This was suggested by the significantly lower pulmonary function found in children with a history of meconium ileus compared to age- and sex-matched children who did not have meconium ileus.

Topics: Case-Control Studies; Child; Cystic Fibrosis; Female; Genotype; Humans; Intestinal Obstruction; Male; Meconium; Nutritional Status; Respiratory Mechanics

The Na+-dependent amino acid transporter named ATB(0) was previously found to be located in 19q13.3 by fluorescence in situ hybridisation. Genetic heterogeneity in the 19q13.2-13.4 region, syntenic to the Cystic Fibrosis Modulator Locus 1 (CFM1) in mouse, seemed to be associated to the intestinal phenotypic variation of cystic fibrosis (CF). We performed fine chromosomal mapping of ATB(0) on radiation hybrid (RH) panels G3 and TNG. Based on the most accurate location results from TNG-RH panel, mapping analysis evidenced that ATB(0) is localised between STS SHGC-13875 (D19S995) and STS SHGC-6138 in 19q13.3, that corresponds with the immediately telomeric/distal segment of the strongest linkage region within the human CFM1 (hCFM1) syntenic region. Regarding to the genomic structure and exon organisation, our results show that the ATB(0) gene is organised into eight exons. The knowledge of the genomic structure allowed us to perform an exhaustive mutational analysis of the gene. Evaluation of the possible implication of ATB(0) in the intestinal phenotype of CF was performed on the basis of the functional characteristics of the encoded protein, its apparent relevance to meconium ileus (MI) and position in relation to the hCFM1 syntenic region. We have analysed this gene in samples from CF patients with and without MI. Several sequence variations in the ATB(0) gene were identified, although none of them seemed to be related to the intestinal phenotype of CF. Even though no particular allele or haplotype in ATB(0) appears to be associated to CF-MI disease, new SNPs identified should be useful in segregation and linkage disequilibrium analyses in families affected by other disorders caused by the impairment of neutral amino acid transport.

Topics: Alleles; Amino Acid Transport System ASC; Chromosome Mapping; Chromosomes, Human, Pair 19; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA; DNA Mutational Analysis; Exons; Gene Frequency; Genes; Genotype; Humans; Intestinal Obstruction; Intestines; Introns; Meconium; Minor Histocompatibility Antigens; Mutation; Phenotype; Polymorphism, Genetic; Polymorphism, Single Nucleotide; Radiation Hybrid Mapping

This study was pursued as an extension of a randomized clinical investigation of neonatal screening for cystic fibrosis (CF). The objective was to determine if CF patients with meconium ileus (MI) were more likely to be malnourished compared with those without MI who were diagnosed during early infancy through neonatal screening.. Nutritional status was evaluated from early infancy to 13 years of age based on anthropometric, biochemical, and dietary assessments.. MI patients (n = 32) were smaller at birth (3117 g compared with 3413 g) and were shorter (22nd percentile compared with 48th percentile) and thinner (24th percentile compared with 49th percentile) compared with non-MI early diagnosed patients (n = 50) up to 13 years of age. Poor growth was particularly evident in 26 MI patients who required surgery for MI (height and weight at the 20th percentile), whereas those treated without surgery (n = 6) showed better height (45th percentile) and weight (37th percentile). Abnormal essential fatty acid profiles were significantly more prevalent in MI compared with non-MI early-diagnosed patients before 3 years of age. Daily intakes of calorie (130% compared with 111% recommended dietary allowances) and protein (339% compared with 279% recommended dietary allowances) were higher but the percentage of fat (37% compared with 38%) and linoleic acid (4.5% compared with 4.7%) in the diet were similar between the two groups.. These results demonstrated a clear association of MI with malnutrition in CF. The observed poor growth among our MI patients was not because of poor dietary intakes, but was related to surgical treatment for MI and poor essential fatty acid status. These findings present new challenges regarding the optimal medical treatment and nutritional intervention for CF patients with MI.

Topics: Case-Control Studies; Cystic Fibrosis; Dietary Fats; Fatty Acids, Essential; Female; Growth; Humans; Infant; Intestinal Obstruction; Male; Meconium; Neonatal Screening; Nutrition Disorders; Nutritional Status; Registries

Meconium disease (MD) results in intestinal obstruction in the neonate where tenacious meconium is found in the distal ileum and proximal colon. The obstructive symptoms improve at several days of age after some of the meconium is passed. We observed premature infants with MD who underwent ileostomy for intestinal obstruction due to tenacious meconium. Afterward, meconium was passed well and the clinical symptoms improved. After closing the ileostomy, growth and defecation became normal. The MD in our cases was documented by histologic changes in the maturation of ganglion cells observed at the time of ileostomy creation and closure. For an objective evaluation of the maturation of intestinal ganglion cells (IGC), we attempted to distinguish immature from mature cells by the expression of cathepsin D. We examined the distribution of cathepsin D in IGC in patients with MD to test the hypothesis that ganglion-cell immaturity might be related to MD. In ganglion cells at the time of ileostomy, cathepsin D was detected in the perinuclear cytoplasm (immature staining pattern), while at the time of ileostomy closure it was detected in intense granules throughout the cytoplasm (mature staining pattern). We propose that it would be possible to evaluate the maturation of IGC by the intracellular distribution of cathepsin D in MD and suggest that immaturity of IGC might be the cause of MD.

Topics: Cathepsin D; Ganglia; Gestational Age; Humans; Ileostomy; Immunohistochemistry; Infant, Newborn; Intestinal Obstruction; Intestines; Meconium

The T-tube ileostomy was first used at Texas Children's Hospital in 1959. The purpose of this study is to update the experience since the initial report of this technique in 1981.. A database of 448 patients with cystic fibrosis (CF) seen in the authors' institution was used to identify 83 patients (18.5%) who presented with meconium ileus. The clinic and hospital charts of these patients were reviewed retrospectively to identify patients who had undergone placement of a T-tube ileostomy.. Surgery was performed in 60 of 83 patients for complications of meconium ileus or failure to evacuate the meconium after a contrast enema. Of these patients, 21 of 60 (35%) underwent placement of a T-tube ileostomy. An additional 8 patients were identified who underwent placement of a T-tube ileostomy but were not included in the CF database, for a total of 29 patients who have been treated with T-tube ileostomy since 1959 at Texas Children's Hospital. Five patients were excluded from analysis because of insufficient data or misdiagnosis. One of the 24 patients in the series died of complications of prematurity. A total of 20 of 23 patients had resolution of their meconium ileus after T-tube irrigation with n-acetylcysteine or pancreatic enzymes. Three patients required additional surgery to relieve persistent bowel obstruction. All patients had the T-tube removed within the first 8 weeks after surgery. Two patients required subsequent repair of an incisional hernia. There were otherwise no complications of this procedure, with an average follow-up of 11.5 years.. In patients with uncomplicated meconium ileus unrelieved by contrast enema, the T-tube ileostomy is an effective and safe treatment.

Topics: Cystic Fibrosis; Humans; Ileostomy; Infant, Newborn; Intestinal Obstruction; Meconium

Bilious vomiting in newborns is an urgent condition that requires the immediate involvement of a team of pediatric surgeons and neonatologists for perioperative management. However, initial detection, evaluation and treatment are often performed by nurses, family physicians and general pediatricians. Bilious vomiting, with or without abdominal distention, is an initial sign of intestinal obstruction in newborns. A naso- or orogastric tube should be placed immediately to decompress the stomach. Physical examination should be followed by plain abdominal films. Dilated bowel loops and air-fluid levels suggest surgical obstruction. Contrast radiography may be required. Duodenal atresia, midgut malrotation and volvulus, jejunoileal atresia, meconium ileus and necrotizing enterocolitis are the most common causes of neonatal intestinal obstruction.

Topics: Duodenum; Enterocolitis, Necrotizing; Humans; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Meconium; Rotation; Vomiting

Topics: Animals; Chromosomes, Human, Pair 19; Cystic Fibrosis; Humans; Intestinal Obstruction; Meconium; Mice

Topics: Adenine; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exons; Guanine; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Sequence Deletion

We present 4 cases of fetal intestinal obstruction and their ultrasonographical findings. With regard to the reported cases we discuss the diagnostic and differential-diagnostic considerations of this rare but not uncommon fetal malformation.

Topics: Adolescent; Adult; Child; Child, Preschool; Diagnosis, Differential; Down Syndrome; Female; Fetal Growth Retardation; Follow-Up Studies; Humans; Infant; Intestinal Atresia; Intestinal Obstruction; Male; Meconium; Pregnancy; Ultrasonography, Prenatal

The variability in intestinal disease severity in patients with cystic fibrosis (CF) has been associated with the expression of secondary modifier genes. The locus containing these modifier genes in CF patients is syntenic with a modifier locus previously associated with survival in CF transmembrane conductance regulator-knockout mice. These previous studies showed that the proportion of CF mice that survive weaning (mildly affected mice) versus those that succumb to obstruction of the small intestine (severely affected) is related to their genetic background and the expression of modifier genes. In the present work, we show that the basal transepithelial chloride transport measured across jejuna obtained from mice of mixed genetic backgrounds segregates into two groups, some mice having low and others having high, near normal chloride transport. Further, we report that the segregation of mice with respect to intestinal chloride transport correlates with their predicted segregation on the basis of genotype at the "modifier locus." These findings support the hypothesis that intestinal disease modification in CF mice correlates with improved chloride transport through non-CF transmembrane conductance regulator chloride channels.

Topics: Animals; Animals, Newborn; Animals, Suckling; Chloride Channels; Chlorides; Crosses, Genetic; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diet; Epithelium; Intestinal Mucosa; Intestinal Obstruction; Ion Transport; Jejunum; Meconium; Membrane Potentials; Mice; Mice, Inbred BALB C; Mice, Knockout; Severity of Illness Index

a) assessment of the relationship between ileus of premature infants and the development of intestinal perforation in premature infants, b) suggestions of an optimal therapeutic procedure. In the study children (n = 50) are included a) with intestinal perforation in conjunction with impaired excretion of meconium (n = 22), b) with an ileus state based on obstruction of the ileum by a viscous meconium treated either surgically or conservatively (n = 28). Surgical treatment involved: a) establishment of a double ileostomy (n = 28), b) insertion of a T drain into the terminal ileum (n = 8), c) removal of meconium from the gut and its primary closure (n = 2). Conservative treatment in 11 children involved irrigography with an liquid contrast substance under X-ray control. The group of children with perforation was compared with the group of children without perforation, risk factors were evaluated by statistical methods. The necessity of ventilation (P = 0.051) and gestation age (P = 0.006) proved to be statistically significant risk factors for the development of perforation. Survival was not influenced by perforation. All 11 children treated conservatively survived, of 39 operated children 26 survived (66.7%). An early start of conservative treatment of ileus of premature infants reduces markedly the risk of intestinal perforation and can thus influence the survival of low birth weight neonates.

Topics: Female; Humans; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Intestinal Perforation; Male; Meconium; Risk Factors

We reviewed 36 cases of neonatal intestinal obstruction admitted to our surgical unit over a 10-year period, 1986-1996, for surgical intervention following the failure of conservative treatment. There were more males than females and the age range was 12 hours-26 days. Imperforate anus was the main cause of the obstruction (27.8%) followed by duodenal atresia (13.9%) and colonic atresia and meconium ileus (11.1% each). There were 8 deaths following surgery (22% mortality rate), the main causes being aspiration pneumonia, septicaemia and hypothermia.

Topics: Age Distribution; Anus, Imperforate; Cause of Death; Colon; Duodenum; Female; Hospital Mortality; Hospitals, General; Humans; Infant Mortality; Infant, Newborn; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Iraq; Male; Meconium; Retrospective Studies; Sex Distribution

Topics: Child; Chromosome Mapping; Chromosomes, Human, Pair 19; Cystic Fibrosis; Forced Expiratory Volume; Genetic Markers; Humans; Intestinal Obstruction; Meconium; Microsatellite Repeats; Nuclear Family; Polymorphism, Genetic

Cystic fibrosis (CF) is an autosomal disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). Neonatal meconium ileus (MI) occurs in 10-20 percent of newborns with CF. The purpose of this study was to determine the allelic frequencies of the CF mutation in French patients with and without MI and the incidence of MI in 7 homozygotes or compound heterozygotes for mutation of the CFTR gene. Our study confirms the positive association between delta F508, the most frequent CF mutation, G542X mutation and MI and a negative association with G551D.

Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; France; Humans; Incidence; Infant, Newborn; Intestinal Obstruction; Meconium; Mutation; Registries; Retrospective Studies; Risk Factors

Sixty-eight neonates with functional ileus were reviewed. Twelve required laparotomy; in seven, histological studies revealed decreased ganglia and ganglion cells of the myenteric plexus (MP) (Group A), and in five, MP was normal (Group B). In the remaining 56 cases, obstructive symptoms were relieved following conservative therapy (Group C). All Group A cases except one had normal birth weight, while Group B and C cases showed significantly lower birth weights. A marked caliber change of the small intestine and/or small-caliber distal intestine with meconium stagnation in the proximal intestine was commonly demonstrated at operation in Group A and B, or on contrast enema in Group C. Four Group A cases died of enteritis, and three survivors suffered from prolonged obstructive symptoms. The grade of histological abnormality of MP correlated with the clinical outcome. In Group B, three died of sepsis shortly after surgery, but two survivors have been free from symptoms. Group A can be categorized as Hirschsprung's disease-allied disorders (HAD). Group B and C can be categorized as meconium-related ileus (MRI). The similarity of the macroscopic findings of HAD and MRI, and the occurrence of MRI exclusively in low birth weight neonates, strongly suggest that functional immaturity of MP plays a role in the etiology of MRI.

Topics: Ganglia; Hirschsprung Disease; Humans; Immunohistochemistry; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Myenteric Plexus

Meconium ileus (MI) is a form of neonatal intestinal obstruction due to an abnormal thickened meconium within the terminal ileum. The aim of this retrospective paper is to review our experience with neonates affected from MI treated at our institute over a twenty year period.. This report deals with 23 neonates with MI: 14 newborns had uncomplicated MI due to obstruction of the terminal ileum with meconium pellets, while 9 presented with complications (intestinal atresia, volvulus, pseudocyst, peritonitis).. A water soluble contrast enema (Gastrografin) was attempted in 12 cases with a success rate of 7/12 (58%). The remaining sixteen neonates underwent laparotomy, with 4 treated by resection and primary anastomosis, 7 by enterostomy (chimney or double-barrelled) and 5 managed with T-tube enterostomy. Survival rate was 93% in uncomplicated MI and 67% in complicated forms.. On the basis of personal experience the authors suggest that the treatment of choice for uncomplicated MI is Gastrografin enema, with T-tube enterostomy to be reserved for enema failure. The surgical treatment of the complicated forms depends on the intra-abdominal findings; nowadays chimney or double barrelled enterostomy is to be reserved in cases where peritonitis, late diagnosis, prematurity or associated anomalies complicate the disease.

Topics: Age Factors; Birth Weight; Enema; Female; Gestational Age; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium

It is unclear whether children with cystic fibrosis (CF) who present with neonatal meconium ileus have a different long-term outcome from those presenting later in childhood with pulmonary complications or failure to thrive. We examined a cohort of patients with meconium ileus, and compared their long-term outcome with children who had CF without meconium ileus and neonates who had meconium obstruction without CF (meconium plug syndrome).. Comparative study using retrospective and follow-up interview data.. Group 1 consisted of 35 surviving CF patients who presented with meconium ileus between 1966 and 1992. Two control groups were also studied: 35 age- and sex-matched CF patients without meconium ileus (group 2), and 12 infants presenting with meconium plug syndrome during the same time period (group 3).. Pulmonary, gastrointestinal, nutritional, and functional status were reviewed, and surgical complications were recorded.. Mean follow-up was 12.6 +/- 6, 12.6 +/- 6, and 9. 3 +/- 4 years in groups 1, 2, and 3, respectively. Patients without CF (group 3) demonstrated better growth and functional status, and had a lower incidence of pulmonary and gastrointestinal problems. Although the presence of meconium ileus among CF patients was associated with an earlier diagnosis, there were no significant differences between groups 1 and 2 with respect to hepatobiliary, nutritional, functional, or respiratory status. Meconium ileus was associated with a higher risk of meconium ileus equivalent (20% vs 6%), although this difference was not statistically significant. Long-term surgical complications (adhesive small bowel obstruction and blind loop syndrome) were seen in 27% of children with meconium ileus; there were no long-term surgical complications in groups 2 or 3, because these infants did not have any neonatal surgical procedures. Children presenting with complicated meconium ileus had a higher rate of long-term surgical complications than those with uncomplicated meconium ileus (36% vs 17%), and those managed with resection or enterostomy had more complications than those treated by enterotomy and lavage (33% vs 0%).. Long-term outcome is similar in CF patients who present with meconium ileus and those who do not, except for a slightly higher incidence of meconium ileus equivalent, and a significantly higher rate of surgical complications. The risk of surgical complications is highest in those presenting with complicated meconium ileus and those undergoing resection or enterostomy. Patients with meconium obstruction who do not have CF have an excellent long-term prognosis. This information will be useful in counseling the families of infants presenting with neonatal meconium obstruction.

Topics: Cystic Fibrosis; Female; Follow-Up Studies; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Postoperative Complications; Prognosis; Retrospective Studies

Topics: Adolescent; Alleles; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genotype; Humans; Intestinal Obstruction; Male; Meconium

Meconium ileus (MI) affects 15% of neonates with cystic fibrosis (CF). The authors reviewed the management and outcome of 51 neonates presenting to a single institution between 1976 and 1995 with MI secondary to CF. Clinical presentation included abdominal distension (96%), bilious vomiting (49%), and delayed passage of meconium (36%). A family history of CF was present in 4 cases (8%). Twenty-three neonates presented with MI and evidence of volvulus, atresia, or perforation (complicated MI). Of these, 16 underwent stoma formation, 1 appendicectomy, and 6 resection with primary anastomosis. Twenty-eight neonates presented with uncomplicated MI. Of these, 11 were managed non-operatively by Gastrografin enema (10) or enteral N-acetylcysteine (1). The remainder required stoma formation (15) or bowel resection with primary anastomosis (2). Early postoperative complications occurred in 2 neonates (4%). In this hospital the 1-year survival for this condition has increased from 49% (1953-1970) to 98% (1976-1995) irrespective of the surgical procedure performed or the presence of volvulus, atresia, or perforation. In our experience, bowel resection with primary anastomosis is as safe as stoma formation and is associated with a reduced length of initial hospital stay.

Topics: Anastomosis, Surgical; Cystic Fibrosis; Enterostomy; Female; Follow-Up Studies; Humans; Incidence; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Intraoperative Complications; Laparotomy; London; Male; Meconium; Postoperative Complications; Retrospective Studies; Survival Rate

Topics: Cyanosis; Cystic Fibrosis; Fatal Outcome; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Sulfhemoglobinemia

Topics: Alleles; Amino Acid Substitution; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; DNA Mutational Analysis; Exons; Female; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Mutation, Missense

The variable clinical manifestations of cystic fibrosis (CF) suggest the influence of modifier genes. For example, meconium ileus is present in approximately 10-15% of neonates with cystic fibrosis; however, the genetic and, or environmental factors that determine whether an individual will develop this complication have not been determined. We propose the HFE gene as a candidate modifier locus for CF based on (1) the suggestion of an association between the HLA loci and CF phenotypes; (2) the location of the HFE gene near the HLA loci and; (3) the similarity between the gastrointestinal manifestations of hereditary hemochromatosis and CF. We have determined the frequency of the C282Y and H63D mutations in a group of 89 CF patients who were homozygous for delta F508 and for whom meconium ileus status was known. The carrier frequency of C282Y among the CF patients with meconium ileus was significantly different from that of our unaffected control group (19.4% versus 7.7%). However, the difference between the meconium ileus and the nonmeconium ileus groups was not significant (19.4% versus 10.3%). There was no difference in the frequency of the H63D among the three groups that were studied. These data are suggestive of a relationship between the development of meconium ileus or other gastrointestinal diseases in CF and the HFE gene. Further study of a larger group of patients is warranted.

Topics: Adult; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Epistasis, Genetic; Female; Genetic Predisposition to Disease; Genotype; Hemochromatosis; Humans; Intestinal Obstruction; Male; Meconium; Sequence Deletion

Between May 1994 and June 1995, nine newborns underwent surgery due to mechanical ileus or intrauterine perforation of the small bowel. Three were very-low-birth-weight infants weighing between 520 and 1,200 g. Surgery was performed in the first 2 days of life and split ileo- or jejunostomas were implanted. Early oral nutrition was initiated. To avoid non-use of the distal bowel and short-bowel syndrome, the aboral stoma was irrigated a few days later with the proximal feces. A new technique was applied to transport the chyle continuously from the oral to the aboral stoma: the stool was collected in an especially constructed stoma bag and transported distally by a roller pump. No major complications were seen. The general outcome was excellent in all cases, and reanastomosis under optimal bowel conditions was achieved in all patients without further problems.

Topics: Feces; Humans; Infant, Newborn; Infant, Premature, Diseases; Infant, Very Low Birth Weight; Intestinal Atresia; Intestinal Obstruction; Meconium; Short Bowel Syndrome

Cystic fibrosis is associated with an inspissated bile syndrome producing cholestasis secondary to plugging of macroscopically normal bile ducts. In extreme neonatal forms, with early profound intrahepatic cholestasis, the process can be associated with a marked decrease in ductal diameter, varying from hypoplasia to atresia. From 1990 to 1995 three infants were identified with cystic fibrosis, persistent jaundice, and complete absence of biliary excretion despite expectant and conservative treatment including choleretics and surgical biliary irrigation. Abdominal ultrasounds showed contracted gallbladders and no evidence of dilated ducts. Liver biopsy results in two infants showed portal fibrosis, paucity of bile ducts, and minimal inflammation. The third infant had moderate inflammation, bile duct replication, and plugging. Two infants had undergone intestinal resection followed by hyperalimenation for complications of meconium ileus in the newborn period. Surgical exploration was undertaken at 7 to 12 weeks of age. Gross findings were typical of biliary atresia with microgallbladders and nonpatency of the cystic duct. Cholangiograms failed to document ductal patency in two patients who were then treated with a Kasai portoenterostomy. The third infant had patent hypoplastic ducts and underwent only biliary irrigation. Although bile flow was transiently achieved, jaundice recurred, and at reexploration at 16 weeks of age a Kasai poroenterostomy was performed. Histological review of the biliary specimens showed microscopically patent ducts in two patients and proximal patency and distal atresia in the third. All the ducts had mural fibrosis with cystic changes. Bile drainage was achieved in each instance, although in one patient with hypoplastic ducts scant output of highly concentrated bile proved insufficient to arrest progressive liver failure. The subsequent two patients responded with resolution of hyperbilirubinemia and normalization of liver function. They remain free of biliary complications at 30 and 40 months postoperatively. This manifestation of cystic fibrosis in infants is suggested by prolonged jaundice unresponsive to choleretics, nondilated bile ducts and gallbladder on ultrasound, absent biliary excretion on nuclear scan, and characteristic liver biopsy. Exploration is warranted, and discovery of atrophic bile ducts may be best managed with reconstruction.

Topics: Biliary Atresia; Cystic Fibrosis; Fatal Outcome; Female; Gallbladder; Hepatic Duct, Common; Humans; Infant; Intestinal Obstruction; Jaundice; Male; Meconium; Portoenterostomy, Hepatic

We report the first case of legionella infection in pregnancy complicated by sepsis and hemodynamic compromise. Legionnaires' disease is rarely found in pregnancy, possibly because subacute infections may often be overlooked and empiric therapy of pneumonia in pregnancy may be curative without definitive etiologic diagnosis.

Topics: Adult; Female; Humans; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Legionnaires' Disease; Male; Meconium; Pregnancy; Pregnancy Complications, Infectious; Shock, Septic

Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Survival Analysis; Survival Rate; Treatment Outcome

Fecal alpha 1-antitrypsin is used as a marker for intestinal protein loss reflecting increased intestinal permeability. Exact data of fecal alpha 1-antitrypsin in newborn infants are not available.. 30 healthy mature neonates and three infants with impaired gastrointestinal passage due to stenoses and atresia respectively, were investigated during the first days of life. The amniotic fluid of 13 and the serum of 17 infants was available. alpha 1-antitrypsin was determined using the radial immunodiffusion method.. Normal newborns showed mean fecal alpha 1-antitrypsin levels (+/-SD) of 2061 +/- 817 mg/dl (day 1), 1186 +/- 720 mg/dl (day 2), 308 +/- 380 (day 3), 35 +/- 27 (day 5), and 27 +/- 21 mg/dl (day 6). Two infants with esophageal atresia presented a much lower pattern, and one with annular pancreas had a fecal alpha 1-antitrypsin pattern comparable with that of normal babies. Serum alpha 1-antitrypsin was normal (275 +/- 52 mg/dl), and amniotic fluid contained 20 +/- 12 mg/dl alpha 1-antitrypsin.. The pattern of neonatal fecal alpha 1-antitrypsin content appears to reflect the meconium clearance of the gut rather than intestinal permeability and "gut closure." We hypothesize that the origin of increased fecal alpha 1-antitrypsin is the result of accumulated secretions from bile, the pancreas, and the duodenum, but alpha 1-antitrypsin originating from swallowed amniotic fluid during pregnancy may play an additional role.

Topics: alpha 1-Antitrypsin; Amniotic Fluid; Esophageal Atresia; Feces; Female; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Pancreas

Fifteen cases of meconium ileus (MI) were treated between 1986 and 1995; 7 responded to conservative treatment. Eight were operated upon, and comprise the study group. Six of the operated babies had no complications; 1 had meconium peritonitis with a pseudocyst and small-bowel atresia, and 1 had a volvulus of a small-bowel segment with necrosis. In all 8 cases a T-tube (TT) was left via an enterotomy; in the complicated cases the enterotomy was pre-anastomotic. The obstruction was relieved in all the babies, without any stoma or bowel resection in the uncomplicated cases. Two complications occurred: 1 patient died of respiratory failure 1 month following surgery and another required a relaparotomy for lysis of adhesions. We conclude that TT ileostomy is an effective and safe procedure for uncomplicated cases of MI that do not respond to conservative therapy, as well as for complicated cases that need an anastomosis.

Topics: Cause of Death; Female; Follow-Up Studies; Humans; Ileostomy; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Male; Meconium; Peritonitis; Postoperative Complications; Survival Rate

There is confusion in the radiological literature as to the site of abdominal calcification in cystic fibrosis (CF) with meconium ileus (MI) in neonates.. To correlate the site of radiographic abdominal calcification with histologic and operative findings.. A review of clinical, radiographic, surgical and histologic data in 58 neonates with CF and MI.. Abdominal calcification was identified in 15 (26 %) neonates: on an abdominal radiograph in 8 (13 %), at laparotomy in 3 and histologically in 10 (37 %) of the 27 resected specimens. The radiographic pattern of calcification varied from small specks in three cases to small, better-defined areas in two. In the other three patients, the calcification was more extensive and curvilinear. Histologically, calcification was found to be intramural in ten resected specimens, of which two also had intraluminal and one serosal calcification. The more extensive, curvilinear calcification identified radiographically correlated with histologically proven dystrophic intramural calcification. The less marked flecks or discrete areas of radiographic calcification may represent intramural, serosal or intraluminal calcification.. Intramural calcification is common microscopically in CF with MI. Extensive radiographic calcification in these patients is more likely to represent intramural rather than serosal or intraluminal calcification.

Topics: Calcinosis; Cystic Fibrosis; Female; Humans; Infant, Newborn; Intestinal Obstruction; Intestines; Male; Meconium; Radiography

Meconium peritonitis is an uncommon chemical peritonitis of a fetus resulting from antenatal bowel perforation. We reported a case of meconium peritonitis with pseudocystic formation diagnosed by color Doppler energy (CDE) at 34 gestational weeks. An echogenic substance inside a fetal abdominal mass was detected using ultrasound. By conventional color Doppler, there was minimal blood flow in the cystic wall or septums of the mass. Using CDE, bowel hyperperistalsis was observed in multiple small bowel loops and the region of intestinal loops into the mass was easily detected. Therefore, the angle independent nature of CDE will play a significant role in the early and accurate diagnosis of meconium peritonitis before birth.

Topics: Adult; Female; Fetal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Peritonitis; Pregnancy; Ultrasonography, Doppler, Color; Ultrasonography, Prenatal

Topics: Abdomen; Adult; Cysts; Female; Fetal Diseases; Humans; Ileal Diseases; Intestinal Obstruction; Meconium; Peritonitis; Pregnancy; Ultrasonography, Prenatal

The sonographic finding of hyperechoic or dilated fetal bowel raises suspicion of a number of prenatal disorders including meconium ileus (MI), meconium peritonitis, congenital infection, neoplasm, or chromosomal trisomy. These findings may also represent transient normal variants. The following case report details the evaluation of one pregnancy with abnormal intestinal echogenic findings on serial sonograms (US), to demonstrate inherent diagnostic difficulties in such a case. A diagnostic algorithm is presented to aid in the proper use of US and DNA mutation analysis for cystic fibrosis (CF), so that the cause of an abnormal abdominal US can be established earlier and more accurately than suggested by previous management schemes. Earlier fetal diagnosis may help to anticipate postnatal problems associated with CF/MI, and therefore provide more optimal clinical management of the affected fetus.

Topics: Adult; Algorithms; Cystic Fibrosis; DNA Mutational Analysis; Female; Humans; Intestinal Obstruction; Meconium; Pregnancy; Prenatal Diagnosis; Sensitivity and Specificity; Ultrasonography

Of 36 neonates with meconium ileus secondary to cystic fibrosis treated over a 10-year period, twenty-one (58%) had simple uncomplicated disease while fifteen (42%) had complications which included perforation (5), volvulus (6) and atresia (5). Gastrografin enema was employed in 20 infants with relief of obstruction in 8 (40%). Operative procedures consisted of resection and primary anastomosis in seventeen patients, stomas were fashioned in six, three had an enterotomy with irrigation only and two had Bishop-Koop enterostomy. Post-operative complications developed in 5 (18%) of these 28 patients. The overall survival rate was 97%. The one death occurred in an infant with short bowel syndrome, patent ductus arteriosus, hydrocephalus and pulmonary damage. There were eight additional patients who had meconium obstruction in the absence of cystic fibrosis.

Topics: Cystic Fibrosis; Diatrizoate Meglumine; Enema; Enterostomy; Female; Humans; Ileal Diseases; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Intestinal Perforation; London; Male; Meconium; Retrospective Studies; Survival Rate

We have generated a mouse carrying the human G551D mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR) by a one-step gene targeting procedure. These mutant mice show cystic fibrosis pathology but have a reduced risk of fatal intestinal blockage compared with 'null' mutants, in keeping with the reduced incidence of meconium ileus in G551D patients. The G551D mutant mice show greatly reduced CFTR-related chloride transport, displaying activity intermediate between that of cftr(mlUNC) replacement ('null') and cftr(mlHGU) insertional (residual activity) mutants and equivalent to approximately 4% of wild-type CFTR activity. The long-term survival of these animals should provide an excellent model with which to study cystic fibrosis, and they illustrate the value of mouse models carrying relevant mutations for examining genotype-phenotype correlations.

Topics: Animals; Animals, Newborn; Base Sequence; Chlorides; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; DNA Primers; Electrophysiology; Gene Targeting; Genotype; Humans; Intestinal Obstruction; Ion Transport; Meconium; Mice; Mice, Transgenic; Molecular Sequence Data; Nasal Cavity; Phenotype; Point Mutation

Markedly premature infants may present with intestinal obstruction and perforation secondary to inspissated meconium in the absence of cystic fibrosis. Between 1990 and 1994, 13 patients were treated for intestinal obstruction secondary to inspissated meconium. The average birth weight was 760 g. Prenatal and postnatal risk factors were identified, and included intrauterine growth retardation, maternal hypertension, prolonged administration of tocolytics, patent ductus arteriosus, hyaline membrane disease, and intraventricular hemorrhage. Stooling was absent or infrequent during the first 2 weeks of life. Surgical presentation consisted of distension and/or perforation between days 2 and 17 of life. Twelve patients required operative intervention. Findings invariably included one or more obstructing meconium plugs with proximal distension and frequent necrosis of the dilated segments. Surgical options consisted of resection or enterotomy, accompanied by primary closure or by distal irrigation and exteriorization. Irrigation led to iatrogenic bowel injury in two patients. One patient was managed successfully with oral and rectal gastrograffin and oral acetylcysteine. Ten patients were discharged, all of whom had normal stooling patterns and tested negatively for cystic fibrosis. Three patients died, two from the primary disease. The markedly premature infant is at risk for obstruction and eventual perforation secondary to meconium plugs, presumably formed in conjunction with intestinal dysmotility. Prompt diagnosis and timely intervention require a high index of suspicion, attention to stooling patterns and abdominal examinations, and screening radiographs when indicated.

Topics: California; Humans; Infant, Newborn; Infant, Premature; Infant, Very Low Birth Weight; Intestinal Obstruction; Intestinal Perforation; Meconium; Risk Factors; Survival Rate; Time Factors

Gastrointestinal complications of cystic fibrosis are becoming more common because patients with cystic fibrosis are living longer. There are 227 cystic fibrosis patients in Norway today, almost half of whom are more than 18 years old. Meconiumileus-equivalent is a complication which increases with age. It is a term used to describe partial or complete intestinal obstruction occurring in patients with cystic fibrosis. It results from abnormally viscid mucofaeculent material in the terminal ileum and right proximal colon. Some patients may experience acute complete obstruction, but most of them suffer from chronic partial obstruction, with recurring colicly abdominal pain and some distension. Of 70 adult cystic fibrosis patients at Aker hospital over a seven year period, 26% had symptoms and signs of this disorder. Conservative treatment is preferable, and surgery should be avoided.

Topics: Abdomen, Acute; Adult; Cystic Fibrosis; Female; Humans; Intestinal Obstruction; Male; Meconium; Radiography

Failure of a small premature newborn to adequately evacuate meconium for days or weeks has been attributed to "probable necrotizing enterocolitis (NEC)" or "microcolon of prematurity." The authors present an unusual type of "meconium plug syndrome" with the same clinical picture, seen in tiny premature babies (500 to 1,500 g), which required a contrast enema or Gastrografin upper gastrointestinal (UGI) series to evacuate the plugs. The obstruction resolved. Twenty babies (480 to 1,500 g) presented with the same clinical picture without any x-ray suggestion of NEC; contrast enemas were performed because of the suspicion of meconium plug syndrome. All 20 had extensive meconium plugs that were evacuated by the enema or by a Gastrografin UGI series. Most of them improved after the plugs were passed. These infants differ from typical full-term babies with meconium plug syndrome in a number of ways: (1) many of the mothers were on magnesium sulfate (MgSO4) or had eclampsia; (2) the plugs were diagnosed late rather than shortly after birth; and (3) the plugs were significant, extending to the right colon. The authors believe that when a tiny premature baby has findings consistent with meconium plug syndrome, the baby should be transported to radiology, for a Gastrografin enema, despite the difficulties involved. Delay postpones the start of feedings, and increases the number of radiographic studies.

Topics: Contrast Media; Diagnosis, Differential; Diatrizoate Meglumine; Enterocolitis, Pseudomembranous; Female; Humans; Infant, Newborn; Infant, Premature, Diseases; Infant, Very Low Birth Weight; Intestinal Obstruction; Meconium; Pregnancy; Pregnancy Complications

Cystic fibrosis (CF), the most common lethal autosomal recessive disease in white populations, is characterized by dysfunctional chloride ion transport across epithelial surfaces. Although recurrent pulmonary infections and pulmonary insufficiency are the principal causes of morbidity and death, gastrointestinal symptoms commonly precede the pulmonary findings and may suggest the diagnosis in infants and young children. The protean gastrointestinal manifestations of CF result primarily from abnormally viscous luminal secretions within hollow viscera and the ducts of solid organs. Bowel obstruction may be present at birth due to meconium ileus or meconium plug syndrome. Complications of meconium ileus include volvulus, small bowel atresia, perforation, and meconium peritonitis with abdominal calcifications. Older children with CF may present with bowel obstruction due to distal intestinal obstruction syndrome or colonic stricture, and tenacious intestinal residue may serve as a lead point for intussusception or cause recurrent rectal prolapse. Radiologic studies often demonstrate thickened intestinal mucosal folds in older children and uncommonly show colonic pneumatosis, peptic esophageal stricture due to gastroesophageal reflux, and duodenal ulcer. Appendicitis due to inspissated secretions is uncommon. Obstruction of ducts and ductules produces exocrine pancreatic insufficiency, pancreatitis, cholestasis, cholelithiasis, and cirrhosis with portal hypertension. On imaging studies, the pancreas is commonly small and largely replaced by fat, sometimes displays calcifications, and is rarely replaced by macrocysts. Radiologic features of hepatobiliary disease include an enlarged radiolucent liver from steatosis, gallstones, a shrunken nodular liver, splenomegaly, and portosystemic collateral vessels. With the improved survival of CF patients, an increased risk for developing gastrointestinal carcinomas has been established, many occurring as early as the 3rd decade.

Topics: Adult; Biliary Tract Diseases; Child; Cystic Fibrosis; Digestive System Diseases; Gastrointestinal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Liver Diseases; Meconium; Pancreatic Diseases; Tomography, X-Ray Computed; Ultrasonography

Topics: Child, Preschool; Colon; Cystic Fibrosis; Female; Humans; Ileum; Intestinal Obstruction; Meconium; Ultrasonography

Neuroblastoma, Hirschsprung's disease, and central hypoventilation (Ondine's curse) are considered aberrations of neural crest cell growth, migration, or differentiation, and as such are considered to be under the general heading of neurocristopathy. Their combined occurrence in a newborn infant presenting with total colonic aganglionosis, central hypoventilation, and multifocal neuroblastoma had not been reported previously. A 2.3-kg white full-term girl required endotracheal intubation because of persistent apnea in the first hours of life. She had progressive abdominal distension and failure to pass meconium; a barium enema was performed, which showed microcolon with meconium pellets at the distal ileum. During laparotomy the distal ileum was found to be obstructed with inspissated meconium; an ileostomy and appendectomy were performed. The resected specimens were aganglionic. An additional 20 cm of aganglionic ileum was removed, and a normally innervated ileostomy was constructed. Numerous attempts at extubation failed because of apnea. The results of an extensive apnea workup, including electroencephalogram, magnetic resonance imaging (MRI), bronchoscopy, and pH probe study, were normal. Sleep studies showed congenital central hypoventilation syndrome, and the patient underwent a tracheostomy. At 3 months, an abdominal ultrasound examination performed within a septic workup showed a right suprarenal mass extending across the midline. Thoracic and abdominal MRI scans showed large bilateral adrenal and posterior mediastinal masses. The serum catecholamines and ferritin level were markedly elevated, suggestive of neuroblastoma. In light of the child's multiple problems, the family chose to forgo further workup (including a tissue biopsy) and therapy. In the following 2 months her tumor load rapidly progressed, and she died of respiratory insufficiency.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adrenal Gland Neoplasms; Colonic Diseases; Female; Follow-Up Studies; Hirschsprung Disease; Humans; Ileal Diseases; Infant, Newborn; Intestinal Obstruction; Meconium; Mediastinal Neoplasms; Neural Crest; Neuroblastoma; Sleep Apnea Syndromes; Tracheostomy

To determine the practice pattern regarding nonoperative treatment of simple meconium ileus, a survey was sent to directors of 66 pediatric radiology departments in the USA and Canada. Thirty-nine responses were received reporting 1,236 patients. A wide variation of enema techniques and contrast media were used. While the success rate did not correlate with osmolality, mode of administration, catheter size, or perforation rate, there was a significantly higher overall success rate with the use of Gastrografin versus non-Gastrografin (p < 0.00076) and the use of additives such as Tween-80 and Mucomyst versus techniques without additives (p < 0.00001). Perforation did not correlate with success rate, osmolality, or type of contrast medium. The only two instances of rectal perforations were associated with the use of balloon-tip catheter.

Topics: Canada; Contrast Media; Data Collection; Diatrizoate Meglumine; Enema; Humans; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Meconium; Practice Patterns, Physicians'; Radiology; Societies, Medical; United States

In nine patients with meconium peritonitis prenatal ultrasonographic findings were correlated with the clinical course and outcome. Ultrasound findings included polyhydramnion (n = 4), ascites (n = 4), disseminated (n = 3) and solitary echogenic areas (n = 1), echopoor cystic areas (n = 3) and echogenic-echopoor solitary areas (n = 1). Intra-abdominal calcifications were found in five patients before delivery. Eight neonates survived and were subsequently followed up, one fetus died in utero. Four of the eight survivors required surgery, namely for meconium ileus (n = 1), perforation secondary to intestinal volvulus (n = 2) and inguinal hernia associated with prenatal rubella infection (n = 1). Three patients were healthy, one patient required drainage of pleural effusion and respirator therapy but recovered without further problems. Cystic fibrosis was diagnosed in the patient with meconium ileus. Postnatal outcome could not be predicted from the prenatal sonographic findings.

Topics: Adult; Calcinosis; Cesarean Section; Cystic Fibrosis; Female; Follow-Up Studies; Gestational Age; Humans; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Polyhydramnios; Pregnancy; Ultrasonography, Prenatal

Meconium ileus is the earliest clinical manifestation of cystic fibrosis. We report 22 neonates with meconium ileus who had clinical evidence of cystic fibrosis. Patients were categorized as simple with inspissated meconium in the ileum with dilated loops proximally or complicated with volvulus or atresia and/or a perforation resulting in meconium peritonitis. Histopathology of the surgically resected specimens of small bowel revealed lesions typical of cystic fibrosis. Genetic studies were performed on all subjects, this study analyzes the usefulness in the detection of delta F508 mutation in formalin-fixed paraffin-embedded tissues obtained from patients with meconium ileus, ten of whom had the delta F508/delta F508 mutation.

Topics: Cystic Fibrosis; Female; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Mutation; Polymerase Chain Reaction; Retrospective Studies

Topics: Adolescent; Child; Child, Preschool; Comorbidity; Cystic Fibrosis; Female; Humans; Incidence; Intestinal Obstruction; Liver Diseases; Liver Function Tests; Male; Meconium; Prevalence; Sweden

The most common aetiology of meconium ileus is a deficiency in trypsin activity caused by cystic fibrosis. The pathogenesis of meconium ileus without mucoviscidosis is less well understood, although a number of causative factors have been suggested. The symptoms and clinical course of nine patients with meconium ileus without mucoviscidosis were reviewed, and the myenteric plexus of a surgical specimen of intestine was examined histologically and cytometrically. The nuclei of the intramural ganglion cells were much smaller than were seen in normal newborn infants. The nuclear areas resembled those seen in fetuses of 5-6 months gestational age, but the number of ganglion cells approached normal. This immaturity of the ganglia was observed both in the contracted distal ileum and dilated proximal ileum. Patients with an ileostomy passed solid faeces for about 1 to 2 months postoperatively, after which time the faeces became watery. The intramural ganglia were mature at the time of ileostomy closure. We conclude that immaturity of the myenteric plexus in the ileum and colon seems to be the main aetiologic factor in meconium ileus without mucoviscidosis.

Topics: Cytodiagnosis; Female; Gestational Age; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Myenteric Plexus

Topics: Adult; Cystic Fibrosis; Female; Fetal Diseases; Gestational Age; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Peritonitis; Polyhydramnios; Pregnancy; Ultrasonography, Prenatal

We have observed five children with cystic fibrosis, who presented over 2 months, with meconium ileus equivalent that failed to respond to medical management. At surgery, four had a stricture in the ascending colon, and all had histopathological changes of post-ischaemic ulceration repair, with mucosal and submucosal fibrosis. The only common change in the management of these children was a switch from conventional enteric-coated pancreatic enzymes to high-strength products 12-15 months before presentation.

Topics: Abdominal Pain; Adolescent; Child; Child, Preschool; Colon; Colonic Diseases; Constriction, Pathologic; Cystic Fibrosis; Fibrosis; Humans; Intestinal Obstruction; Lipase; Male; Meconium; Pancreatic Extracts; Pancreatin; Pancrelipase

Recently we have created a mouse model of cystic fibrosis (CF) by insertional gene targeting to exon 10. In common with CF subjects, this model displays a low incidence of meconium ileus. This contrasts strikingly with the very high level of fatal intestinal obstruction in the three other CF mouse models so far described. We investigate here the molecular basis of this difference in phenotype. We show that the partial duplication consequent upon insertional gene targeting allows exon skipping and aberrant splicing to produce normal Cftr mRNA, but at levels greatly reduced compared with wild-type mice. Furthermore, instead of the predicted mutant Cftr transcript, a novel mRNA is produced that utilizes cryptic splice sites in the disrupting plasmid sequence. However, we have previously shown that these mice display the ion transport defect characteristic of CF, and mutant animals can be distinguished from their normal littermates on this basis. Consistent with this, residual CFTR function has recently been observed for several "mild" mutations in CF individuals who display pancreatic sufficiency but still develop lung disease. We conclude that (i) residual wild-type mRNA in the exon 10 insertional mutant mouse ameliorates the severity of the intestinal phenotype observed in the absolute "null" CF mice, (ii) the presence of low-level residual wild-type Cftr mRNA does not correct the CF ion transport defect, and (iii) the long-term survival of this insertional mutant mouse provides the opportunity to address the factors important in development of lung disease.

Topics: Animals; Animals, Newborn; Base Sequence; Cloning, Molecular; Cystic Fibrosis; Disease Models, Animal; DNA, Complementary; Exons; Gene Expression; Intestinal Obstruction; Lung; Meconium; Mice; Mice, Mutant Strains; Molecular Sequence Data; Mutagenesis, Insertional; Polymerase Chain Reaction; RNA, Messenger

Meconium ileus, rare in Malaysia, accounts for 3.7% of all neonatal intestinal obstructions (excluding imperforate anus) seen in the University Hospital, Kuala Lumpur, from 1980-1990. This paper retrospectively reviews our clinical experience with 5 cases of meconium ileus seen over a 12-year period from 1980-1991 in the University Hospital, Kuala Lumpur. Three of the neonates were Malays, and two were Punjabis. Four of them were full-term and one preterm. The birth weights ranged from 1900 to 3700 g, with a mean of 2670 g. One of them also had a sibling with meconium ileus. Two of them were found to have foetal ascites and one had intestinal obstruction, antenatally by ultrasonography. The remaining two were symptomatic soon after birth. Extensive calcification was observed on plain abdominal radiographs in three babies and dilated bowels in the other two. All of them underwent laparotomy and uncomplicated meconium ileus was confirmed in two cases, meconium peritonitis in two and one meconium pseudocyst in addition to meconium peritonitis. There was one intraoperative death, and one long-term survivor who did not have cystic fibrosis. The remaining three did not have additional features suggestive of cystic fibrosis, and finally succumbed to respiratory infection. Sweat test was not done to confirm the diagnosis for logistic reasons. The management of such patients proved to be a challenge to clinicians because of the rarity of this condition.

Topics: Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Malaysia; Male; Meconium; Survival Rate; Tomography, X-Ray Computed

Seven full-term infants with aganglionosis extending into the small bowel presented with clinical, radiological, and operative features of meconium ileus. Misdiagnosis resulted in inappropriate treatment. The correct diagnosis was eventually established by rectal suction biopsy, mostly after either recurrent intestinal obstruction or stomal dysfunction, and after cystic fibrosis had been excluded. For two patients, the results of rectal suction biopsies were initially misleading. Two infants died. Extensive intestinal aganglionosis should be considered a rare possibility in all infants with meconium ileus. In such cases, histological examination of the appendix may avoid this potential pitfall.

Topics: Biopsy, Needle; Female; Hirschsprung Disease; Humans; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Male; Meconium; Rectum

We prospectively screened for liver disease patients with cystic fibrosis who were more than 3 years of age and who were followed at the cystic fibrosis center of the University of Milan. From January 1991 to December 1992, we screened 189 patients; clinical, biochemical, and echographic abnormalities suggestive of overt liver disease were present in 34 (18%). To define risk factors for the development of liver disease associated with cystic fibrosis, we evaluated the possible role of specific mutations of the CFTR (cystic fibrosis transmembrane regulator) gene and of different clinical and demographic characteristics (sex, pancreatic status, meconium ileus or its equivalent) through a comparison of patients with cystic fibrosis and overt liver disease (n = 34) and those without liver disease (n = 155). Genetic analysis failed to reveal any significant difference in the allele frequencies of defined (delta F508, 1717-1G-A, G542X, N1303K, W1282X, R553X) and undefined mutations of the CFTR gene in the two groups of patients; genotype frequencies were also not significantly different. Pancreatic insufficiency was present in all patients with liver disease and in 87.3% of those without liver disease. A male predominance was found in the group with liver disease. The frequency of meconium ileus or its equivalent was significantly higher in patients with cystic fibrosis and liver disease (35.3%) than in patients without liver disease (12.3%) (p = 0.0025). In the 31 patients with a history of meconium ileus or its equivalent, the following hepatic abnormalities occurred more frequently than in the 155 patients with cystic fibrosis who did not have meconium ileus: hepatomegaly, biochemical abnormalities, heterogeneous echographic pattern of the liver, and microgallbladder. Twenty-four patients with a history of meconium ileus or its equivalent underwent hepatobiliary scintigraphy (with technetium-labeled iminodiacetic acid derivatives), which showed morphologic abnormalities suggestive of impaired biliary drainage in 21 patients and abnormalities in function in 11. The risk of acquiring liver disease was increased almost fourfold in patients with a history of meconium ileus or its equivalent, in comparison with patients who had cystic fibrosis but were unaffected by these complications (odds ratio, 3.9043; 95% confidence interval, 1.666 to 9.149). We conclude that patients with cystic fibrosis and meconium ileus or its equivalent may benefit from prophylactic tre

Topics: Adolescent; Child; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; Female; Genotype; Humans; Intestinal Obstruction; Liver Diseases; Male; Meconium; Mutation; Prospective Studies; Risk Factors; Sex Factors

Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Infant; Intestinal Obstruction; Male; Meconium; Risk Factors

Topics: Celiac Disease; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Exocrine Pancreatic Insufficiency; Humans; Infant, Newborn; Intestinal Obstruction; Lung; Male; Meconium; Membrane Proteins; Phenotype; Point Mutation

Cystic fibrosis is most often the underlying cause of meconium ileus. We describe the diagnosis and treatment of a patient with chronic intestinal pseudo-obstruction, and not with cystic fibrosis, whose initial manifestation was meconium ileus.

Topics: Chronic Disease; Humans; Infant, Newborn; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Male; Meconium

Topics: Asian People; Humans; Ileum; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium

The glycine-to-aspartic acid missense mutation at codon 551 (G551D), which is within the first nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator (CFTR), is the third most common cystic fibrosis (CF) mutation, with a worldwide frequency of 3.1% among CF chromosomes. Regions with a high frequency correspond to areas with large populations of Celtic descent. To determine whether G551D confers a different phenotype than does delta F508, the most common CF mutation, we studied 79 compound heterozygotes for G551D/delta F508, from nine centers in Europe and North America. Each subject was matched, by age and sex, with a delta F508 homozygote from the same center. A retrospective cohort analysis was performed on the following outcome parameters: age at diagnosis, sweat chloride, meconium ileus at birth, height, weight, weight for height, FVC, FEV1, chest X-ray score, pseudomonas colonization, pancreatic sufficiency, and Shwachman clinical score. There was less meconium ileus among the G551D/delta F508 compound heterozygotes (relative risk 0.33; 95% confidence interval .13-.86), as well as a trend toward later age at diagnosis of pancreatic insufficiency. No statistically significant difference was found between the groups for any other parameter. These results suggest that the CF genotype can be a predictor of pancreatic and intestinal phenotype. Prenatal counseling for the two genotype groups should differ only with respect to probability of meconium ileus. Clinical outcome (after survival of meconium ileus) for G551D/delta F508 compound heterozygotes and delta F508 homozygotes is indistinguishable; therefore, prognostic counseling should not differ.

Topics: Adolescent; Aspartic Acid; Child; Child, Preschool; Codon; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Glycine; Homozygote; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Membrane Proteins; Mutation; Risk Factors

Cystic fibrosis results from defects in the gene encoding a cyclic adenosine monophosphate-dependent chloride ion channel known as the cystic fibrosis transmembrane conductance regulator (CFTR). To create an animal model for cystic fibrosis, mice were generated from embryonic stem cells in which the CFTR gene was disrupted by gene targeting. Mice homozygous for the disrupted gene display many features common to young human cystic fibrosis patients, including failure to thrive, meconium ileus, alteration of mucous and serous glands, and obstruction of glandlike structures with inspissated eosinophilic material. Death resulting from intestinal obstruction usually occurs before 40 days of age.

Topics: Animals; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Digestive System; Disease Models, Animal; Exocrine Glands; Gallbladder; Genitalia, Male; Genotype; Growth; Intestinal Obstruction; Liver; Male; Meconium; Membrane Proteins; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Mucus; Mutagenesis; Pancreas; RNA, Messenger; Salivary Glands

Neonatal gastrointestinal perforation has been associated with mortality rates of 40% to 70%. Over the past 20 years, 81 infants (46 boys and 35 girls) were treated for a gastrointestinal perforation at this institution. Perforation occurred from birth to 50 days (average, 8.2 days). Etiologies included necrotizing enterocolitis (NEC) (68%), meconium ileus (10%), and idiopathic gastric perforation (7%). Seventy-six infants underwent surgical exploration and five infants, considered too small or too sick to withstand a laparotomy, were treated with peritoneal lavage only. There were 29 deaths, an overall mortality of 36%. Ninety percent of the death occurred in patients with NEC, while all patients with gastric perforations survived. There has not been a significant improvement in survival in recent years, partly because of an increase in the proportion of NEC-related perforations. However, there is a narrowing of the mortality gap between low birth weight and normal weight infants. As the risk inherent to laparotomy in neonates is decreasing, other factors, such as the underlying etiology or the site of perforation, play a more important prognostic role.

Topics: Enterocolitis, Pseudomembranous; Female; Follow-Up Studies; Humans; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Intestinal Perforation; Male; Meconium; Retrospective Studies; Stomach Rupture; Survival Rate

Between January 1985 and May 1990, 16 neonates were treated for meconium ileus (MI) at this hospital. All babies were born to Chinese couples. Seven of them were premature, but none of them weighed less than 1,000 g. Eight patients underwent operations either because of mistaken diagnosis, as ileal atresia or long-segment Hirschsprung's disease, or because of complicated MI, including two meconium peritonitis and one associated with ileal atresia. Gastrograffin enema was successful in management of eight uncomplicated MI. The albumin content in the meconium of the last nine cases, including four complicated cases, ranged from 9.2 to 93.3 mg/g dry meconium. Usually, albumin is not present in normal meconium. All cases received sweat test, which were negative. Three patients died in the follow-up period. Sepsis of unknown origin, multiple congenital anomalies, and severe metabolic problems were the causes of death. The other 13 patients are doing well. They have exhibited no pulmonary or digestive problems during their follow-up period, which ranged from 11 months to 5 years. They are healthy and receive regular diets. Growth and development are appropriate for their age groups.

Topics: Albumins; Asian People; China; Diatrizoate Meglumine; Enema; Female; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Peritonitis

Although meconium ileus in the absence of cystic fibrosis is considered a rare event, it was found that eight of 37 (21.6%) newborn infants with meconium ileus had no laboratory or clinical evidence of cystic fibrosis.

Topics: Female; Humans; Ileal Diseases; Infant, Newborn; Infant, Premature; Intestinal Obstruction; Male; Meconium

Cystic fibrosis patients born with meconium ileus (MI) have had an improved outcome over the last three decades. The authors reviewed the impact of surgical management and long-term nutritional care on the survival of patients with MI. Of the 59 cases of MI seen from 1959 to 1989, 48 cases were managed operatively using either the Bishop-Koop ileostomy (BK), the Mikulicz ileostomy, primary resection and anastomosis (RA), or ileostomy. Six-month survival of MI has improved from 37% to 100%. Nonoperative cases (n = 11) had 100% long-term survival. The RA survivors required less late operative intervention (20%) as compared with other surgical patients (81%). A comparison of serial growth percentiles of CF patients with MI with those of their non-MI CF peers showed similar long-term decreases. These data confirm: (1) There is an improved survival for MI independent of the surgical procedure; (2) The BK ileostomy is an effective and time-tested MI treatment; (3) Primary resection and anastomosis in selected cases may have a lower surgical morbidity rate; and (4) Meconium ileus does not adversely affect the long-term nutritional outcome of CF patients.

Topics: Chi-Square Distribution; Cystic Fibrosis; Female; Humans; Ileal Diseases; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Nutritional Status; Retrospective Studies; Survival Analysis; Treatment Outcome

This article reviews the management and mortality of 53 neonates with meconium ileus and cystic fibrosis treated in one paediatric surgical centre from 1972 to 1990. Forty-four patients (83 per cent) presented with simple meconium ileus and 26 of 40 patients in this group were successfully treated with Gastrografin enemas. Four patients suffered a perforation as a result of the enema (perforation rate 5 per cent). The 1-year survival rate for simple meconium ileus was 81 per cent and for complicated meconium ileus 75 per cent.

Topics: Cystic Fibrosis; Enema; Female; Humans; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Male; Meconium

Topics: Abdominal Pain; Adolescent; Cystic Fibrosis; Female; Humans; Intestinal Obstruction; Meconium; Radiography; Syndrome

During 13 years, 47 infants were treated for mechanical ileus resulting from intestinal obstruction located proximally to the coecum (duodenal obstruction, malrotation, jejuno-ileal obstruction and meconium ileus). The mortality and morbidity were low. A total of three infants died; all of them in the immediate postoperative period. One infant died on account of rupture of the anastomosis; in two infants, death was caused by pulmonary complications and disseminated intravasal coagulation. Seven reoperations were performed and among these five infants on account of adhesions-/fibrous band ileus. At follow-up examination five infants were slightly underweight and retarded in growth; only one child had troublesome gastrointestinal problems. We recommend the use of antenatal ultrasound in the 30th week of gestation more frequently and always when polyhydramnios is present. This should raise the suspicion of gastrointestinal obstruction. Postnatal screening for associated congenital anomalies should also be done, since these are the most frequent cause of death in this patient category.

Topics: Duodenal Obstruction; Female; Follow-Up Studies; Humans; Ileal Diseases; Infant; Infant, Newborn; Intestinal Obstruction; Jejunal Diseases; Male; Meconium; Prognosis

Kawasaki syndrome appeared after operation for meconium ileus equivalent in a 4-year-old child with cystic fibrosis. The course and management are described.

Topics: Child, Preschool; Cystic Fibrosis; Female; Humans; Intestinal Obstruction; Meconium; Mucocutaneous Lymph Node Syndrome; Postoperative Complications

Abdominal plain films of 133 neonates, with 82 cases of meconium plug syndrome (MPS), 27 cases of meconium ileus (MI) and 24 cases of neonatal Hirschsprung's disease (HD), were reviewed to assess the value of such radiographs for diagnosis. The radiographs were examined according to a list of 11 parameters. By using multivariate discriminant analysis, it appeared that 4 parameters i.e. dilatation of bowel loops, varying loop calibre, fluid levels and colonic gas were most important in discriminating among the three disorders. For each parameter the weight (in points) was derived. To classify patients, three group-scores had to be calculated: the group-score with the largest value indicated the most likely disorder. So in 99%, 88% and 63% of MPS, HD and MI, respectively, an accurate diagnosis could be predicted. The overall diagnostic accuracy was 89%. Such a diagnosis can be a sound basis for further investigation.

Topics: Dilatation, Pathologic; Female; Hirschsprung Disease; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Radiography; Syndrome

Topics: Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Trypsinogen

Topics: Amiloride; Chlorides; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Absorption; Intestinal Mucosa; Intestinal Obstruction; Meconium; Membrane Potentials; Nasal Mucosa; Rectum; Sweat

Topics: Cystic Fibrosis; Female; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Parenteral Nutrition; Peritonitis; Radiography

Topics: Anastomosis, Surgical; Female; Hirschsprung Disease; Humans; Ileal Diseases; Ileostomy; Infant, Newborn; Intestinal Obstruction; Intestines; Meconium; Myenteric Plexus; Postoperative Complications; Reoperation

Topics: Acetylcysteine; Female; Humans; Hypernatremia; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Meconium

CF heterogeneity has been evidenced from both clinical and genetic observations. At least two clinical forms of CF are easily distinguishable: CF with meconium ileus and CF without meconium ileus. The results of prenatal diagnosis have shown that the recurrence rates of CF are different in these two clinical forms. Molecular analysis of Restriction Fragment Length Polymorphisms (RFLPs) tightly linked to the cystic fibrosis (CF) gene defined several types of CF and normal chromosomes in a French sample of 64 families with CF. The CF mutation is tightly linked to one XV-2C and KM19 RFLPs haplotype but is differently linked to J3.11 RFLP alleles, depending on whether or not the clinical form of CF is associated with ileus. A distortion of the segregation ratio observed between normal and CF haplotypes in the families with ileus could explain the high recurrence rate of CF in such families.

Topics: Alleles; Cystic Fibrosis; Gene Frequency; Genetic Variation; Haplotypes; Heterozygote; Homozygote; Humans; Intestinal Obstruction; Meconium; Mutation; Pedigree; Polymorphism, Restriction Fragment Length; Prospective Studies

Echogenicity of the fetal bowel seen sonographically can vary at different gestational ages. Increased echogenicity of small bowel loops is seen commonly in the second trimester. We encountered a case of increased bowel echogenicity with shadowing in the third trimester in a fetus who had meconium ileus associated with cystic fibrosis. Other reports also indicate that a persistent echogenic appearance of fetal small bowel in the third trimester, particularly with shadowing, is worrisome and can be associated with meconium ileus.

Topics: Adult; Cystic Fibrosis; Female; Fetal Diseases; Humans; Intestinal Obstruction; Meconium; Pregnancy; Pregnancy Trimester, Third; Ultrasonography

A case of meconium peritonitis due to meconium ileus is described. The condition was detected antenatally as fetal ascites on ultrasonography. Plain X-ray of abdomen post-natally showed specks of calcification mainly at the flanks, while on ultrasonography specks of high echogenic areas were seen throughout the abdomen which has been described as "snow-storm sign". The aetiology of meconium ileus is briefly discussed.

Topics: Adult; Ascites; Diagnosis, Differential; Female; Fetal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Peritonitis; Pregnancy; Prenatal Diagnosis; Ultrasonography

Topics: Adult; Cesarean Section; Cystic Fibrosis; Female; Fetal Distress; Humans; Infant, Newborn; Infant, Premature; Intestinal Obstruction; Intestine, Small; Meconium; Pregnancy; Prenatal Diagnosis; Ultrasonography

We set out to determine if the clinical course or genetic profiles of patients with cystic fibrosis who had meconium ileus differed from those of other patients with cystic fibrosis. Since 1950 we have followed 158 patients with meconium ileus among 1175 patients with cystic fibrosis (13.4%). Patients with meconium ileus had lower birth weight (3026 +/- 610 gm) than patients with no meconium ileus (3169 +/- 534 gm; p less than 0.008); the deficit was especially evident in female patients. Survival in the first year of life increased from 55% in those born between 1958 and 1972 to 96% in those born between 1973 and 1987. Since 1973 the median survival of male and female patients with meconium ileus was similar to that in female patients with no meconium ileus (21 years), whereas 78% of males with no meconium ileus survived to this age (p less than 0.0001). Patients with meconium ileus born before 1972 had lower weight and height percentiles at age 13 years compared with patients with no meconium ileus, but this difference was not as apparent in patients born after 1973. There were no differences between the two groups in forced vital capacity, forced expiratory volume in 1 second, or forced expiratory flow in the middle half of forced vital capacity. Patients with meconium ileus acquired Pseudomonas aeruginosa at a younger age than did patients with no meconium ileus (4.20 +/- 4.67 vs 7.18 +/- 5.19 years), but there was no difference in age of acquisition of P. cepacia. In families in which the first child had meconium ileus, 29% of subsequent siblings with cystic fibrosis had meconium ileus, compared with 6% of siblings born to families in which the first child did not have meconium ileus. Allelic frequencies and haplotypic variants for cystic fibrosis chromosomes with respect to DNA markers closely linked to the cystic fibrosis locus were similar in families with cystic fibrosis with meconium ileus and those with no meconium ileus. These findings suggest that patients with cystic fibrosis and those without meconium ileus do not have major intrinsic differences and that the previously poor outlook in patients with meconium ileus has improved greatly.

Topics: Adolescent; Alleles; Anthropometry; Birth Weight; Child; Cystic Fibrosis; Female; Gene Frequency; Genetic Markers; Haplotypes; Humans; Infant, Newborn; Intestinal Obstruction; Longitudinal Studies; Male; Meconium; Prognosis

This report describes 51 neonates with meconium ileus and emphasizes a changing pattern of treatment and improved survival. Twenty-four neonates had uncomplicated meconium ileus due to inspissated meconium obstructing the distal ileum. Twenty-seven neonates had 41 complications of meconium ileus including volvulus (18), bowel atresia (13), perforation (5), and giant cystic meconium peritonitis (5). Nine patients with uncomplicated cases responded to nonoperative clearing of meconium using a meglumine diatrizoate (Gastrografin) enema. Six of 7 patients with enema failures underwent laparotomy, purse-string enterotomy, and intraluminal irrigation. The remaining 9 patients with uncomplicated meconium ileus had resection and enterostomy. Complicated cases were managed by resection and anastomosis (13) or enterostomy (14). Survival at 1 year was 92% in patients with uncomplicated meconium ileus and 85% for those with complicated meconium ileus. Nonoperative Gastrografin enema or enterotomy-irrigation can relieve obstruction in uncomplicated meconium ileus and avoid an enterostomy in most cases.

Topics: Cystic Fibrosis; Female; Humans; Ileal Diseases; Infant, Newborn; Intestinal Obstruction; Male; Meconium

Although dehydrated obstructing mucus is thought to account for the obstructive pathology involving the lungs, the pancreas, the reproductive system, and the intestinal tract, its relationship with CF-associated liver disease remains largely hypothetical and little is known about possible risk factors. Complete clinical and autopsy records were available in 38 of 73 deaths occurring over a 10-year period. The liver was normal in only five cases, and they were all infants. Steatosis was the only lesion present in 9, hypoxic liver disease was documented in 8, and biliary cirrhosis in 16 (focal in 10 and multilobular in 6). There was no relationship between the presence of cirrhosis, gallbladder abnormalities, age at death, and clinical status recorded during the year precoding their demise. Mucus plugs characterized by amorphous eosinophilic material within proliferated bile ductules were present in 75% of children with focal or multilobular biliary cirrhosis as opposed to 14% in those without (p = 0.015). A history of meconium ileus or its equivalent was recorded more frequently (p = 0.038) in those with cirrhosis. Finally, biliary cirrhosis was invariably present when there was a history of meconium ileus or its equivalent and when mucus plugs were noted. These findings suggest that patients with intestinal obstruction are at greater risk for the development of cirrhosis and that strategies should be developed to increase the detergent capacity of bile and its flow in order to decrease the viscosity of mucus in the biliary tree.

Topics: Bile; Child; Cystic Fibrosis; Female; Humans; Infant, Newborn; Intestinal Obstruction; Liver Cirrhosis, Biliary; Male; Meconium; Retrospective Studies; Risk Factors

Three neonates with meconium ileus who failed to respond to non-operative measures were successfully treated by appendicectomy and irrigation with Gastrografin into the small bowel via the appendix stump. The meconium was emptied out and the stump ligated. This method avoids enterotomy, enterostomies or resection of bowel. The post-operative course is simplified and hospital stay decreased. Removal of the appendix precludes future disease of this organ which may be problematic in patients with cystic fibrosis.

Topics: Appendectomy; Appendix; Combined Modality Therapy; Diatrizoate Meglumine; Female; Humans; Ileal Diseases; Infant, Newborn; Intestinal Obstruction; Intubation, Gastrointestinal; Male; Meconium; Therapeutic Irrigation

41 families with cystic fibrosis, (CF) were tested for restriction fragment length polymorphisms (RFLPs) detected by four DNA probes all of which are tightly linked to the CF gene. 17 of the families had an affected child with, and 24 had one without, meconium ileus. In all cases, CF segregates with these gene probes; however, those with and those without meconium ileus differed in haplotype for CF chromosomes with respect to pJ3.11, which suggests that because of multiallelism (different mutations of the same locus) some CF patients present with meconium ileus and others do not.

Topics: Alleles; Cystic Fibrosis; Haplotypes; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Polymorphism, Restriction Fragment Length

Four of ten fetuses carrying a risk of 1:4 for cystic fibrosis were found to have low levels of microvillar enzymes in the amniotic fluid obtained between 17 and 18 weeks' gestational age. On sonography performed prior to the amniocentesis, three fetuses showed enlarged bowel loops. At autopsy, meconium ileus was detected. Enlarged bowel loops are a sign which has not been described previously so early in pregnancies.

Topics: Cystic Fibrosis; Female; Fetal Diseases; Humans; Intestinal Obstruction; Meconium; Pregnancy; Prenatal Diagnosis; Ultrasonography

Topics: Female; Fetal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Pregnancy; Prenatal Diagnosis; Ultrasonography

Meconium ileus (MEC), failure to thrive (FTT), and a combination of FTT and pulmonary symptoms (COMB) are the most frequent symptoms of cystic fibrosis (CF) at the time of diagnosis. The purpose of this study was to compare to normal controls (NC) the pulmonary function of CF infants at the time of diagnosis, when grouped by these symptoms. The measurements of pulmonary function included oxygen saturation (SaO2), functional residual capacity (FRC), mixing index (MI), total respiratory system compliance (Crs), and maximal flow at FRC (VmaxFRC). Compared to NC (n = 33), the MEC group (n = 5) had a higher MI (54 vs. 42%) and no difference in SaO2, Crs or VmaxFRC. There were no significant differences between FTT (n = 8) and NC groups although there was a tendency for Crs to be lower in the FTT group (5.1 vs. 6.8 ml/cm H2O). When compared to all other groups, the COMB group (n = 11) had significantly lower SaO2, MI, Crs, and VmaxFRC. The normal lung function in the MEC group is consistent with the normal anatomy reported in CF infants dying secondary to meconium ileus. Longitudinal evaluation of the infants in this study, following initiation of care as patients with a diagnosis of CF, may allow us to determine whether symptoms at diagnosis remain an important determinant of lung function in infancy.

Topics: Cystic Fibrosis; Failure to Thrive; Functional Residual Capacity; Humans; Infant; Intestinal Obstruction; Lung; Lung Compliance; Lung Volume Measurements; Meconium

Prone positioning is valuable for accurate diagnosis of intramural air in the descending colon. In the supine position, partial collapse of the descending colon can minic intramural air. Conversely, complete collapse of the descending colon eliminates the interface between intraluminal and intramural air so that the recognition of intramural air is obscured. The tendency for the descending colon to distend with gas in the prone position may reduce these false positives and negatives for intramural air.

Topics: Air; Colon; Diagnosis, Differential; Enterocolitis, Pseudomembranous; Female; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Posture; Radiography

Topics: Adult; Cystic Fibrosis; Diseases in Twins; Enterostomy; Female; Humans; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Meconium; Pregnancy; Radiography

Three babies said to have had 'meconium stained liquor' were subsequently found never to have passed meconium. The green discoloured liquor was the result of bilious vomiting in utero secondary to intestinal obstruction.

Topics: Bile; Diagnosis, Differential; Female; Fetal Distress; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Pregnancy; Vomiting

Topics: Female; Humans; Ileal Diseases; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Male; Meconium; Peritonitis; Pregnancy

Five neonates with uncomplicated meconium ileus were successfully managed by laparotomy and T tube ileostomy. This method seems to offer advantages over other surgical techniques used in the management of uncomplicated meconium ileus that fails to respond to decompression by Gastrografin enema.

Topics: Humans; Ileal Diseases; Ileostomy; Infant; Infant, Newborn; Intestinal Obstruction; Intubation; Meconium; Therapeutic Irrigation

Topics: Contrast Media; Humans; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Intestines; Meconium; Radiography

Topics: Bile; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Vomiting

Topics: Cystic Fibrosis; Diagnosis, Differential; Dilatation, Pathologic; Female; Fetal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Intestines; Male; Meconium; Pregnancy; Prenatal Diagnosis; Ultrasonography

Topics: Adolescent; Adult; Ascites; Bile Duct Diseases; Cecal Diseases; Child; Child, Preschool; Colonic Diseases; Cystic Fibrosis; Duodenal Diseases; Gallbladder Diseases; Gastrointestinal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Intussusception; Jaundice, Neonatal; Liver Diseases; Male; Meconium; Peritonitis; Prenatal Diagnosis; Tomography, X-Ray Computed; Ultrasonography

Topics: Adult; Colon; Cystic Fibrosis; Female; Humans; Ileum; Infant, Newborn; Intestinal Obstruction; Meconium; Pregnancy; Prenatal Diagnosis; Ultrasonography

Topics: Chlorides; Cystic Fibrosis; Exocrine Pancreatic Insufficiency; Humans; Infant, Newborn; Intestinal Obstruction; Liver Cirrhosis, Biliary; Lung Diseases; Meconium; Sex Factors; Sweat

Antenatal appendicular perforation leading to localized meconium peritonitis and intestinal obstruction is reported in a premature neonate. The baby was successfully treated by a limited ileocaecal resection.

Topics: Appendicitis; Female; Fetal Diseases; Humans; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Pregnancy; Rupture, Spontaneous

Topics: Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Peritonitis

From 1969 to 1984, 42 neonates were managed for meconium ileus caused by cystic fibrosis. Simple, uncomplicated meconium ileus occurred in 24 infants (57%) and complicated meconium ileus occurred in 18 (43%). Meglumine diatrizoate (Gastrografin) enema completely relieved the obstruction in 13 patients with simple meconium ileus (54%) and caused colonic and rectal perforations in three (13%). Six operative procedures were used in 29 patients: double enterostomy (seven), resection with primary anastomosis (seven), Bishop-Koop enterostomy (seven), intraluminal lavage (four), colostomy (three), and Mikulicz enterostomy (one). Postoperative complications included malabsorptive diarrhea (nine), pneumonia (three), intestinal obstruction (two), total parenteral nutrition-catheter sepsis (two), and anastomotic leak (one). Infants managed nonoperatively by Gastrografin enema had a significantly shorter hospitalization (average, 15 days) than those undergoing operation for simple meconium ileus (54 days) and complicated meconium ileus (111 days). Postoperative survival rate was 100% with a late survival rate of 86%.

Topics: Cystic Fibrosis; Diatrizoate Meglumine; Enterostomy; Female; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium

Over the last 11 years, 22 neonates were treated with water-soluble contrast enemas to relieve the obstruction of meconium ileus. Fifteen babies had a gestational age of at least 36 weeks, and 16 weighed more than 2,500 g. All presented with clinical findings of a bowel obstruction, confirmed by roentgenograms, and each eventually had high sweat chloride levels. Each neonate had from 1 to 4 water-soluble contrast enemas administered slowly by syringe over 15 to 30 minutes. Eight enemas were successful in relieving the obstruction, four newborns requiring only one enema. Fourteen were unsuccessful, three having more than one enema. Seven of these 14 had intraabdominal pathology that would have required surgery. In five babies the bowel was perforated by the enema, the colon in three, and terminal ileum in two. These perforations were all immediately recognized during the course of the enema and operated on forthwith; a stoma was made in four cases. Only one of these five babies would have required an operation because of a volvulus. There were no fluid or electrolyte disturbances caused by the contrast material, and none of the babies with perforations died. Although this enema technique was successful in one third of cases, and despite the fact that perforations ensued in one quarter of cases, the procedure still seems warranted if the following precautions are taken: establishment of proper temperature, fluid, and electrolyte balance; the radiologist is not rushed, is extremely gentle, willing to repeat the study until no further progress is evident; the surgeon is available for an immediate laparotomy should a perforation occur.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Diatrizoate Meglumine; Enema; Humans; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Meconium; Radiography

A child with cystic fibrosis developed the meconium ileus equivalent in the postappendectomy period. This problem has not been emphasized in patients with cystic fibrosis. Operative evacuation only partially relieved the obstruction. Transient upper gastrointestinal bleeding fortuitously produced beneficial effect, which completely resolved the persisting ileus.

Topics: Appendectomy; Appendicitis; Child, Preschool; Cystic Fibrosis; Feces; Humans; Intestinal Obstruction; Male; Meconium; Peritonitis; Postoperative Complications

Topics: Anus, Imperforate; Enterocolitis, Pseudomembranous; Hirschsprung Disease; Humans; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Contrast Media; Humans; Infant, Newborn; Intestinal Obstruction; Iodobenzoates; Iohexol; Male; Meconium; Radiography; Triiodobenzoic Acids

Topics: Ambulatory Care; Catheters, Indwelling; Child; Child, Preschool; Home Nursing; Humans; Infant; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Intestinal Perforation; Meconium; Parenteral Nutrition; Parenteral Nutrition, Total; Short Bowel Syndrome

Topics: Adult; Calcinosis; Female; Humans; Ileal Diseases; Infant, Newborn; Intestinal Obstruction; Meconium; Peritonitis; Pregnancy; Prenatal Diagnosis; Ultrasonography

Ultrasonography has proven to be a helpful imaging modality in evaluating the child with a distended gasless abdomen. The presence of an unsuspected mass can easily be detected as well as the presence of ascites and/or abnormally dilated loops of small bowel. Thickened loops of bowel can also be easily detected in the abdomen with very little gas. Closed loop obstruction and intussusception, as well as meconium ileus equivalent (distal intestinal obstruction syndrome), are described.

Topics: Abdomen; Child, Preschool; Female; Humans; Ileal Diseases; Infant; Infant, Newborn; Intestinal Obstruction; Intussusception; Male; Meconium; Mesenteric Cyst; Ultrasonography

Two cases are reported of patients in the third trimester of pregnancy in whom routine ultrasound examination revealed progressive dilatation of the fetal intestines. Intestinal obstruction was suspected and amniocentesis as well as amniography with Urografin were done. In both cases a regression in dilatation of bowel was noted on ultrasonography. The Urografin swallowed by the fetus was deemed to have relieved the obstruction of the fetal intestine. The babies were delivered in good condition and passed large amounts of watery meconium per rectum. In spite of the risks inherent in the use of Urografin amniography, as well as of amniocentesis, there appear to be a great number of advantages to the treatment of meconium obstruction of the fetus in utero.

Topics: Adult; Amniocentesis; Amniotic Fluid; Diatrizoate; Diatrizoate Meglumine; Evaluation Studies as Topic; Female; Fetal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Pregnancy; Prenatal Diagnosis; Radiography; Risk; Syndrome; Ultrasonography

From June 1980 to September 1984, forty-five newborns (weight greater than or equal to 2000 g), initially presumed normal, were seen with bilious vomiting in the first 72 hours and were prospectively followed up. Nine (20%) required surgical intervention, five (11%) had nonsurgical obstruction such as meconium plug or left microcolon, and the remaining 31 (69%) had idiopathic bilious vomiting. Infants with idiopathic bilious vomiting had a benign transient course and resumed feedings by 1 week of age; 30 of the 31 had normal or nonspecific findings on initial plain abdominal roentgenogram. Specific findings on the initial plain abdominal roentgenogram were noted in five infants, and four (80%) of these had a lesion requiring surgical intervention; 56% (5/9) of neonates with surgical lesions had normal or nonspecific findings on the plain abdominal roentgenograms. None developed bowel ischemia or midgut infarction secondary to a volvulus as they were identified by contrast studies shortly after the initial episode of bilious vomiting. Although the majority of "normal" neonates with bilious vomiting do not have a surgical lesion, this study indicates that 56% of surgical cases will be missed if contrast studies are not done.

Topics: Age Factors; Bile; Colon; Contrast Media; Female; Gastric Lavage; Gastrointestinal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Intubation, Gastrointestinal; Male; Meconium; Radiography, Abdominal; Vomiting

In contrast to bloatedness and steatorrhoea the meconium ileus equivalent is a less well known gastrointestinal complication in cystic fibrosis and thus less frequently correctly diagnosed. The term, first used by Jensen in 1962, notifies partial or complete obstruction due to increasing viscosity of gut contents. The occurrence of the meconium ileus equivalent increases with age (approx. 10%), recurrences are possible. Among 73 patients with cystic fibrosis 8 patients with ileus equivalents were observed, two of them had a recurrence Surgery was performed only in one case. In all other patients the intestinal obstruction was relieved by oral and enema administration of N-acetylcysteine.

Topics: Acetylcysteine; Adolescent; Child; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; Female; Follow-Up Studies; Humans; Ileal Diseases; Intestinal Obstruction; Male; Meconium; Recurrence

During the 15 years from 1970 to 1984, 38 infants, all with cystic fibrosis, were treated for meconium ileus at The Montreal Children's Hospital and Ste-Justine Hospital for Children. Thirteen patients (34%) had complicated meconium ileus that included 7 perforations (2 colon, 5 ileum), 4 volvulus, and 2 atresia with meconium pseudocyst. In this group, various operations were done: resection with primary anastomosis for atresia, or resection with enterostomy for peritonitis or volvulus. One died shortly after surgery. Of 25 patients with uncomplicated meconium ileus (66%), one died shortly after arrival from respiratory distress, leaving 24 patients available for study. Gastrografin enema was attempted on 20 patients with eight successes (40%). Of the remaining 16 patients with unresolved meconium ileus, nine were treated with laparotomy and ileostomy, and one with laparotomy and T-tube irrigation. Six patients were treated by laparotomy and enterotomy for irrigation with N-acetylcysteine and evacuation by Fogarty catheter, a technique not widely used. No one succumbed in this group. This latter method of management is recommended for patients with simple uncomplicated meconium ileus.

Topics: Cystic Fibrosis; Humans; Ileal Diseases; Ileostomy; Infant, Newborn; Intestinal Obstruction; Laparotomy; Meconium; Therapeutic Irrigation

Topics: Adult; Cystic Fibrosis; Diagnosis, Differential; Humans; Ileal Diseases; Intestinal Obstruction; Male; Meconium

A small left colon has been observed in neonates due to several causes: aganglionosis, meconium plug, and neonatal small left colon syndrome. We report a case of small left colon in an infant with cystic fibrosis, underscoring the need to consider this disease in the diagnostic work up.

Topics: Adult; Barium Sulfate; Colon; Cystic Fibrosis; Enema; Female; Glycosuria; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Pregnancy; Pregnancy Complications; Radiography

The effects of neonatal diagnostic screening on cystic-fibrosis (CF) -related morbidity were evaluated by comparing hospital admissions for CF-related illness in the first 2 years of life in 40 patients detected by means of neonatal screening and 56 patients born in the 3 years before screening began. Unscreened patients without meconium ileus had a mean of 27.25 hospital days for CF-related illness, and screened patients a mean of 3.9 days. There was no trend with time towards fewer days spent in hospital: the change was sudden. The difference was significant and could not be attributed to non-comparability of groups, changes in admission policy, or changes in management. In patients with meconium ileus there was no significant difference in hospital admissions between the groups. Neonatal screening significantly reduces CF morbidity in the first 2 years of life.

Topics: Child, Preschool; Cystic Fibrosis; Female; Hospitalization; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Length of Stay; Male; Mass Screening; Meconium

Topics: Female; Humans; Ileal Diseases; Infant, Newborn; Intestinal Obstruction; Meconium; Ultrasonography

Meconium ileus (MeI) is the presenting symptom in 10 to 15 percent of infants with cystic fibrosis (CF), but it is a rare phenomenon in patients who do not have this disease. We report four Bedouin siblings who had MeI with no laboratory evidence of CF.

Topics: Electrolytes; Female; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Sweat

In recent years patients with cystic fibrosis (CF) have experienced longterm survival and have demonstrated a number of intra-abdominal complications. This report evaluates the intra-abdominal complications seen in 69 of 189 children with cystic fibrosis from 1972 to 1983. Forty-one patients were boys and twenty-eight girls. Complications occurred in 36 neonates, with meconium ileus (MI) noted in 33 and giant cystic meconium peritonitis (GCMP) in 3. Meconium ileus equivalent occurred in seven older children presenting with bowel obstruction. In addition, rectal prolapse occurred in 12, inguinal hernia in 10, intussusception in 3, cholelithiasis in 3, GE reflux in 4, stress ulcer in 1 and appendicitis in 1. Three infants with GCMP survived resection and enterostomy. Infants with MI were divided into simple (15) or complicated (18) cases. Nonoperative therapy using gastrografin enema was successful in three of eight with simple MI. Operative enterotomy and irrigation was successful in three cases while resection and enterostomy was done in nine. MI was complicated by atresia, volvulus and/or perforation in 18 cases requiring resection and anastomosis or enterostomy. Survival for MI was 86% compared to 36% in 25 MI patients treated in the previous two decades. Meconium ileus equivalent was successfully managed using gastrografin enema in five of seven children. Only 3 of 12 children with rectal prolapse required repair. Two cases of intussusception were reduced while one required resection. Three of 10 children had hernia recurrence due to chronic pulmonary problems.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Abdomen; Adolescent; Child; Child, Preschool; Cystic Fibrosis; Female; Hernia, Inguinal; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Intussusception; Male; Meconium; Peritonitis; Rectal Prolapse

The incidence of cystic fibrosis in Ontario, Canada has been determined from clinical data, from the cystic fibrosis database of the Hospital for Sick Children, Toronto, and from population statistics in the Province of Ontario. The survey included 420 confirmed cases of cystic fibrosis born during the period 1966-1980. The mean incidence during this period was one in 2,927. In the last 5-year period, a decline was noted in incidence that may have reflected in part the effectiveness of early diagnosis and genetic counseling in affected families. During the period of the survey, over 60% of cases were diagnosed within the first year of life, 74% by age 2 years, and 90% by age 5 years. Clinical diagnosis in the first year of life was more common in males (65%) than in females (54%), a consistent finding during the period of the survey. The incidence of meconium ileus was 15.7% of ascertained cases of cystic fibrosis, with similar incidences in males (16.4%) and females (14.4%). Although survival has not been the subject of this survey, mortality in the neonatal period was significantly higher in males than in females with cystic fibrosis.

Topics: Age Factors; Child; Child, Preschool; Cystic Fibrosis; Epidemiologic Methods; Female; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Ontario; Sex Factors

Intestinal obstruction and necrotizing enterocolitis are two of the most common surgical emergencies of the gastrointestinal tract. This article reviews the signs and symptoms of these conditions and presents guidelines for their management.

Topics: Colon; Diagnosis, Differential; Duodenal Obstruction; Emergencies; Enterocolitis, Pseudomembranous; Fluid Therapy; Humans; Ileum; Infant, Newborn; Infant, Premature, Diseases; Intestinal Atresia; Intestinal Obstruction; Jejunum; Meconium; Postoperative Complications

Topics: Clinical Enzyme Tests; Cystic Fibrosis; Female; Humans; Intestinal Obstruction; Meconium; Pregnancy; Prenatal Diagnosis

Topics: Humans; Infant, Newborn; Intestinal Obstruction; Meconium

Cystic fibrosis (CF) was diagnosed in 12 children from a total of 15,314 children admitted to the Paediatric Department at Jordan University Hospital (JUH) over the period 1976-1980. A diagnosis of meconium ileus was made in five children. The remaining seven affected children were diagnosed clinically on the basis of recurrent chest problems, failure to thrive, positive family history and a high level of sweat chloride, range 72-115 mEq/l (mean 97.6 mEq/l). Five children died (41.7%); three of the group with meconium ileus died during the post-operative period and two of the other group died soon after the diagnosis was made. The cause of such a high mortality rate is multifactorial. As CF was only recently recognized in Jordan, it is believed that the prevalence of CF among Jordanian children is underestimated. CF is still a very serious illness that demands early diagnosis and an intensive programme of continuing care. Therefore, further prospective study to evaluate its prevalence and impact on child health in Jordan is urgently needed.

Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Jordan; Male; Meconium; Pilot Projects; Sweat

Topics: Diatrizoate; Diatrizoate Meglumine; Enema; Humans; Hypotension; Hypoxia; Infant, Newborn; Intestinal Obstruction; Intraoperative Complications; Male; Meconium; Plasma Volume

Out of 813 laparotomies performed for surgical problems of small bowel in infants and children, resections were necessary in 327 patients. A primary enterostomy was done in 75 cases with subsequent anastomosis. The different surgical problems, the operative findings, the various procedures adopted and the results thereof are discussed here. The greatest danger was seen to arise from the ischaemia of the bowel and from peritonitis. In doubtful conditions of small bowel two-stage operation seems advisable.

Topics: Child; Child, Preschool; Enterocolitis, Pseudomembranous; Hernia; Humans; Ileostomy; Infant; Infant, Newborn; Intestinal Diseases; Intestinal Obstruction; Meconium; Prognosis; Stomach

Topics: Adult; Cystic Fibrosis; Diseases in Twins; Female; Humans; Intestinal Obstruction; Intestine, Small; Male; Meconium; Pregnancy; Pregnancy Trimester, Third; Prenatal Diagnosis; Twins, Monozygotic; Ultrasonography

The radiographic findings of 200 cases of meconium peritonitis were analyzed; 194 cases were discovered in newborn infants and six cases in fetuses. The radiographic features in the newborn group could be categorized as pneumoperitoneum with intestinal obstruction (adhesions) and calcification (35 cases), intestinal obstruction with calcification (143 cases), intestinal obstruction with no radiographically visible calcification (5 cases), and calcification alone (11 cases). All six cases of the fetal group were diagnosed when the mothers had been hospitalized for polyhydramnios and a plaque-like or ring-like calcification showed up in the fetal abdomen on the plain radiograph. Meconium peritonitis is one of the few conditions that can be diagnosed before birth and is almost the only condition around the time of birth to produce calcification in the abdomen. Therefore, if there is any sign of polyhydramnios, radiographs or ultrasonograms of the maternal abdomen should be obtained to detect any calcification within the peritoneal cavity of the fetus. A simple experiment carried out in rats showed that it takes at least eight days after the meconium escapes into the peritoneal cavity for calcification in the meconium to be radiographically demonstrable.

Topics: Adult; Calcinosis; Female; Fetal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Peritonitis; Pneumoperitoneum; Polyhydramnios; Pregnancy; Radiography

Calcified intraluminal meconium is rarely observed in newborn. Only 27 cases are related in litterature; the authors report 4 new cases and point out the role of intestinal obstruction (principaly anorectal malformations, but also atresia, meconium ileus or Hirschsprung disease). Pathogenesis is unknown, while intestinal urinary fistula is frequently described.

Topics: Abnormalities, Multiple; Calcinosis; Digestive System Abnormalities; Humans; Infant, Newborn; Intestinal Fistula; Intestinal Obstruction; Male; Meconium; Urinary Fistula; Urinary Tract

Children with cystic fibrosis may develop intestinal obstruction later in life due to inspissation of small bowel content. Two children with cystic fibrosis who developed intestinal obstruction at the age of 5 and 14 years respectively are reported. The condition may be precipitated by reduction or stopping pancreatic supplement, dehydration or intercurrent chest infection. The intensive therapy of chest complications has improved the survival of children with cystic fibrosis, so that it is likely that in future more cases of meconium ileus equivalent will present in later childhood and in adulthood.

Topics: Adolescent; Age Factors; Cystic Fibrosis; Humans; Intestinal Obstruction; Intestine, Small; Male; Meconium; Radiography

Life tables were calculated for 273 British children with cystic fibrosis for the period 1974-9. There was a marked improvement in survival rates in the meconium ileus group compared with the 1969-73 data, but there was little improvement in patients presenting later with other symptoms.

Topics: Adolescent; Age Factors; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Intestine, Large; Male; Meconium

Topics: Australia; Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; United States

Topics: Diatrizoate Meglumine; Enema; Female; Humans; Infant, Low Birth Weight; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Intestinal Obstruction; Male; Meconium; Radiography

Meconium ileus is the initial clinical manifestation of cystic fibrosis. Once the intestinal obstruction is corrected either medically or surgically the course of disease is that of cystic fibrosis. It is suggested that babies with this disease should be treated in specialized pediatric cystic fibrosis centers to prevent early death or some avoidable complications. Genetic counseling for affected families is recommended when a baby is born with this condition. Usually such infants are born at full term and there is no sex predilection. Prognosis is uncertain and the outlook depends, as it does in cystic fibrosis, on the degree and rate of progression of the pulmonary involvement. This paper presents a series of unusual patients with meconium ileus who have reached adulthood, thus, offering hope that a satisfactory life beyond adolescence is possible in this formerly fatal disease of infancy.

Topics: Adult; Cystic Fibrosis; Female; Follow-Up Studies; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Prognosis

Topics: Cystic Fibrosis; Diagnosis, Differential; Hirschsprung Disease; Humans; Ileal Diseases; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Male; Meconium

Topics: Colon; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Radiography

A prospective study, followed by a retrospective audit, was made of the radiographs and clinical features of 225 consecutive neonates with abdominal distension presenting during a period of 4 years. More than one major cause for the distension was found in a third of cases. Excluding cases of ano-rectal atresia, diagnostic difficulties were encountered in 31% of cases and, occasionally, the diagnosis was revised several times as investigations proceeded. Neuhauser's sign of 'bubbly' meconium is unreliable, being found in cases of meconium ileus, ileal atresia, Hirschsprung's disease and necrotising enterocolitis. Concomitant small-bowel atresia should be suspected in all cases of meconium ileus; intestinal malrotation should be considered in association with duodenal and intestinal atresias. In the absence of an obvious anatomical defect, it is difficult to differentiate between the various causes of functional obstruction by radiological means alone and all such infants should be referred to a specialised centre for intensive investigation and treatment. Very often, the skills of a neonatologist, paediatric surgeon and, especially, an experienced pathologist will be required in addition to those of the radiologist.

Topics: Anal Canal; Contrast Media; Enterocolitis, Pseudomembranous; Hirschsprung Disease; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestine, Small; Meconium; Prospective Studies; Radiography, Abdominal; Rectum; Retrospective Studies

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Adult; Carcinoma; Female; Hirschsprung Disease; Humans; Hyperparathyroidism; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Multiple Endocrine Neoplasia; Pedigree; Pheochromocytoma; Quebec; Thyroid Neoplasms

In 20 years the authors have met with 115 cases of meconium peritonitis (MP). The high incidence in China is shown. In all cases there were intraabdominal calcifications. There were no cases of fibrocystic disease. The authors classify 3 types: 1. Neonatal obstructive type 41 cases. 2. Free perforation with: Free pneumoperitoneum 15 cases. Localized pneumoperitoneum 23 cases. 3. No signs or symptoms, with potential intestinal obstruction 23 cases. The series shows a low incidence of atresia. The authors suggest necrotizing enterocolitis as a cause because of the high incidence of stenosis. Three prenatally diagnosed cases are discussed. All had hydramnios and fetal abdominal calcifications. Two of these were confirmed after birth. The mortality is high (42.6%). Abdominal x-ray in all cases with polyhydramnios may lead to a positive diagnosis of MP.

Topics: Abdomen; Adult; Calcinosis; China; Enterocolitis, Pseudomembranous; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Pneumoperitoneum; Polyhydramnios; Pregnancy; Prenatal Diagnosis

Topics: Cystic Fibrosis; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium

The clinical histories, chest X-ray appearances, skin prick test results, pulmonary function and IgE levels of 20 patients with cystic fibrosis (C.F.) complicated by nasal polyps have been compared with those of 97 C.F. controls. The patients who had developed polyps had a different clustering of symptoms to the controls with heavier birthweights, later presentations, milder gastrointestinal symptoms in infancy, less infection with Staphylococcus aureus and better vital capacities. There was no evidence on history, skin testing or IgE levels that the polyps patients were more allergic. It is likely, therefore, that nasal polyps in C.F. are due to a primary effect of the C.F. gene, although they could also be the effect of chronic nasal infection.

Topics: Birth Weight; Child, Preschool; Cystic Fibrosis; Humans; Hypersensitivity; Infant, Newborn; Intestinal Obstruction; Meconium; Nasal Polyps; Staphylococcal Infections; Vital Capacity

Meconium diseases in infancy cannot be neatly separated into discrete categories of meconium plug syndrome, meconium ileus, and meconium peritonitis; nor can the therapy of each condition. A patient with any of the three may or may not have cystic fibrosis. All babies with any form of meconium obstruction or atresia must have a sweat chloride test to confirm or rule out this diagnosis. Repeated gastrografin enemas can decrease the need for operative intervention in all babies with meconium plug syndrome and in selected babies with meconium ileus. Babies with meconium diseases can now be managed with a low perinatal mortality.

Topics: Barium Sulfate; Cystic Fibrosis; Female; Hirschsprung Disease; Humans; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Intestinal Obstruction; Male; Meconium; Peritonitis; Retrospective Studies

Topics: Cystic Fibrosis; Enema; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Postoperative Complications; Pregnancy

Topics: Diatrizoate; Diatrizoate Meglumine; Enema; Enterocolitis, Pseudomembranous; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

The diagnosis of Hirschsprung's disease is at times difficult, particularly in the young patient. Since 1972 we have used rectal suction biopsy as a screening technique in neonates and infants with failure to pass meconium or evidence of obstruction. In addition, it is used to confirm the diagnosis of Hirschsprung's disease when suspected by barium enema study. This technique has been used in 444 patients, 302 of whom were less than one year of age. No anesthesia is necessary, and there have been no associated complications. Only one patient early in the study had an initial misdiagnosis. There have been no false-positive or false-negative specimens since this initial problem, and no patients have undergone inappropriate pull-through procedures for suspected Hirschsprung's disease. It is recommended that all neonates who do not pass meconium in the first 48 hours of life undergo rectal suction biopsy to establish the diagnosis of congenital megacolon.

Topics: Biopsy, Needle; Child; Child, Preschool; Diagnosis, Differential; Evaluation Studies as Topic; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Megacolon; Rectum

Topics: Cystic Fibrosis; Esophageal Atresia; Humans; Infant, Newborn; Intestinal Obstruction; Intestines; Meconium; Megacolon; Prognosis

A recurrence rate for meconium ileus of 39% was found among C.F. affected siblings in a study of 488 families with at least one C.F. child born over a 24 year period. The recurrence rate was highly significant and indicated a familial trend for the occurrence of meconium ileus. The findings of the study support the existence of genetic heterogeneity in cystic fibrosis.

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

There are many treatment modalities for uncomplicated meconium ileus. However, for neonates with intestinal obstruction unrelieved by nonoperative measures we advocate T-tube ileostomy with postoperative irrigation of the intestine utilizing pancreatic enzyme. Our series includes 11 infants with uncomplicated meconium ileus who underwent T-tube ileostomy 24--96 hr after birth. Of the 7 females and 4 males only 2 infants failed to pass meconium. One infant required reexploration for persistent obstruction and 1 infant, the only death encountered, had overwhelming pulmonary infection and intracranial hemorrhage. The remaining 9 infants passed meconium between the 1st and 11th postop days. The T-tube was removed by pulling it out between the 10th and the 14th postop day with the T-tube tract closing spontaneously in all patients. Our experience with T-tube ileostomy suggests that resection of dilated but viable ileum is not necessary for prompt intestinal function in the neonate. With the use of pancreatic enzyme irrigation, the tenacious meconium in these patients can be rapidly liquified and expelled per rectum or T-tube and the necessity of mechanical removal during the operative procedure avoided.

Topics: Catheterization; Female; Humans; Ileostomy; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium

Meconium Ileus Equivalent is a condition which occurs in patients with cystic fibrosis where the ileum becomes obstructed by bowel content of abnormal consistency. Intercurrent chest infection or absence of pancreatic extract supplements are often the exacerbating cause. Plain films of the abdomen show small bowel obstruction and in some a nodular pattern in the small bowel, the nodules being larger than those seen in quiescent cystic fibrosis. Gastrografin enemas and/or enemas with acetyl cysteine can be used in the treatment of the condition, the Gastrografin having the advantage of confirming the diagnosis radiologically. The condition is important as it can be the presenting feature of cystic fibrosis in childhood and even in early adult life, and the operative mortality and morbidity are high. Conservative treatment should be pursued vigorously and operation only undertaken if unavoidable.

Topics: Child; Child, Preschool; Cystic Fibrosis; Humans; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Radiography

Topics: Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Prognosis

Topics: Digestive System; Digestive System Abnormalities; Duodenum; Enterocolitis, Pseudomembranous; Gastrointestinal Diseases; Hernia, Umbilical; Humans; Infant, Newborn; Intestinal Obstruction; Meckel Diverticulum; Meconium; Megacolon

An autopsy case of bilateral renal dysplasia with other congenital malformations is reported. Malformations included severe pancreatic fibrosis with meconium ileus, situs inversus totalis, cardiovascular anomalies, and others. The only syndrome of multiple congenital malformations involving renal dysplasia which is similar to the present case is Ivemark's syndrome, though the present case lacked hepatic lesions. Apart from the pancreatic lesion, there were no abnormalities compatible with cystic fibrosis (mucoviscidosis). It was considered that meconium ileus in the present case was caused by pancreatic achylia secondary to severe pancreatic fibrosis, unrelated to cystic fibrosis, but closely allied to renal dysplasia.

Topics: Abnormalities, Multiple; Cystic Fibrosis; Diagnosis, Differential; Female; Humans; Infant, Newborn; Intestinal Obstruction; Kidney; Meconium; Pancreatic Diseases; Situs Inversus

A child with cystic fibrosis was given cimetidine in addition to pancreatic extract. Although the stool frequency was reduced the cimetidine had to be withdrawn because meconium ileus equivalent developed.

Topics: Child; Cimetidine; Cystic Fibrosis; Guanidines; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium

Review of the newborn nursery notes of 87 infants who had cystic fibrosis without meconium ileus (MI) indicated that 12 of them had clinical features consistent with the meconium plug syndrome (MPS). This high incidence of meconium abnormalities suggests that MI and the MPS probably represent different gradations of the same underlying pathologic abnormality. The findings further suggest that a quantitative pilocarpine iontophoresis sweat test be performed in every newborn infant with any type of meconium abnormality, including the spontaneous passage of meconium plugs.

Topics: Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Diseases; Intestinal Obstruction; Meconium; Pilocarpine; Sweat; Syndrome

A study of 29 neonates with intestinal obstruction has shown that a massively dilated, bulbous terminal segment of bowel is characteristic of neonatal infestinal obstruction of congenital origin. The sign is an important diagnostic indicator but its absence does not exclude intestinal atresia.

Topics: Dilatation, Pathologic; Humans; Ileal Diseases; Ileum; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Jejunal Diseases; Jejunum; Meconium; Radiography; Retrospective Studies

Meconium ileus equivalent is an unusual cause of intestinal obstruction in adults. In this paper we report of our experience with a 29-year-old male with a long-standing history of cystic fibrosis and recurrent abdominal pain. Following barium examination of the stomach and small bowel, the patient developed increasing abdominal pain and evidence of meconium ileus equivalent as the etiology of his small bowel obstruction. The obstruction was relieved by administration of a 20% sodium diatrizoate enema and oral saline cathartics. The clinical and radiographic findings of meconium ileus equivalent are reviewed, as is the use of water-soluble contrast agents in the management of this condition. The role of prior barium study in precipitating this condition is discussed.

Topics: Cathartics; Contrast Media; Diatrizoate; Enema; Humans; Intestinal Obstruction; Intestine, Small; Meconium; Radiography; Sodium Chloride

Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Diabetes Mellitus; Female; Gastrointestinal Diseases; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Liver Cirrhosis; Malabsorption Syndromes; Meconium; Puberty; Rectal Prolapse

Eleven cases of neonatal intestinal obstruction associated with a white ileal meconium plug are described; 6 of these presented with complications--such as, giant meconium pseudocyst, perforation, volvulus, or atresia. Most of these complications are presumed to have arisen during the intrauterine period. Only one patient could be relieved of the ileal meconium plug by enemas. The condition of ileal meconium plug is not as benign as a meconium plug in the rectum or distal colon. A plea is made to restrict the name meconium plug syndrome to cases in which the meconium plug is white and chalky and the consequent intestinal obstruction can be relieved by enemas, without evidence of intestinal dysfunction in later life.

Topics: Enema; Female; Humans; Ileal Diseases; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Syndrome

A technique of intestinal anastomosis, developed for the treatment of meconium ileus, was used by the authors in the treatment of neonates suffering from various other intestinal anomalies such as atresia, volvulus and apple-peel syndrome. This technique was used in 6 neonates to make a safe intestinal reconstruction.

Topics: Female; Humans; Ileostomy; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Intestine, Small; Male; Meconium; Methods

Topics: Cystic Fibrosis; Diatrizoate; Diatrizoate Meglumine; Enema; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestine, Small; Meconium

Topics: Cystic Fibrosis; Humans; Ileal Diseases; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Meconium ileus equivalent is a late intestinal occlusion occurring in patients suffering from mucoviscidosis. Three cases are reported in children aged, respectively, 3 years, 6 years and 10 years. In one of the observations (three year old girl), the intestinal obstruction with feces was first manifestation of a mucoviscidosis that was previously undetected. The radiological signs are identical to those found in cases of neonatal meconium ileus: intestinal obstruction and accumulation of closely spaced matter in a distended ileum (terminal part). As in cases of meconium ileus without complications, treatment consisted of enemas with water-soluble products. The high osmotic pressure of these products made it possible to remove the obstruction.

Topics: Child; Child, Preschool; Cystic Fibrosis; Enema; Female; Humans; Intestinal Obstruction; Intestine, Small; Male; Meconium

Three cases of premature infants with obstruction of the ileum and proximal colon in the absence of cystic fibrosis are presented. Meconium inspissation complicated low-grade bowel obstruction in 2 infants. In the third, the bowel was anatomically normal, with the tenacious meconium the apparent primary cause of obstruction. Distinguishing features of this unusual type of blockage and other meconium syndromes are also discussed. This condition may simulate necrotizing enterocolitis, requiring surgery for differentiation. It should be considered in the differential diagnosis of low small bowel or colonic obstruction in the premature infant who is several days of age.

Topics: Colonic Diseases; Diagnosis, Differential; Enterocolitis, Pseudomembranous; Female; Humans; Ileum; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Male; Meconium; Radiography

Topics: Adult; Cysteine; Diatrizoate; Drug Therapy, Combination; Female; Humans; Intestinal Obstruction; Meconium; Pancreatin

Topics: Blood Viscosity; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Polycythemia

Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Female; Growth Disorders; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Pancreatic Diseases; Respiratory Tract Infections; Sweating

1. The mucus glycoproteins in meconium from normal infants and from infants having cystic fibrosis with meconium ileus have been studied. 2. Whereas normal meconium contained about 50% protein-bound carbohydrate, the meconium from cystic fibrosis contained only about 10%. 3. Glycopolypeptides were prepared from the mucus of glycoproteins. The oligosaccharide units from this material were released and fractionated. The fractions ranged widely in size and composition. 4. The fractions from cystic fibrosis specimens had a significantly higher content of fucose than those from normal specimens.

Topics: Carbohydrate Metabolism; Cystic Fibrosis; Fucose; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Monosaccharides; Mucoproteins; Oligosaccharides

Topics: Germany, West; Humans; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Intestinal Obstruction; Meconium; Postoperative Complications

During the 14 yr from 1965 through 1978, 49 infants presented shortly after birth with intestinal obstruction due to impacted meconium. Three of these patients did not have fibrocystic disease. Eight patients were cured by a Gastrografin enema. There were 18 patients who had complications that included associated atresia, volvulus, and/or peritonitis. Various operations were done including resection with either primary anastomosis or enterostomy or varieties of the foregoing. Twenty-three babies had the simple uncomplicated form of meconium ileus. Eleven of these underwent resection and six patients died. Twelve patients were treated by laparotomy, ileotomy through a purse-string suture and prolonged irrigations using acetylcysteine. Of this group only one succumbed. This latter course of management is recommended for patients with simple uncomplicated meconium ileus as it involves no resection, no enterostomy, nor any primary anastomosis.

Topics: Cystic Fibrosis; Female; Humans; Ileostomy; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Laparotomy; Male; Meconium; Methods

Screening for cystic fibrosis (CF) in newborns is desirable, and efforts should continue in establishing a system of easily available, reliable, simple, and inexpensive tests. In addition to the Boehringer-Mannheim (BM) test for the detection of albumin, we propose the assay for lactase and beta-D-fucosidase in meconium. These latter two enzymes are present in the meconium of babies with CF and absent in meconium of most healthy babies. In a mass screening program for CF involving 20,182 specimens of meconium using only the BM strip, we found 46 positive results. Twenty-nine specimens came from infants with CF, six of whom had meconium ileus. Seventeen specimens yielded false-positive results by the BM test. Eleven of these would have been excluded by the addition of the lactase and beta-D-fucoside assay, thus reducing the false-positive test results by nearly 61%. In a comparative study of the three methods, the lactase and beta-D-fucosidase yielded 1.2% false-positives when examined independently. Performance of these two assays may allow greater specificity in diagnosis when used in addition to the BM test.

Topics: Albumins; alpha-L-Fucosidase; beta-Galactosidase; False Positive Reactions; Galactosidases; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Mass Screening; Meconium; Sweat

Although successful treatment of meconium ileus with Gastrografin enema has been attributed to the hypertonicity of this material, Tween 80, present in a 0.75% concentration, may be its most effective component. Tween 80 acts as a surface-active substance and stool emulsifier. An enema solution of 1%-2% Tween 80 and isotonic sodium diatrizoate has been effective in the relief of meconium or fecal mass obstruction in infants and children.

Topics: Adolescent; Child, Preschool; Diatrizoate; Enema; Fecal Impaction; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Polyethylene Glycols; Polysorbates

Topics: Adolescent; Adult; Airway Obstruction; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Infertility; Intestinal Obstruction; Liver; Lung; Male; Meconium; Middle Aged; Pancreas; Respiratory Tract Infections

Topics: Adult; Amniotic Fluid; Bilirubin; Colon; Diseases in Twins; Erythroblastosis, Fetal; Female; Humans; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Pregnancy; Prenatal Diagnosis

The meconium plug syndrome (MPS) is a common cause of low intestinal obstruction in newborn infants. Usually, it is benign and not associated with other intestinal dysfunction. We describe the cases of three infants in whom there was intestinal obstruction consistent with the MPS and in which cystic fibrosis (CF) was later documented. We suggest that there is an association between the MPS and CF, and that the condition of all infants with the MPS be evaluated for the possibility of both Hirschsprung's disease and CF.

Topics: Cystic Fibrosis; Diagnosis, Differential; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Megacolon; Radiography; Syndrome

Topics: Enema; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

A case of meconium ileus equivalent in a 30-year old man is presented. The pathology and therapy for the condition are discussed.

Topics: Acetylcysteine; Adolescent; Adult; Age Factors; Child; Child, Preschool; Cystic Fibrosis; Fecal Impaction; Humans; Ileum; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Radiography; Respiration, Artificial

The milk curd syndrome is an as yet little known cause of intestinal obstruction in neonates. Two personal cases are reported, and 37 others are collected from the world literature. Infants with milk curd syndrome have a normal discharge of meconium. The intestinal obstruction appears usually between the 2nd and 16th day after birth. In most of the cases, the milk curd was found in the distal part of the ileum. The aetiology of the milk curd obstruction syndrome remains still unclear. The composition of artificial milk feeding formula in connexion with transitory absorption deficiency of amino acids, and the possibility of a temporarily insufficient excretion of bile acids are discussed.

Topics: Female; Humans; Infant Nutritional Physiological Phenomena; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Milk, Human; Syndrome

Topics: Cystic Fibrosis; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Prognosis

This is a case report of meconium ileus complicated by intestinal volvulus and infarction in a black infant.

Topics: Black People; Colon; Cystic Fibrosis; Ductus Arteriosus, Patent; Female; Humans; Ileostomy; Ileum; Infant; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Jejunum; Meconium; Pregnancy

A baby with congenital jejunal and ileal atresia is described. There was meconium-like staining of the amniotic fluid which was probably due to fetal regurgitation or vomiting.

Topics: Amniotic Fluid; Female; Humans; Ileum; Infant, Newborn; Intestinal Obstruction; Jejunum; Male; Meconium; Pregnancy

Six neonates are described in whom plain films of the abdomen demonstrated scattered flecks of calcification. Three of the patients had rectal atresia with recto-urethral fistula, one rectal atresia without fistula, one jejunal atresia and one a severe stenosis of the distal small bowel associated with log segment aganglionosis. At operation the calcification was found to be in meconium within the bowel lumen and not as expected to represent serosal plaques following meconium peritonitis due to an intrauterine bowel perforation. Previous case reports are reviewed and possible mechanism discussed.

Topics: Calcinosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Male; Meconium; Radiography

Two newborns with intestinal obstruction of the terminal ileum without mucoviscidosis were cured by Gastrografin enema. A further case of a premature infant showed, at autopsy, a large intestine containing normal meconium, but an ileum which was plugged with tenacious meconium containing PAS-positive and alcian blue-negative mucus. Our 3 cases were similar to cases of "meconium disease" reported by Rickham and of "meconium plug in the small intestine" reported by Emery. The primary cause of this disease lies in the large amount of PAS-positive mucus secreted from goblet cells of the ileal mucosa and the hypertrophy of lymph nodules in the ileal submucosa.

Topics: Contrast Media; Cystic Fibrosis; Enema; Female; Humans; Hypertonic Solutions; Hypertrophy; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Mucosa; Intestinal Obstruction; Male; Meconium; Mucus

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Pancreatic Diseases; Syndrome

Neonatal small left colon syndrome is a functional disease of the lower colon which produces typical signs and symptoms of intestinal obstruction. It is manifest in the first 24-48 hours of life, particularly in infants of diabetic mothers and, if detected early, it can be fully cured by radiographic contrast enemas, not unlike meconium plug syndrome. Intestinal perforation and death may occur, however. The etiology of the disease is unknown but it may relate to neurohumoral imbalances between the autonomic nervous system and glucagon.

Topics: Colonic Diseases; Contrast Media; Enema; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pregnancy; Pregnancy in Diabetics; Solubility; Syndrome; Water

Six patients with meconium ileus, in whom bowel resection and end-to-end anastomosis were performed, are presented. Meconium ileus may be simple or complicated. The simple form may be treated by Gastrografin enema. Surgery is indicated if conservative treatment fails and for patients with a complicated obstruction. Provided the bowel is disobstructed and carefully resected, end-to-end anastomosis is preferable. Confirmation of the diagnosis by careful histological examination of the resected bowel is urged.

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

The survival rates in 204 patients suffering from cystic fibrosis observed between January 1956 and June 1976 were recorded. The patients were divided into five groups according to the symptoms present at the time of diagnosis and survival rates were recorded separately for each group. Survival was less good in the group of patients presenting initially with pulmonary symptoms compared to the group presenting with gastrointestinal symptoms. In the group presenting with meconium ileus survival was poor initially, but later in the course of the disease it became similar to that observed in the other groups. The differences in survival between the different categories occurred during the first two years after diagnosis. The study shows that, on clinical grounds, a severe form of the disease which is characterized by early manifestation of pulmonary symptoms and poor survival can be distinguished from a more protracted form with a better survival. Because of the great inter-individual variability large numbers of patients will have to be evaluated in order to achieve statistically significant results in studies which attempt to compare different therapeutic approaches. If such numbers cannot be reached, it may be necessary to compare only patients who belong to the same symptomatic category.

Topics: Child, Preschool; Cystic Fibrosis; Gastrointestinal Diseases; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Lung Diseases; Meconium; Switzerland

Twelve patients with meconium ileus equivalent complicating cystic fibrosis in the postneonatal period were seen at the Mayo Clinic in the years 1950 through 1975. In a child or young adult with known or suspected cystic fibrosis, the triad of recurrent colicky abdominal pain, a mass in the right lower quadrant, and mechanical intestinal obstruction provides a clue to diagnosis of meconium ileus equivalent. The clinical suspicion of meconium ileus equivalent may be confirmed by meglumine diatrizoate (Gastrografin) enema, which in most uncomplicated cases also serves as treatment.

Topics: Adolescent; Adult; Barium Sulfate; Child; Child, Preschool; Cystic Fibrosis; Diatrizoate Meglumine; Enema; Female; Humans; Infant; Intestinal Obstruction; Male; Meconium; Minnesota; Pain

In this study the clinical data and treatment concept in 197 patients with cystic fibrosis, seen during the past 20 years at the University Children's Hospital of Berne (Switzerland), are presented. The results showed that during recent years the median age of survival, the mean age at death and the cumulative survival rates have improved. Furthermore, this study demonstrates that by forming groups of patients with similar age at diagnosis the cumulative survival rate declined in the group with early diagnosis much more markedly than in the group of patients with later diagnosis. Further evaluation of these data revealed that most patients of the former group had a more severe form of cystic fibrosis than the latter group. Since there are different patterns of severity in this disease, it will be necessary to develop staging for the purpose of statistical comparisons.

Topics: Adolescent; Age Factors; Child; Child, Preschool; Cystic Fibrosis; Follow-Up Studies; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Switzerland

Apparently spontaneous neonatal intestinal perforation may be difficult to diagnose if the condition is not kept in mind. It may present with free perforation or with the late effects of meconium peritonitis. Five cases are described. There is a high mortality associated with bacterial peritonitis, and the importance of early diagnosis and energetic resuscitation is underlined.

Topics: Colonic Diseases; Female; Humans; Ileum; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Jejunum; Male; Meconium; Peritonitis; Rupture, Spontaneous; Stomach Rupture

Neonatal small left colon syndrome is a cause of functional colon obstruction in newborn infants, with a high association of maternal diabetes. There has been much confusion regarding its relationship to meconium plug syndrome and aganglionosis. Six cases of aganglionosis (2 in infants of diabetic mothers) were encountered in which the radiographic findings were identical to small left colon syndrome, indicating the need for caution in dismissing aganglionosis solely on the basis of a small left colon seen radiographically in a newborn infant with colon obstruction. Small left colon and meconium plug syndrome are overlapping entities in the total spectrum of functional intestinal obstruction in newborn infants.

Topics: Adolescent; Adult; Colon; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Megacolon; Pregnancy; Pregnancy in Diabetics; Radiography; Syndrome

Meconium ileus is in most cases difficult to diagnose preoperatively; however, aganglionosis should be excluded in every case (rectal double-suction biopsy, manometry). In cases of uncomplicated meconium ileus (no perforation, no further intestinal obstructions) lysis of the meconium by a Gastrografin enema is tried first. In case of no relief, an intraoperative puncture of the bowel and Gastrografin instillation is performed. If there is no relief a small bowel resection is performed and a Bishop-Koop anastomosis or a chimney anastomosis established. A distinction must be made between meconium ileus, meconium disease, meconium plug syndrome, and curd obstruction. Results show a survival rate of 80%.

Topics: Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Methods

Topics: Adult; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Radiography; Syndrome

Topics: Acute Disease; Antifungal Agents; Candida albicans; Candidiasis; Cystic Fibrosis; Flucytosine; Fungemia; Humans; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Jaundice; Male; Meconium; Rare Diseases; Vomiting

Topics: Celiac Disease; Child; Child, Preschool; Cystic Fibrosis; Diagnosis, Differential; Humans; Infant; Intestinal Obstruction; Iontophoresis; Meconium; Pilocarpine; Prognosis; Rectal Prolapse

Thirty dated pregnant Srague-Dawley rats received IM MgSO4 (750 mg/kg/ X 5 days) until birth. Fifteen untreated pregnant Sprague-Dawley rats of similar gestation served as controls. A total of 453 rats were born. Ninety neonates (two from each of 45 liters) were randomly removed before suckling, sacrificed, and the water content of the meconium and serum magnesium determined. The remaining 363 bewborn rats were observed for delayed passage of meconium or abdominal distention. Hypermagnesemic (6.3 +/- 0.5 mg/100 ml) neonates had neither abdominal distention nor delayed passage of meconium. The water content of meconium in hypermagnesemic rats (84.7 +/- 4.2% and in untreated controls (85.5 +/- 3.8%) were statistically similar. Dated pregnant mongrel dogs were given magnesium sulfate by continuous intravenous infusion and by bolus intravenous injection. The water content of meconium, transit time, and basic electrical rhythm (BER) of treated neonates were similar to untreated neonatal controls. The water content of meconium, transit time, and BER of neonatal dogs made hypermagnesemic (12.0 +/- 1.2 mg/100 ml) in the postnatal period were similar tp untreated neonatal controls. In adult and newborn pupies, receiving a continuous intravenous magnesium sulfate infusion, deep tendon reflexes ceased at serum magnesium levels of 9.0-10.0 mg/100 ml, spontaneous respiration at 14.0-15.0 mg/100 ml, and BER was suppressed (transiently) at 20.0-25.0 mg/10 ml. These data suggest that high (sublethal) doses of MgSO4 in pregnant and newborn animals have little effect on BER, meconium transit time or water content of the neonates. No correlation between MgSO4 treatment and the meconium plug syndrome was observed in the experiments.

Topics: Animals; Animals, Newborn; Colonic Diseases; Dogs; Female; Intestinal Obstruction; Magnesium; Meconium; Pregnancy; Pregnancy Complications; Rats; Syndrome

Topics: Humans; Ileum; Infant, Newborn; Intestinal Obstruction; Mathematics; Meconium; Viscosity; Water

Gastrografin (methylglucamine diatrizoate) enemas were carried out in 2 newborn infants with meconium ileus. Evacuation was slow and incomplete. Both patients died within 72 hours following enemas from bowel necrosis, perforation and peritonitis. Although it is not possible to implicate Gastrografin directly as the cause, it is suggested that it may have contributed substantially to bowel necrosis. Recent experimental evidence of colonic inflammation and occasionally necrosis caused by Gastrografin lends support to this hypothesis. Caution should be exercised to prevent not only the systemic osmotic effects of Gastrografin, but also potential local injury to the bowel, especially when underlying disease interferes with intestinal viability.

Topics: Diatrizoate; Diatrizoate Meglumine; Enema; Female; Humans; Infant, Newborn; Intestinal Diseases; Intestinal Obstruction; Meconium; Necrosis; Radiography

Topics: Diatrizoate; Enema; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium

Topics: Apgar Score; Diseases in Twins; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Radiography

The incidence of and mortality from cystic fibrosis in New Zealand for the years 1965-1971 have been reviewed. Data were obtained from hospital admission and death records. All of the 171 cases were of European parentage except for one part-Maori child. The minimum incidence of cystic fibrosis in the European population (one in 3185 live births) is comparable with that in other populations of similar background but somewhat lower than that derived by Becroft (1968). The data show that, during the period of the survey, approximately 30 percent of cystic fibrosis suffereres died at less than 12 months of age.

Topics: Child; Child, Preschool; Cystic Fibrosis; Ethnicity; Hospitalization; Humans; Infant; Intestinal Obstruction; Meconium; New Zealand; Residence Characteristics

Experimental evidence suggests that Gastrografin in the neonatal gastrointestinal tract leads to hypertonic dehydration by two mechanisms: loss of water from the circulating blood volume, and absorption of osmolarly active substances across intact, normal mucosa of the gastrointestinal tract. Clinical documentation of absorption of Gastrografin is presented. Renal excretion of contrast material was seen following its use in the treatment of meconium ileus.

Topics: Diatrizoate; Diatrizoate Meglumine; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Absorption; Intestinal Obstruction; Kidney; Meconium; Radiography, Abdominal; Urinary Bladder

Topics: Enterocolitis, Pseudomembranous; Female; Humans; Ileum; Infant; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Intestines; Jejunum; Male; Meconium

Topics: Colonic Diseases, Functional; Diagnosis, Differential; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Intestinal Obstruction; Meconium; Megacolon; Pregnancy; Pregnancy in Diabetics; Radiography

An incidence of the genetic recessive disease cystic fibrosis (mucoviscidosis) far in excess of that reported recently from other countries, has been encountered in the South West African Afrikaner. This has probably resulted from the immigration of a segment of the South African Afrikaner population rich in the gene, into South West Africa, where, for religious reasons and reasons of geographical isolation, the gene has persisted and, perhaps, increased in frequency. Malaria, which killed many of the early settlers, might have selectively spared carriers of the gene, thus enriching its occurrence in the population. Details of patients, particularly with regard to the criteria of diagnosis, are given with the relevant population and birth figures, from which an estimate of the incidence of the disease and of its carrier rate has been made. Screening of the newborn for the condition and the compiling of a register of potential and obligatory carriers are also discussed.

Topics: Africa, Southern; Child; Cystic Fibrosis; Gene Frequency; Heterozygote; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Mass Screening; Meconium; White People

Topics: Child; Child, Preschool; Cough; Cystic Fibrosis; Diet; Drainage; Humans; Infant; Infant, Newborn; Intestinal Absorption; Intestinal Obstruction; Liver Cirrhosis; Meconium; Psychology; Respiratory Tract Infections

Topics: Diagnosis, Differential; Enterocolitis, Pseudomembranous; Female; Humans; Infant, Newborn; Intestinal Obstruction; Jejunum; Male; Meconium; Megacolon; Radiography

A child diagnosed as having cystic fibrosis by customary criteria has been given regular parenteral soya oil emulsion from near birht. Sweat tests have improved, pancreatic achylia was relieved, and the child at present remains entirely well. Correction of the essential fatty acid deficiency found in cystic fibrosis may prevent some of the manifestations of the disease.

Topics: Cystic Fibrosis; Emulsions; Female; Glycine max; Humans; Ileostomy; Infant; Infant, Newborn; Infusions, Parenteral; Intestinal Obstruction; Meconium; Oils; Pancreatic Extracts; Surgical Wound Infection; Sweat

Intestinal surgery in the foal is seldom necessary but some conditions in which it is essential are well recognized. The foal is a good surgical subject but success depends mainly on operating promptly before irreversible degenerative changes take place. Exploratory laparatomy is recommended whenever mechanical obstruction is suspected. Indications fall broadly into two main categories: 1. Cases arising from congenital defects. These form a miscellaneous group and present considerable difficulties in diagnosis and effective treatment. 2. Cases related to acute onset of obstruction, mainly due to volvulus or torsion. By far the most important condition in this category is volvulus of the ileum close to the ileocaecal valve. Aetiological factors are discussed, and the value of resection of affected small intestine and creation of a new jejuno-caecal opening is indicated. It is suggested that with improvement of such techniques it will be possible to save a higher proportion of foals suffering from mechanical intestinal obstruction.

Topics: Abdomen; Anesthesia; Animals; Cecal Diseases; Duodenal Obstruction; Hernia; Herniorrhaphy; Horse Diseases; Horses; Intestinal Diseases; Intestinal Obstruction; Intestine, Large; Intestines; Intussusception; Meconium; Postoperative Care; Pyloric Stenosis; Torsion Abnormality

Intraluminal bowel obstruction secondary to inspissated feces is a known complication of cystic fibrosis. When seen in the older child, it is termed "meconium ileus equivalent." We studied a case in which nonsurgical resolution of the obstruction was obtained with N-acetylcysteine enemas and pancreatic replacement enzymes given orally and by enema. The pathogenesis of this disorder and the basis for the treatment are described. Recognition of this complication and familiarity with its medical management are important in caring for the older child with cystic fibrosis.

Topics: Acetylcysteine; Child; Cystic Fibrosis; Enema; Feces; Female; Humans; Intestinal Obstruction; Lipase; Meconium

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium

The past 5 years have seen a continuing improvement in life expectancy in cystic fibrosis.

Topics: Adolescent; Age Factors; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Prognosis

Eleven cases of intestinal occlusion (4 personal and 7 culled from hospital records) are presented. The importance of certain symptoms, early diagnosis and the need for minimum trauma in surgical management are discussed.

Topics: Cystic Fibrosis; Digestive System Abnormalities; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium

Topics: Age Factors; Child; Child, Preschool; Cystic Fibrosis; Germany, East; Humans; Infant; Intestinal Obstruction; Mass Screening; Meconium; Prognosis

Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Diseases in Twins; Female; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Life Expectancy; Longitudinal Studies; Male; Meconium; Pregnancy; Racial Groups; Registries; Sex Factors; Twins, Dizygotic; Twins, Monozygotic; United States

Topics: Appendicitis; Barium Sulfate; Constipation; Diverticulum; Duodenal Diseases; Duodenal Obstruction; Duodenum; Enema; Humans; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestine, Small; Meconium; Megacolon; Preoperative Care; Radiography; Syndrome

Topics: Diatrizoate; Enema; Female; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Radiography

Topics: Abnormalities, Multiple; Anal Canal; Child, Preschool; Colostomy; Female; Humans; Infant; Intestinal Obstruction; Intestine, Large; Kidney; Male; Meconium; Penis; Rectal Fistula; Sacrum; Spine; Urethra; Urinary Bladder; Urogenital Abnormalities; Vagina

Topics: Child, Preschool; Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Radiography

Topics: Barium Sulfate; Colon; Colonic Diseases; Diagnosis, Differential; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Megacolon; Radiography

Topics: Colonic Diseases; Diagnosis, Differential; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Megacolon; Radiography

Topics: Calcinosis; Constipation; Female; Fetal Diseases; Gastrointestinal Motility; Humans; Infant; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Pregnancy; Radiography, Abdominal; Time Factors; Tissue Adhesions; Vomiting

Topics: Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Radiography; Therapeutic Irrigation

Topics: Cystic Fibrosis; Female; Gene Frequency; Genes, Recessive; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Polymorphism, Genetic; Russia; Urban Population

Topics: Ileum; Intestinal Obstruction; Intestine, Small; Meckel Diverticulum; Meconium; Megacolon

Topics: Adolescent; Adult; Biopsy; Calculi; Colonic Diseases; Cystic Fibrosis; Esophageal and Gastric Varices; Female; Gallbladder Diseases; Gastrointestinal Diseases; Humans; Intestinal Mucosa; Intestinal Obstruction; Male; Meconium; Pneumatosis Cystoides Intestinalis; Splenomegaly

Topics: Diatrizoate; Enema; Fluoroscopy; Humans; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Intestinal Obstruction; Meconium; Methods; Pneumonia

Topics: Amino Sugars; Animals; Animals, Newborn; Diatrizoate; Disease Models, Animal; Dogs; Enema; Intestinal Obstruction; Meconium; Sorbitol

Topics: Cystic Fibrosis; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Lung Diseases, Obstructive; Male; Meconium; Pregnancy; Prognosis; Rectal Prolapse; Sweat; Trypsin

Topics: Candida; Candidiasis; Child, Preschool; Colon; Colonic Diseases; Cystic Fibrosis; Duodenum; Female; Humans; Hyperplasia; Infant; Infant, Newborn; Intestinal Obstruction; Liver Cirrhosis, Biliary; Male; Meconium; Pancreas; Submandibular Gland

Topics: Animals; Diagnosis, Differential; Humans; Infant Nutritional Physiological Phenomena; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Milk; Radiography; Syndrome

Topics: Calcinosis; Female; Fetal Diseases; Hernia; Humans; Infant; Intestinal Obstruction; Intestinal Perforation; Jejunum; Male; Meconium; Peritonitis; Pregnancy; Radiography; Tissue Adhesions

Topics: Contrast Media; Enema; Female; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Radiography

Topics: Amniotic Fluid; Bile; Female; Fetal Diseases; Humans; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Meconium; Polyhydramnios; Pregnancy; Vomiting

Topics: Anal Canal; Body Fluids; Body Water; Body Weight; Deuterium; Duodenum; Esophageal Atresia; Hernia, Umbilical; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Jejunum; Meconium; Megacolon; Radioisotope Dilution Technique; Rectum; Tracheoesophageal Fistula; Water

Topics: Acetylcysteine; Amino Sugars; Diatrizoate; Enema; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Radiography; Sorbitol

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Time Factors

Topics: Barium Sulfate; Colon; Enema; Female; France; Ganglia, Autonomic; Humans; Ileostomy; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Megacolon; Prognosis; Radiography; Sex Factors

Topics: Animals; Diatrizoate; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Milk; Radiography

Topics: Enema; Female; Fetus; Gastrointestinal Motility; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Magnesium; Magnesium Sulfate; Meconium; Pregnancy

Topics: Age Factors; Birth Weight; Cystic Fibrosis; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Postoperative Complications; Pyloric Stenosis; Time Factors

Topics: Anti-Bacterial Agents; Child; Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Physical Therapy Modalities

Topics: Catheterization; Female; Humans; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Jejunum; Meconium; Postoperative Complications

Topics: Diagnosis, Differential; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Megacolon; Radiography

Topics: Appendicitis; Child; Child, Preschool; Diagnosis, Differential; Enterocolitis, Pseudomembranous; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Intussusception; Meckel Diverticulum; Meconium; Pancreatitis; Peptic Ulcer Perforation; Peritonitis; Radiography

Topics: Duodenum; Emergencies; Esophageal Atresia; Female; Gastrointestinal Diseases; Hernia, Diaphragmatic; Hernia, Inguinal; Hernia, Umbilical; Hernia, Ventral; Hernias, Diaphragmatic, Congenital; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestinal Perforation; Jejunum; Meconium; Pneumothorax; Pregnancy; Pyloric Stenosis; Renal Veins; Rupture; Stomach Diseases; Thrombophlebitis

Topics: Biopsy; Down Syndrome; Humans; Infant, Newborn; Intestinal Obstruction; Jejunum; Meconium; Megacolon; Methods; United Kingdom

Topics: Animals; Barium Sulfate; Cysteine; Enema; Ileum; Intestinal Obstruction; Intestine, Large; Intestine, Small; Male; Meconium; Radiography; Rats; Viscosity

Topics: Colon; Female; Ganglia; Humans; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Diseases; Intestinal Obstruction; Intestine, Small; Laparotomy; Meconium; Thrombophlebitis; Vena Cava, Inferior

Topics: Autopsy; Bile; Biopsy; Child; Cystic Fibrosis; Female; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Jaundice, Neonatal; Liver; Liver Cirrhosis, Biliary; Male; Meconium

Topics: Acute Disease; Autopsy; Chronic Disease; Colon, Sigmoid; Female; Fetal Diseases; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Male; Meconium; Muscle, Smooth; Peritonitis; Pregnancy; Tissue Adhesions

Topics: Barium; Diagnosis, Differential; Enema; Humans; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Diseases; Intestinal Obstruction; Intestine, Small; Meconium; Megacolon; Radiography

Topics: Acid-Base Equilibrium; Animals; Animals, Newborn; Barium Sulfate; Contrast Media; Dehydration; Depression, Chemical; Diatrizoate; Dogs; Enema; Hemodynamics; Humans; Hypertonic Solutions; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Methods; Plasma Volume; Sodium; Water-Electrolyte Balance

Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Life Expectancy; Meconium; Prognosis

Topics: Duodenal Obstruction; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestine, Small; Meconium; Megacolon; Mesentery

Topics: Anal Canal; Barium Sulfate; Cecum; Child; Colitis; Colon; Diagnosis, Differential; Female; Fluoroscopy; Ganglia, Autonomic; Humans; Ileitis; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Megacolon; Rectal Diseases; Rectum

Topics: Contrast Media; Cystic Fibrosis; Diatrizoate; Enema; Female; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Peritonitis; Radiography

Topics: Female; Follow-Up Studies; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Methods; Postoperative Complications; Radiography

Topics: Diatrizoate; Enema; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Iodides; Meconium; Radiography

Topics: Colonic Diseases; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Radiography

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium; Solvents; Surface-Active Agents

Topics: Diagnosis, Differential; Dilatation; Female; Genital Diseases, Female; Genitalia, Female; Humans; Hymen; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Peritonitis; Radiography

Topics: Anemia; Body Weight; Chloramphenicol; Chlortetracycline; Cough; Cystic Fibrosis; Diabetes Mellitus, Type 1; Diet Therapy; Feces; Humans; Hunger; Hypoproteinemia; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Pancreatic Juice; Pancreatin; Physical Therapy Modalities; Prognosis; Rectal Prolapse; Sweat; Tooth Discoloration; Vomiting

Topics: Cathartics; Cystic Fibrosis; Enema; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Catheterization; Diagnosis, Differential; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Infusions, Parenteral; Intestinal Obstruction; Male; Meconium; Megacolon; Pregnancy; Respiratory Distress Syndrome, Newborn; Sepsis; Umbilical Veins

Topics: Cystic Fibrosis; Diatrizoate; Enema; Female; Humans; Infant, Newborn; Infusions, Parenteral; Intestinal Obstruction; Meconium; Osmolar Concentration; Surface-Active Agents

Topics: Cystic Fibrosis; Diatrizoate; Enema; Female; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Methods; Postoperative Complications; Radiography; Respiratory Tract Diseases

Topics: Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Methods; Prognosis; Time Factors

Topics: Calcinosis; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Peritoneum

Topics: Anti-Bacterial Agents; Catheterization; Cystic Fibrosis; Germany, East; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pneumonia; Postoperative Complications; Staphylococcal Infections

Topics: Abdomen, Acute; Aerosols; Anti-Bacterial Agents; Breathing Exercises; Cough; Cystic Fibrosis; Drainage; Heat Exhaustion; Humans; Intestinal Obstruction; Lung Diseases; Meconium; Mucus; Nasal Polyps; Physical Therapy Modalities; Pneumonia, Staphylococcal; Pneumothorax; Pulmonary Atelectasis; Rectal Prolapse; Respiratory Tract Infections; Sputum

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Methods; Peritonitis; Postoperative Complications

Topics: Acetylcysteine; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Methods

Topics: Abdomen; Abdominal Muscles; Abnormalities, Multiple; Colonic Diseases; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Adult; Animals; Female; Gelatin; Glucose; Glycine; Humans; Infant; Infant Nutritional Physiological Phenomena; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Intestinal Absorption; Intestinal Obstruction; Meconium; Milk; Pregnancy; Radiography; Xylose

Topics: Female; Fetal Diseases; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Laparotomy; Male; Meconium; Peritonitis; Philosophy, Medical; Pregnancy; Prenatal Care; Radiography; Torsion Abnormality

Topics: Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium

Topics: Bone Development; Female; Femur; Humans; Humerus; Ilium; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Peritonitis; Pregnancy; Radiography; Scapula

Topics: Abnormalities, Multiple; Cystic Fibrosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Pancreas; Pancreatic Diseases

Topics: Cystic Fibrosis; Diatrizoate; Enema; Female; Fluoroscopy; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Methods

Topics: Cystic Fibrosis; Diagnosis, Differential; Humans; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Methods; Prognosis

Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Intestinal Obstruction; Male; Meconium; Radiography

Topics: Cysts; Humans; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Male; Meconium; Peritonitis

Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Female; Fetal Diseases; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Meconium; Methods; Pregnancy

Topics: Alpha-Globulins; Cystic Fibrosis; Densitometry; Electrophoresis; Intestinal Obstruction; Meconium; Mucoproteins; Proteins; Serum Albumin

Topics: Duodenal Diseases; Duodenal Ulcer; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Diseases; Intestinal Obstruction; Intestinal Perforation; Intestine, Small; Intussusception; Laparotomy; Meckel Diverticulum; Meconium; Methods; Peritonitis; Suture Techniques; Time Factors

Topics: Ampulla of Vater; Cystic Fibrosis; Female; Fetal Diseases; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Male; Meconium; Peritonitis; Polyhydramnios; Pregnancy; Radiography

Topics: Aspartate Aminotransferases; Bilirubin; Blood Glucose; Cholestasis; Humans; Hypoglycemia; Infant, Newborn; Intestinal Obstruction; Jaundice, Neonatal; Jejunum; Liver; Male; Meconium; Prednisolone

Topics: Adult; Bronchiectasis; Cystic Fibrosis; Humans; Intestinal Obstruction; Intestines; Liver Function Tests; Male; Meconium; Radiography; Respiratory Tract Infections

Topics: Barium; Colon; Fetus; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Meconium; Radiography; Time Factors

Topics: Diagnosis, Differential; Enema; Female; Humans; Hypoxia, Brain; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Megacolon; Radiography

Topics: Humans; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Radiography

Topics: Albumins; Child; Child, Preschool; Cystic Fibrosis; Electrophoresis; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Nitrogen; Proteins; Sodium; Sweat

Topics: Acetylcysteine; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intubation, Gastrointestinal; Meconium; Radiography

Topics: Adult; Female; Fetal Diseases; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Pancreatic Diseases; Peritonitis; Pregnancy

Topics: Abdomen, Acute; Collateral Circulation; Cystic Fibrosis; Female; Fetal Diseases; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Intestines; Male; Meconium; Peritoneum; Peritonitis; Pneumoperitoneum; Pregnancy; Radiography, Abdominal; Sepsis; Stomach Rupture

Topics: Enema; Humans; Hydrogen Peroxide; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Barium Sulfate; Biopsy; Chymotrypsin; Diagnosis, Differential; Feces; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Intestinal Obstruction; Intestine, Small; Male; Meconium; Megacolon; Radiography; Trypsin

Topics: Animals; Dogs; Gas Gangrene; Humans; Hydrogen Peroxide; Infant, Newborn; Infusions, Parenteral; Intestinal Obstruction; Male; Meconium; Mesenteric Vascular Occlusion; Postoperative Complications

Topics: Colostomy; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Parasympathetic Nervous System

Topics: Acetylcysteine; Adult; Cystic Fibrosis; Enema; Humans; Intestinal Obstruction; Male; Meconium; Mucus; Pancreas; Pharmaceutical Vehicles

Topics: Barium Sulfate; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Radiography, Abdominal

Topics: Animals; Dogs; Female; Humans; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Absorption; Intestinal Atresia; Intestinal Diseases; Intestinal Obstruction; Intestine, Small; Jejunum; Male; Meconium; Postoperative Complications; Rabbits; Rats; Swine

Topics: Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium; Radiography

Topics: Abdominal Muscles; Anus, Imperforate; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Intestines; Male; Meconium; Megacolon

Topics: Cholestasis; Cystic Fibrosis; Esophageal Perforation; Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Male; Meconium; Polyhydramnios; Pregnancy; Prognosis

Topics: Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium

Topics: Anti-Bacterial Agents; Cysteine; Cystic Fibrosis; Diet Therapy; Humans; Intestinal Obstruction; Meconium; Pulmonary Fibrosis; Radiography; Respiratory Therapy

Topics: Abdomen, Acute; Child, Preschool; Cystic Fibrosis; Duodenal Diseases; Esophagus; Female; Hirschsprung Disease; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Intestinal Obstruction; Intestinal Perforation; Male; Meckel Diverticulum; Meconium; Posture; Pyloric Stenosis; Radiography

Topics: Diagnosis, Differential; Female; Humans; Ileostomy; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium

Topics: Congenital Abnormalities; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestines; Male; Meconium

Topics: Acetylcysteine; Anti-Bacterial Agents; Bronchi; Bronchial Diseases; Child; Cystic Fibrosis; Digestive System; Drainage; Fecal Impaction; Humans; Hyperglycemia; Intestinal Obstruction; Liver Cirrhosis; Lung; Lung Diseases; Meconium; Mucus; Pneumatosis Cystoides Intestinalis; Respiratory Function Tests; Respiratory Insufficiency; Respiratory Therapy; Respiratory Tract Diseases; Salivary Glands; Sweat

Topics: Female; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Male; Meconium

Topics: Child, Preschool; Chlorides; Cystic Fibrosis; Female; Humans; In Vitro Techniques; Infant; Infant, Newborn; Intestinal Obstruction; Japan; Male; Meconium; Sodium; Sweat

Topics: Adult; Cystic Fibrosis; Humans; Intestinal Obstruction; Male; Meconium

Topics: Child, Preschool; Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Male; Meconium

Topics: Adolescent; Appendicitis; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Ileostomy; Infant; Intestinal Obstruction; Male; Meconium; Water-Electrolyte Balance

Topics: Anti-Bacterial Agents; Colitis; Enema; Female; Hernia, Umbilical; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Male; Meconium; Peritonitis; Pneumoperitoneum

Topics: Cysts; Female; Fetal Diseases; Humans; Hyperbilirubinemia; Infant, Newborn; Intestinal Obstruction; Intestinal Perforation; Intestine, Small; Meconium; Obstetric Labor Complications; Perineum; Peritonitis; Pregnancy

Topics: Ascites; Cysts; Female; Humans; Infant; Intestinal Obstruction; Laparotomy; Male; Meconium; Peritonitis

Topics: Chymotrypsin; Feces; Humans; Infant, Newborn; Intestinal Obstruction; Meconium; Pancreas; Pancreatic Diseases; Trypsin

Topics: Duodenal Obstruction; Humans; Infant; Intestinal Obstruction; Jejunum; Meconium; Megacolon; Posture; Radiography; Vomiting

Topics: Adolescent; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Intestinal Obstruction; Male; Massachusetts; Meconium

Topics: Colonic Diseases; Diagnosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Laparotomy; Meconium; Postoperative Care; Radiography; Surgical Procedures, Operative

Topics: Congenital Abnormalities; Cystic Fibrosis; Diagnosis; Female; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Meconium; Microbiology; Pathology; Polyhydramnios; Postoperative Complications; Pregnancy; Prognosis; Radiography; Statistics as Topic; Surgical Procedures, Operative

Topics: Cystic Fibrosis; Fetal Diseases; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Prognosis; Surgical Procedures, Operative

A mass of inspissated meconium in the distal colon or rectum is a relatively common cause of neonatal intestinal obstruction. The meconium-plug syndrome is unrelated to cystic fibrosis and meconium ileus. The clinical picture is frequently indistinguishable, without contrast study of the colon, from other forms of mechanical intestinal obstruction requiring laparotomy. A barium enema examination is almost always diagnostic, and use of this procedure usually results in dislodgement and passage of the plug.No single cause for the excessive viscosity and tenaciousness of the obstructive meconium mass has been identified. Previous reports have generally emphasized the normal ganglion-cell content of the colon in affected patients.Two infants are described who fulfilled all criteria for this syndrome but who were not rendered asymptomatic, as normally anticipated, by removal of the plug. Subsequent studies revealed the presence of Hirschsprung's disease in both patients. This diagnosis should be considered when an infant with meconium-plug obstruction of the colon fails to follow the usual satisfactory clinical course after the plug has been passed.

Topics: Barium Sulfate; Colonic Diseases; Colostomy; Cystic Fibrosis; Enema; Fetal Diseases; Hirschsprung Disease; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Laparotomy; Male; Meconium; Megacolon; Radiography; Rectum; Syndrome

Topics: Fetal Diseases; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Meckel Diverticulum; Meconium; Peritonitis; Surgical Procedures, Operative

Surgeons whose practice involves many infants and children should be acquainted with all abnormalities of pancreatic malformation and function. Conditions amenable to surgical treatment are few, but serious. Trauma to the pancreas in childhood is most commonly diagnosed by fever, leukocytosis, rectus spasm and elevated serum amylase. Drainage of the lesser sac and debridement of devitalized tissue may prevent the sequelae of pseudocyst formation which seems to follow the untreated injury. True congenital cysts are characterized by an epithelial lining.Mucoviscidosis complicated by meconium ileus remains a challenging disease of the newborn that requires early operation. Ten per cent of infants with cystic fibrosis may be threatened by intestinal obstruction from this cause. Some children surviving the newborn period go on to develop obstruction later. Annular malformation of pancreas may produce upper intestinal (duodenal) obstructive symptoms immediately after birth. Surgical correction by duodenojejunostomy should be postponed only long enough to correct severe fluid or electrolyte imbalances. Idiopathic spontaneous hypoglycemia has the most serious prognosis if convulsions are allowed to recur. Increased metabolic rates in infants increase the need for control of blood sugar levels by either administration of cortisone or pancreatic resection. If adenoma is the cause, a conservative resection of the tumor suffices. If serial frozen section fails to reveal either tumor or hypertophy of insulin-producing cells, blind pancreatectomy may be indicated, for irreversible brain damage develops early in uncontrolled hypoglycemia.

Topics: Child; Congenital Abnormalities; Cystic Fibrosis; Drainage; Female; Humans; Hypoglycemia; Infant; Infant, Newborn; Infant, Newborn, Diseases; Insulin; Intestinal Obstruction; Meconium; Neoplasm Recurrence, Local; Pancreas; Pancreatectomy; Pancreatic Cyst; Pancreatic Diseases; Surgical Procedures, Operative

Topics: Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pathology; Radiography

Topics: Anal Canal; Anus, Imperforate; Congenital Abnormalities; Diagnosis; Gastrointestinal Diseases; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Megacolon; Radiography

Topics: Calcinosis; Cystic Fibrosis; Humans; Ileostomy; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature, Diseases; Intestinal Obstruction; Meconium; Radiography

Topics: Diagnosis, Differential; Humans; Ileus; Infant, Newborn; Infant, Premature, Diseases; Intestinal Obstruction; Meconium; Megacolon; Radiography; Surgical Procedures, Operative

Topics: Adolescent; Child; Cystic Fibrosis; Diagnosis; Diet; Diet Therapy; Drug Therapy; Feces; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Lipase; Meconium; Pancreatic Extracts; Pancreatin; Radiography

Topics: Cystic Fibrosis; Diagnosis; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Amino Acids; Biochemical Phenomena; Biochemistry; Chymotrypsin; Diphtheria Toxin; Electrophoresis; Humans; Ileus; Immunoelectrophoresis; Infant, Newborn; Intestinal Obstruction; Meconium; Mucoproteins; Proteins; Trypsin

Two of 15 children who survived neonatal meconium ileus had "meconium ileus equivalent." They were treated with hydration, pancreatic enzyme therapy and antibiotics. One of these children died from pulmonary disease at the time of the bowel obstruction. The survival rate of infants with meconium ileus is steadily improving because of prompt operative intervention, better preoperative and postoperative care and long-term treatment with enzyme supplements and antibiotics. Late intestinal obstruction due to adhesive bands, volvulus, intussusception or "meconium ileus equivalent" may occur in children previously treated for meconium ileus of infancy. The omission of pancreatic enzyme supplementation and the occurrence of respiratory infections are frequently associated with "meconium ileus equivalent."In this series of patients four of the infants treated surgically for neonatal meconium ileus died in the early postoperative period.

Topics: Anti-Bacterial Agents; Child; Cystic Fibrosis; Fetal Diseases; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Intestinal Volvulus; Intussusception; Male; Meconium; Pancreas; Peritoneal Diseases; Postoperative Care; Respiratory Tract Infections; Surgical Procedures, Operative; Survival Rate

Topics: Barium; Barium Sulfate; Cystic Fibrosis; Enema; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Radiography; Radiography, Abdominal

Topics: Cystic Fibrosis; Humans; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis

Topics: Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Adolescent; Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pathology; Postoperative Complications; Prognosis; Surgical Procedures, Operative

Topics: Aerosols; Child; Cystic Fibrosis; Diagnosis; Genetics, Medical; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Pancreatic Extracts; Pathology; Radiography; Respiratory Therapy; Surgical Procedures, Operative

Topics: Humans; Ileum; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Postoperative Care; Radiography; Surgical Procedures, Operative

Topics: Child; Congenital Abnormalities; Duodenal Obstruction; Humans; Infant; Infant, Newborn; Intestinal Atresia; Intestinal Obstruction; Intussusception; Meconium; Surgical Procedures, Operative; Water-Electrolyte Balance

Topics: Calcinosis; Congenital Abnormalities; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Surgical Procedures, Operative

Topics: Congenital Abnormalities; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Pathology; Peritonitis; Surgical Procedures, Operative

Topics: Adolescent; Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Secretions; Meconium; Pancreatic Extracts; Pathology; Postoperative Complications; Surgical Procedures, Operative; Tetracycline

Topics: Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Liver Diseases; Meconium; Megacolon; Peritonitis; Pseudomonas Infections; Radiography; Sepsis

Topics: Adrenal Gland Diseases; Congenital Abnormalities; Emergencies; Esophagus; Hemorrhage; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Meconium; Peritonitis; Radiography

Topics: Acetylcysteine; Child; Cysteine; Cystic Fibrosis; Diagnosis; Humans; Ileus; Infant; Infant Nutritional Physiological Phenomena; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Intussusception; Meconium; Pancreatic Extracts; Rectal Prolapse; Surgical Procedures, Operative

Topics: Acetylcysteine; Cysteine; Cystic Fibrosis; Fetal Diseases; Hernia, Umbilical; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Surgical Procedures, Operative

Topics: Congenital Abnormalities; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pancreas; Pancreatic Neoplasms; Pancreatitis; Surgical Procedures, Operative

Topics: Fetal Diseases; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestinal Perforation; Meconium; Peritonitis; Surgical Procedures, Operative

Topics: Abdomen; Abdomen, Acute; Cystic Fibrosis; Gastrointestinal Diseases; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Meconium; Megacolon; Peritonitis; Surgical Procedures, Operative

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Anti-Bacterial Agents; Child; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Neomycin; Nursing; Pancreatic Extracts; Physical Therapy Modalities; Tetracycline

Topics: Child; Cystic Fibrosis; Exhibitions as Topic; Humans; Hypertension; Hypertension, Portal; Infant; Infant, Newborn; Intestinal Obstruction; Intestines; Liver Cirrhosis; Meconium; Nasal Polyps; Osteoarthropathy, Secondary Hypertrophic; Pancreas; Pneumatosis Cystoides Intestinalis; Pneumothorax; Pulmonary Heart Disease; Radiography; Radiography, Thoracic; Sinusitis

Topics: Child; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Intestinal Secretions; Intestine, Small; Intestines; Meconium; Pathology

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Contrast Media; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Volvulus; Meconium; Peritonitis; Radiography; Surgical Procedures, Operative

Topics: Adolescent; Anti-Bacterial Agents; Antibiotics, Antitubercular; Blood Protein Disorders; Bronchopneumonia; Child; Cystic Fibrosis; Diabetes Mellitus; Diabetes Mellitus, Type 1; Diagnosis; Diagnosis, Differential; Diet; Diet Therapy; Electrolytes; Exercise Therapy; Gastroenterology; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Liver Diseases; Meconium; Pancreatic Extracts; Sweat

Topics: Adrenal Insufficiency; Congenital Abnormalities; Cysts; Hernia, Umbilical; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Diseases; Intestinal Obstruction; Intussusception; Meconium; Megacolon; Mesenteric Vascular Occlusion; Pathology; Peritonitis; Surgical Procedures, Operative

Topics: Abetalipoproteinemia; Celiac Disease; Child; Cystic Fibrosis; Food Hypersensitivity; Glutens; Humans; Infant; Infant, Newborn; Intestinal Absorption; Intestinal Obstruction; Lipid Metabolism; Lipoproteins; Meconium; Povidone; Starch; Syndrome; Terminology as Topic

Topics: Congenital Abnormalities; Diagnosis, Differential; Duodenum; Gastrointestinal Tract; Humans; Ileum; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Radiation Injuries; Radiography; Rectum

Topics: Humans; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Intestines; Meckel Diverticulum; Meconium; Peritonitis; Polyps

Topics: Fetal Diseases; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestine, Small; Meconium; Peritonitis

Topics: Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Child; Colon; Constriction, Pathologic; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Peritonitis

In a review of cases of intestinal atresia or stenosis in the newborn at Children's Hospital of Los Angeles it was noted that in approximately 10 per cent there was clinical or anatomic evidence of fibrocystic disease of the pancreas. Histologic sections of the bowel in all these cases showed the alterations of the mucosa commonly found in fibrocystic disease. Extensive ulceration, foreign body reaction and calcium deposition in the bowel wall were observed in five cases. Sections through the site of obstruction in one patient showed narrowing of the diameter of the bowel with preservation of muscular layers, but replacement of the central portion by a vascular, fibrous diaphragm in which giant cells, hemosiderin and calcium were prominent. This suggested that in certain instances obstruction of the intestine may be caused by fibrosis secondary to injury of the mucosa by abnormal meconium. Awareness of the common association of intestinal obstruction and fibrocystic disease of the pancreas and recognition of the histologic change in the bowel in fibrocystic disease may lead to early diagnosis of this disorder in some infants with intestinal obstruction.

Topics: Child; Constriction, Pathologic; Cystic Fibrosis; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestines; Los Angeles; Meconium; Pancreas

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Meconium

Topics: Child; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Intestines; Meconium

Topics: Child; Cystic Fibrosis; Fetal Diseases; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Fetal Diseases; Humans; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; X-Rays

Topics: Ascites; Child; Dystocia; Female; Fetal Diseases; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Peritonitis; Pregnancy

With the present-day development and understanding of anesthetic methods, fluid and electrolyte therapy, antibiotic medications and pediatric care, many congenital anomalies once uniformly fatal are now being successfully treated by emergency operations in the neonatal period. The eight most common of these which demand emergency operation in the immediate postnatal period are esophageal atresia and tracheoesophageal fistula, diaphragmatic hernia with dislocation of the abdominal viscera into the chest, malrotation of the intestine with obstruction, intestinal atresia, meconium ileus, imperforate anus, omphalocele and myelomeningocele. Although infants born with any of these serious problems often are born prematurely and often have more than one congenital anomaly, survival rates in the surgical treatment of these conditions are steadily improving. Early diagnosis and prompt treatment are the most important factors in the continued improvement of these survival rates.

Topics: Anus, Imperforate; Child; Esophageal Atresia; Fetal Diseases; Hernia, Diaphragmatic; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Atresia; Intestinal Obstruction; Meconium; Tracheoesophageal Fistula

Topics: Child; Cystic Fibrosis; Fetal Diseases; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Proteins

Topics: Child; Digestive System Abnormalities; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Volvulus; Intestines; Meconium

Topics: Child; Humans; Ileus; Infant; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Peritonitis

Topics: Child; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Viscosity

Topics: Anastomosis, Roux-en-Y; Child; Cystic Fibrosis; Disease Management; Humans; Ileostomy; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Pancreas

Topics: Child; Disease; Fetal Diseases; Fetus; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Feces; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Peritonitis

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pulmonary Atelectasis

Topics: Child; Cystic Fibrosis; Fetal Diseases; Humans; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Child; Fetal Diseases; Humans; Ileus; Infant; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Fetal Diseases; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Child; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Any newborn who continues to vomit in the first few days of life, particularly if the vomitus contains bile and if the abdomen is distended, should have immediate investigation because intestinal obstruction in the newborn is a fatal condition unless promptly recognized and surgically corrected. The most common cause of obstruction at this age is atresia and the simplest possible surgical procedure which adequately corrects this deformity should be done. It is also possible to successfully correct the obstruction caused by other congenital deformities such as annular pancreas and meconium ileus. Although prematurity is a definite factor in the outcome, intestinal obstruction in the newborn can be corrected with a surprisingly low mortality. Occasionally unusual methods are needed to tide these infants over the critical period of postoperative care.

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature; Intestinal Obstruction; Meconium; Pancreas; Pancreatic Diseases

Topics: Child; Humans; Ileus; Infant; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Colostomy; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Abscess; Child; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Peritonitis

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Disease; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestine, Small; Meconium; Pancreas; Pancreatic Diseases; Survivors

Topics: Child; Cystic Fibrosis; Disease; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Diseases; Intestinal Obstruction; Intestines; Meconium; Pancreas; Pancreatic Diseases

Topics: Child; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Peritonitis

Topics: Child; Cystic Fibrosis; Disease; Humans; Ileum; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Volvulus; Meconium; Pancreas; Pancreatic Diseases; Peritonitis

Topics: Child; Humans; Infant; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Child; Fetal Diseases; Humans; Ileus; Infant; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Disease; Humans; Hydrogen Peroxide; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pancreas; Pancreatic Diseases

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Disease; Humans; Ileus; Infant; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pancreas; Pancreatic Diseases; Twins

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature; Infant, Premature, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Fetal Diseases; Humans; Ileus; Infant; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Colon; Disease; Exocrine Pancreatic Insufficiency; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pancreas; Pancreatic Diseases

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Dystocia; Female; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Peritonitis; Pregnancy

Topics: Child; Cystic Fibrosis; Cysts; Disease; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pancreas; Pancreatic Diseases

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Meconium

Topics: Child; Congenital Abnormalities; Digestive System Abnormalities; Gastrointestinal Tract; Humans; Ileum; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Fetal Diseases; Humans; Ileus; Infant; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Disease; Humans; Ileus; Infant; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pancreas; Pancreatic Diseases

Topics: Child; Colon; Humans; Ileus; Infant; Infant, Newborn; Intestinal Obstruction; Meconium; Pancreas

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Health Services; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestines; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Child; Cystic Fibrosis; Humans; Ileus; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Hirschsprung Disease; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Megacolon; Mesentery

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pancreas

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Pancreas

Topics: Blood; Blood Group Antigens; Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium; Polysaccharides

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Intestine, Small; Intestines; Meconium; Pancreas

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Meconium

Because the clinical appearance of newborn infants having intestinal obstruction is disarmingly normal, vomiting is reason for immediate search for the cause. To this end the character of stools and meconium that are passed should be carefully observed, lest valuable time elapse before correct diagnosis is made. In three cases of volvulus observed by the authors, there was moderate to pronounced distention of the abdomen at birth-a sign which may be helpful in diagnosis. Roentgenograms are the most helpful diagnostic aid. Since the roentgenographic appearance of the normal infant abdomen differs from that of the adult, interpretations should be made with that in mind. In this connection the absence of gas shadows is significant. Although there are dangers in the use of barium in infants, early diagnosis is so important that use of the substance is justifiable if it will help in correct appraisal. The treatment is always surgical, and the procedure of choice is primary anastomosis. Proper preoperative and postoperative care and treatment, including maintenance of fluid and electrolyte balance and blood volume, are of great importance.

Topics: Adult; Disease; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestinal Volvulus; Intestines; Meconium; Vomiting

Topics: Abscess; Fetal Diseases; Humans; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Intestines; Meconium; Peritonitis

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Meconium

Topics: Celiac Disease; Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Intestinal Obstruction; Meconium

Topics: Cystic Fibrosis; Humans; Ileus; Infant, Newborn; Intestinal Obstruction; Intestinal Pseudo-Obstruction; Intestines; Meconium

Topics: Blood Group Antigens; Humans; Infant, Newborn; Intestinal Obstruction; Intestines; Meconium; Rh-Hr Blood-Group System