morphine and Ileus

Meconium ileus (MI) is one presenting manifestation of Cystic Fibrosis (CF), classically associated with class I-III CF transmembrane conductance regulator (CFTR) mutations and pancreatic insufficiency (PI). D1152H is a class IV mutation that corresponds with a milder CF phenotype and pancreatic sufficiency (PS). We present the case of an infant with G542X/D1152H mutations and MI who required surgical intervention with small bowel resection. The sweat testing was normal, and this child presently remains PS, however at age 5 continues to experience short gut syndrome and failure to thrive. Eight cases were identified in the CF Registry and seven cases in the literature describing patients with D1152H and echogenic bowel (EB) or MI. Our case highlights the importance of CFTR gene sequencing in infants with EB or MI and sweat testing not suggestive of CF. It is our practice to perform full CFTR gene sequencing for infants who present with MI, recognizing protocols for newborn screening across the United States vary. Increased awareness of D1152H association with PS may also well inform both prenatal and postnatal genetic counseling.

Topics: Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Ileus; Infant; Infant, Newborn; Meconium; Meconium Ileus; Mutation; Phenotype; Pregnancy

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

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 ileus (MI) is the earliest clinical manifestation of cystic fibrosis (CF), occurring in up to 20% of patients with CF. Our aim was to review and integrate current knowledge about the diagnosis and management of fetuses and neonates with MI that may aid the pediatric surgeon in caring for these patients.. We identified areas of interest including pathophysiology, prenatal diagnosis, nonoperative and operative management, postoperative management, and prognosis. We performed a Medline search using the search term meconium ileus for English language articles published in the last 20 years. We reviewed reference lists to identify other articles of historical significance.. Meconium ileus is primarily associated with CF transmembrane (conductance) regulator mutations F508del, G542X, W1282X, R553X, and G551D, and modifier genes have been found to explain approximately 17% of the phenotypic variability. Mouse, pig, and ferret models for CF demonstrate neonatal bowel obstruction mimicking MI. Sonographic findings of hyperechoic masses and dilated bowel in a high-risk fetus are suggestive of MI. Less than 7% of low-risk fetuses with hyperechoic bowel will have MI. Contemporary series of noninvasive management with Gastrografin enema report success rates of 36% to 39%, significantly lower than historical values. The optimal surgical technique remains controversial, although primary anastomosis results in surgical complication rates between 21% and 31%, higher than those noted with delayed anastomosis. Pulmonary function for patients with CF and MI at 15 and 25 years old is similar to those without MI, although height and weight percentiles may be lower.. This review for pediatric surgeons presents an examination of the literature and synthesizes current information about the pathophysiology, prenatal diagnosis, nonoperative and operative management, postoperative management, and prognosis of the patient with CF and MI.

Topics: Amniocentesis; Anastomosis, Surgical; Colon; Colonic Diseases; Cystic Fibrosis; Enema; Female; Fetal Diseases; Humans; Ileus; Infant, Newborn; Meconium; Pregnancy; Prognosis; Ultrasonography, Prenatal

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

To extend previous evaluations of costs of cystic fibrosis (CF) diagnosis and examine key issues in assessing the CF cost of care.. Costs for CF newborn screening (NBS) including CF multi-mutation testing are analyzed by using data from the Wisconsin State Laboratory of Hygiene. Electronic data from 2 Wisconsin CF centers are used to illustrate the complexity of analyzing CF health care utilization and costs.. The current cost-per-newborn of a CF multi-mutation test is 50% higher than testing for a single mutation. Data collection for the cost-of-care study requires a combination of electronic and manual data collection; modeling of cost data requires consideration of any censoring. Hospitalizations are shown to have a large impact on costs and show high variability at the individual level. Sixty-nine percent of children with meconium ileus had some hospitalization versus 56% of children without meconium ileus.. A cost-benefit analysis of CF multi-mutation testing is warranted. The study of health care cost data is complex and utilization varies between children. Individual-level modeling of CF costs must include factors contributing to the severity of the disease and allow for consideration of individual-level utilization, such as the number of hospitalizations.

Topics: Cost of Illness; Cost-Benefit Analysis; Cystic Fibrosis; DNA Mutational Analysis; Health Care Costs; Hospitalization; Hospitals, Pediatric; Hospitals, University; Humans; Ileus; Immunoassay; Infant, Newborn; Length of Stay; Meconium; Models, Econometric; Neonatal Screening; Severity of Illness Index; Wisconsin

Although meconium ileus (MI) is the earliest manifestation of cystic fibrosis (CF), and is associated with poorer growth, the longitudinal pulmonary progression of CF children with MI is not clear. To test the hypothesis that MI is associated with worse pulmonary outcomes, we prospectively compared from diagnosis to 12 years of age 32 CF children with MI to 50 CF children without MI who were diagnosed during early infancy through neonatal screening. Pulmonary outcome measures included respiratory symptoms, respiratory infections, pathogens, antibiotic usage, hospitalizations, quantitative chest radiology, spirometry, and lung volume determinations. Obstructive lung disease was defined as percent predicted spirometry values below the lower limits of normal. Longitudinal analyses revealed no significant differences in cough, wheezing, respiratory infections, prevalence of and median times to acquisition of Pseudomonas aeruginosa or Staphylococcus aureus, antibiotic usage, and chest radiograph scores between the two groups. However, MI children showed significantly worse forced expiratory volume in 1 sec (FEV(1)), forced vital capacity (FVC), forced expiratory flow between 25-75% of FVC (FEF(25-75)), % predicted FEV(1), % predicted FEF(25-75), and total lung capacity (TLC). These differences were particularly apparent beginning at age 8-10 years. MI children also had higher rates of and shorter median times to obstructive lung disease. Subgroup analyses showed MI children treated surgically and those treated medically had similar pulmonary outcomes. In conclusion, MI children have worse lung function and more obstructive lung disease than those without MI. Such abnormalities are accompanied by reduced lung volume. MI is a distinct CF phenotype with more severe pulmonary dysfunction.

Topics: Airway Obstruction; Cystic Fibrosis; Female; Humans; Ileus; Infant; Infant, Newborn; Lung; Lung Diseases; Male; Meconium; Neonatal Screening; Prognosis; Prospective Studies; Respiratory Function Tests; Time Factors; Wisconsin

We experienced an increased incidence of meconium-related ileus (MRI) in extremely premature infants (EPIs) while adopting the antenatal magnesium sulfate (MgSO4) protocol for fetal neuroprotection in our neonatal intensive care unit. This study aimed to test whether antenatal MgSO4 use was associated with increased risk of MRI in EPIs.. The incidences of complicated MRI requiring aggressive enema or surgical intervention and other intestinal complications were compared among period 1 (January 2012-December 2013, n = 79), before adoption of the antenatal MgSO4 protocol for fetal neuroprotection; period 2 (January 2014-March 2016, n = 72), when the protocol was adopted; and period 3 (April 2016-September 2018, n = 75), when the protocol was temporarily withdrawn due to concern regarding intestinal complications in EPIs.. Despite similar baseline clinical characteristics among infants across the study periods, the MRI and MRI with surgical treatment incidences were higher in period 2 than those in periods 1 and 3 (13% vs. 8% and 6%, p = 0.391, and 11% vs. 0% and 1%, p = 0.001, respectively). In multivariable analysis, exposure to antenatal MgSO4 independently increased the risk of MRI (adjusted odds ratio, 3.8; 95% confidence interval, 1.4, 10.6).. Antenatal MgSO4 may increase the risk of MRI, frequently requiring surgical intervention, in EPIs with a gestational age of 25 weeks or less.

Topics: Female; Gestational Age; Humans; Ileus; Infant; Infant, Extremely Premature; Infant, Newborn; Magnesium Sulfate; Meconium; Pregnancy

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

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

Very low birth weight (VLBW) neonates experience various problems, including meconium-related ileus (MRI). This study investigated the risk factors of MRI and surgical MRI in VLBW infants. VLBW neonates admitted to the Neonatal Intensive Care Unit of Seoul National University Children's Hospital from October 2002 to September 2016 were included in the study. The diagnostic criteria for MRI were a decreased frequency of defecation with intolerable feeding, vomiting, and increased gastric residue (>50%); meconium-filled bowel dilatation in an imaging study; and no evidence of necrotizing enteritis or spontaneous intestinal perforation. Medical MRIs and surgical MRIs were managed through conventional treatment and surgical intervention. Of 1543 neonates, 69 and 1474 were in the patient and control groups, respectively. The risk factors for MRI include low birth weight (BW), cesarean section delivery, fetal distress, maternal diabetes, maternal hypertension, and maternal steroid use. Low BW and fetal distress were independent risk factors for MRI. Compared to the medical MRI group (n = 44), the risk factors for surgical MRI (n = 25) included males, younger gestational age, low BW, and meconium located at the small bowel. Male gender and low BW were independent risk factors for surgical MRI. Low BW and fetal distress were independent risk factors for MRI and male gender and low BW were independent risk factors for surgical MRI. In VLBW neonates, careful attention to the risk factors for MRI could minimize or avoid surgical interventions.

Topics: Apgar Score; Birth Weight; Case-Control Studies; Disease Susceptibility; Enterocolitis, Necrotizing; Female; Gestational Age; Humans; Ileus; Infant, Extremely Low Birth Weight; Male; Meconium; Prognosis; Republic of Korea; Risk Assessment; Risk Factors

Very low birth weight infants (VLBWIs) are at risk of surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI). We conducted this study to verify whether the timing of stoma closure and that of enteral nutrition establishment after stoma closure in VLBWIs differ among the most common disorders.. A retrospective multicenter study was conducted at 11 institutes. We reviewed the timing of stoma closure and enteral nutrition establishment in VLBWIs who underwent stoma creation for intestinal disorders.. We reviewed the medical records of 73 infants: 21 with NEC, 24 with FIP, and 25 with MRI. The postnatal age at stoma closure was 107 (28-359) days for NEC, 97 (25-302) days for FIP, and 101 (15-264) days for MRI (p = 0.793), and the postnatal age at establishment of enteral nutrition was 129 (42-381) days for NEC, 117 (41-325) days for FIP, and 128 (25-308) days for MRI (p = 0.855). The body weights at stoma closure were 1768 (620-3869) g for NEC, 1669 (1100-3040) g for FIP, and 1632 (940-3776) g (p = 0.614) for MRI. There were no significant differences among the three groups.. The present study revealed that the time and body weights at stoma closure and the postoperative restoration of bowel function in VLBWIs did not differ among the three diseases.

Topics: Age Factors; Body Weight; Enteral Nutrition; Enterocolitis, Necrotizing; Female; Humans; Ileus; Infant; Infant, Newborn; Infant, Very Low Birth Weight; Intestinal Perforation; Male; Meconium; Multicenter Studies as Topic; Retrospective Studies; Risk; Surgical Stomas; Time Factors; Treatment Outcome

Meconium ileus (MI) is a perinatal complication in cystic fibrosis (CF), which is only minimally influenced by environmental factors. We derived and examined MI prevalence (MIP) scores to assess CFTR phenotype-phenotype correlation for severe mutations.. MIP scores were established using a Canadian CF population (n = 2,492) as estimates of the proportion of patients with MI among all patients carrying the same CFTR mutation, focusing on patients with p.F508del as the second allele. Comparisons were made to the registries from the US CF Foundation (n = 43,432), Italy (Veneto/Trentino/Alto Adige regions) (n = 1,788), and Germany (n = 3,596).. The prevalence of MI varied among the different registries (13-21%). MI was predominantly prevalent in patients with pancreatic insufficiency carrying "severe" CFTR mutations. In this severe spectrum MIP scores further distinguished between mutation types, for example, G542X (0.31) with a high, F508del (0.22) with a moderate, and G551D (0.08) with a low MIP score. Higher MIP scores were associated with more severe clinical phenotypes, such as a lower forced expiratory volume in 1 second (P = 0.01) and body mass index z score (P = 0.04).. MIP scores can be used to rank CFTR mutations according to their clinical severity and provide a means to expand delineation of CF phenotypes.Genet Med 18 4, 333-340.

Topics: Adolescent; Adult; Alleles; Canada; Child; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genetic Association Studies; Genotype; Humans; Ileus; Male; Meconium; Mutation; Phenotype; Prevalence; Registries; Respiratory Function Tests; Severity of Illness Index; Young Adult

Surgical intestinal disorders (SID), such as necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI), are serious morbidities in extremely low birth weight (ELBW, birth weight <1000 g) infants. From 2010, we performed enteral antifungal prophylaxis (EAP) in ELBWI to prevent for SID. The aim of this study was to identify disease-specific risk factors and to evaluate the efficacy of prevention for SID in ELBW infants.. A retrospective chart review of all consecutive patients between January 2006 and March 2015, which included 323 ELBW infants who were admitted to Shizuoka Children's Hospital, was conducted.. The number of infants with NEC, FIP, and MRI was 9, 12, and 13, respectively; 28 in 323 ELBW infants died. The control group defined the cases were not SID. In-hospital mortality was higher in infants with NEC relative to those in the control group. On logistic regression analysis, low gestational age and cardiac malformations were associated with increased risk of NEC. IUGR were associated with increased risk of MRI. EAP decreased risk of NEC and FIP. Low gestational weight and NEC were associated with increased risk of death.. Survival to hospital discharge after operation for NEC in ELBW infants remains poor. EAP decreased risk of NEC and FIP in ELBW infants.

Topics: Case-Control Studies; Enterocolitis, Necrotizing; Female; Gestational Age; Heart Defects, Congenital; Humans; Ileus; Infant; Infant, Extremely Low Birth Weight; Infant, Newborn; Infant, Premature, Diseases; Intestinal Perforation; Male; Meconium; Retrospective Studies; Risk Factors

Meconium ileus is most often associated with mutations in the CFTR gene; however recently, mutations in GUCY2C in the Bedouin population have also been shown to result in this phenotype. This gene codes for an intestinal transmembrane receptor that generates cyclic GMP, which activates cystic fibrosis transmembrane receptor. We report a third family that supports the association of variants in the GUCY2C gene with meconium ileus (MI). A Lebanese kindred was studied and individuals affected with MI had either homozygous or compound heterozygous variants in GUCY2C. The earliest manifestation of the affected individuals was the presence of second trimester fetal echogenic bowel, thus resulting in the expansion of the differential diagnosis of this ultrasound finding.

Topics: Amino Acid Sequence; Base Sequence; Echogenic Bowel; Family Health; Female; Fetal Diseases; Genetic Predisposition to Disease; Genotype; Humans; Ileus; Infant, Newborn; Lebanon; Male; Meconium; Molecular Sequence Data; Mutation; Pedigree; Receptors, Enterotoxin; Receptors, Guanylate Cyclase-Coupled; Receptors, Peptide; Sequence Homology, Amino Acid

In this work, we are interested to study the implication of -509C/T polymorphism, located in the promoter region of TGFB1 (transforming growth factor β1), in the phenotypic variability of CF patients.. The present study enrolled 111 CF patients and 100 healthy control subjects. The study of the -509C/T polymorphism was performed using PCR-RFLP method.. We found that patients carried non-F508del homozygous mutation with TT genotype was associated to lung symptoms (P=0.04). This association was not found in the sub-groups of patients with F508del at homozygous state P=0.145. No association was found between this polymorphism and the variability of digestive, pancreatic and ileus meconial symptoms.. On the basis of our results, the -509C/T polymorphism of the TGFB1 gene seems to be a modulator factor of cystic fibrosis.

Topics: Adolescent; Adult; Case-Control Studies; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diabetes Mellitus; Digestive System Diseases; Female; Humans; Ileus; Infant; Infant, Newborn; Male; Meconium; Pancreatitis; Phenotype; Polymerase Chain Reaction; Polymorphism, Restriction Fragment Length; Polymorphism, Single Nucleotide; Promoter Regions, Genetic; Respiratory Insufficiency; Transforming Growth Factor beta1; Young Adult

The existence of pleiotropy in disorders with multi-organ involvement can suggest therapeutic targets that could ameliorate overall disease severity. Here we assessed pleiotropy of modifier genes in cystic fibrosis (CF). CF, caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, affects the lungs, liver, pancreas and intestines. However, modifier genes contribute to variable disease severity across affected organs, even in individuals with the same CFTR genotype. We sought to determine whether SLC26A9, SLC9A3 and SLC6A14, that contribute to meconium ileus in CF, are pleiotropic for other early-affecting CF co-morbidities. In the Canadian CF population, we assessed evidence for pleiotropic effects on (1) pediatric lung disease severity (n = 815), (2) age at first acquisition of Pseudomonas aeruginosa (P. aeruginosa) (n = 730), and (3) prenatal pancreatic damage measured by immunoreactive trypsinogen (n = 126). A multiple-phenotype analytic strategy assessed evidence for pleiotropy in the presence of phenotypic correlation. We required the same alleles to be associated with detrimental effects. SLC26A9 was pleiotropic for meconium ileus and pancreatic damage (p = 0.002 at rs7512462), SLC9A3 for meconium ileus and lung disease (p = 1.5 × 10(-6) at rs17563161), and SLC6A14 for meconium ileus and both lung disease and age at first P. aeruginosa infection (p = 0.0002 and p = 0.006 at rs3788766, respectively). The meconium ileus risk alleles in SLC26A9, SLC9A3 and SLC6A14 are pleiotropic, increasing risk for other early CF co-morbidities. Furthermore, co-morbidities affecting the same organ tended to associate with the same genes. The existence of pleiotropy within this single disorder suggests that complementary therapeutic strategies to augment solute transport will benefit multiple CF-associated tissues.

Topics: Alleles; Amino Acid Transport Systems; Amino Acid Transport Systems, Neutral; Antiporters; Canada; Child; Child, Preschool; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Genes, Modifier; Genetic Pleiotropy; Genotype; Humans; Ileus; Infant, Newborn; Male; Meconium; Models, Genetic; Morbidity; Mutation; Polymorphism, Single Nucleotide; Pseudomonas aeruginosa; Pseudomonas Infections; Sodium-Hydrogen Exchanger 3; Sodium-Hydrogen Exchangers; Sulfate Transporters

Previous studies of risk factors for progression of lung disease in cystic fibrosis (CF) have suffered from limitations that preclude a comprehensive understanding of the determinants of CF lung disease throughout childhood. The epidemiologic component of the 27-year Wisconsin Randomized Clinical Trial of CF Neonatal Screening Project (WI RCT) afforded us a unique opportunity to evaluate the significance of potential intrinsic and extrinsic risk factors for lung disease in children with CF.. Describe the most important intrinsic and extrinsic risk factors for progression of lung disease in children with CF.. Variables hypothesized at the onset of the WI RCT study to be determinants of the progression of lung disease and potential risk factors previously identified in the WI RCT study were assessed with multivariable generalized estimating equation models for repeated measures of chest radiograph scores and pulmonary function tests in the WI RCT cohort.. Combining all patients in the WI RCT, 132 subjects were observed for a mean of 16 years and contributed 1,579 chest radiographs, and 1,792 pulmonary function tests. The significant determinants of lung disease include genotype, poor growth, hospitalizations, meconium ileus, and infection with mucoid Pseudomonas aeruginosa. The previously described negative effect of female sex was not seen.. Modifiable extrinsic risk factors are the major determinants of progression of lung disease in children with CF. Better interventions to prevent or treat these risk factors may lead to improvements in lung health for children with CF.

Topics: Adolescent; Child; Child, Preschool; Cohort Studies; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Progression; Female; Genotype; Hospitalization; Humans; Ileus; Infant; Infant, Newborn; Longitudinal Studies; Lung; Male; Meconium; Neonatal Screening; Nutrition Disorders; Oligopeptides; Pseudomonas aeruginosa; Pseudomonas Infections; Radiography; Respiratory Function Tests; Risk Factors; Young Adult

The majority of bowel obstructions in extremely low birth weight (ELBW) neonates are meconium-related ileus (MRI). ELBW neonates with bowel obstruction may recover by conservative treatment, but some do not. Considering the high surgical morbidity rates, unnecessary surgery should be avoided. We sought to identify a reasonable treatment strategy under these conditions.. ELBW neonates who started to have bowel obstruction with an unclear cause within 14 days of age were enrolled. The study period was from January 2009 to August 2011. The enrolled patients had daily Gastrografin(®) enemas until 14 days of age or until the obstruction resolved. If the obstruction lasted beyond around 14 days of age, the patient underwent surgical intervention. The clinical data of the patients were collected and analyzed.. Fourteen patients were enrolled. Twelve patients had MRI, which resolved within 14 days without surgery. Two patients with persistent obstruction underwent surgery, and they were found to have Hirschsprung's disease and ileal volvulus, respectively.. For ELBW neonates with bowel obstruction of unclear etiology, the early and frequent administration of a Gastrografin(®) enema is reasonable. Surgery should be considered if the obstruction lasts beyond approximately 14 days after birth.

Topics: Age Factors; Diatrizoate Meglumine; Digestive System Surgical Procedures; Enema; Female; Hirschsprung Disease; Humans; Ileus; Infant, Extremely Low Birth Weight; Infant, Newborn; Male; Meconium; Treatment Outcome

In term newborns meconium ileus is frequently associated with cystic fibrosis. Reports on meconium ileus in preterm infants being diagnosed with cystic fibrosis early after birth are very scarce. Associations between genotype and phenotype in cystic fibrosis and its particular comorbidities have been reported.. Two extremely preterm twin infants (26 weeks of gestation) born from a Malaysian mother and a Caucasian father were presented with typical signs of meconium ileus. Despite immediate surgery both displayed a unique and finally lethal course. Mutation analysis revealed a novel, probably pathogenic cystic fibrosis mutation, p.Cys524Tyr. The novel mutation might explain the severity of disease next to typical sequelae of prematurity.. Preterm neonates with meconium ileus have to be evaluated for cystic fibrosis beyond ethnical boundaries, but may take devastating clinical courses despite early treatment. The novel, potentially pathogenic CF mutation p.Cys524Tyr might be associated with severe meconium ileus in neonates. Disease-modifying loci are important targets for intestinal comorbidity of cystic fibrosis.

Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diseases in Twins; Fatal Outcome; Humans; Ileus; Infant, Newborn; Infant, Premature; Infant, Premature, Diseases; Male; Meconium; Mutation

To identify the incidence and outcomes of cholestasis and meconium ileus (MI) in infants with cystic fibrosis (CF).. Retrospective cohort study.. Single-centre study.. From January 1986 to December 2011, 401 infants with CF (69 with MI) presented to our centre.. (1) incidence of cholestasis, (2) identification of risk factors for cholestasis, (3) association between the presence of cholestasis and MI and the development of clinically significant CF-associated liver disease (CFLD) defined as multilobular cirrhosis with portal hypertension.. Cholestasis occurred in 23 of 401 infants (5.7%). There was a significantly higher incidence of cholestasis in infants with MI (27.1%) compared to those without MI (1.2%) (p<0.001). Infants with MI had a 30.36-fold increased risk of developing cholestasis compared to those without MI (p<0.001). Cholestasis resolved in all children, at a median (range) age of 9.2 (0.8-53.2) months in the MI group and 10.2 (2.0-19.4) months in the non-MI group. The majority of cholestatic infants (87.0%) and infants with MI (92.8%) did not develop clinically significant CFLD, not significantly different than either the 93.9% of non-cholestatic infants nor the 93.7% infants without MI.. Cholestasis is an uncommon condition in CF affecting only 5.7% of the screened newborn CF population. The greatest risk factor for developing cholestasis is the presence of MI. However, the presence of MI appears not to be associated with the development of CFLD. An effect of neonatal cholestasis on the development of CFLD cannot be excluded by this study.

Topics: Cholestasis; Cohort Studies; Cystic Fibrosis; Female; Humans; Ileus; Incidence; Infant; Infant, Newborn; Male; Meconium; Prognosis; Retrospective Studies; Risk Factors

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

The aim of the present article was to determine the prevalence of liver involvement in Hispanic patients with cystic fibrosis (CF) and identify associations with age and severity of liver involvement.. We used 1994-2005 Epidemiologic Study of CF data to compare abnormal liver findings between Hispanic and non-Hispanic white patients with CF.. Of 30,727 patients with CF, 5015 had liver involvement. Of 1957 Hispanic patients, 20.8% had liver involvement compared with 16.0% of 28,770 non-Hispanic white patients (odds ratio [OR] 1.38, 95% confidence interval [CI] 1.23-1.54). This higher prevalence of liver involvement persisted after adjusting for demographics and meconium ileus and was especially high in the first year of life (adjusted OR 3.14, 95% CI 2.27-4.35). Ten percent of infants with only elevated liver enzymes progressed to more severe liver disease.. The Hispanic population with CF has more liver involvement (both elevated liver enzymes and clinical liver disease) than the non-Hispanic white population with CF, especially during the first year of life.

Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Cystic Fibrosis; Female; Hispanic or Latino; Humans; Ileus; Infant; Infant, Newborn; Liver; Liver Diseases; Male; Meconium; Middle Aged; North America; Odds Ratio; Prevalence; White People; Young Adult

Mucins are excellent candidates for contributing to the presence of meconium ileus (MI) in cystic fibrosis (CF) due to their extensive genetic variation and known function in intestinal physiology. The length of variants in mucin central repetitive regions has not been explored as "risk" factors for MI in CF.. We investigated the length polymorphisms in the central repetitive regions of MUC1, MUC2, and MUC5AC by Southern blot and tested for association with MI in CF subjects.. No significant associations were found for the allele sizes of any of the genes with respect to the prevalence of MI (p values=0.33, 0.16, and 0.71 for MUC1, MUC2, and MUC5AC, respectively).. The genetic length variants in the central repetitive region of three MUC genes studied are not associated with MI in subjects with CF.

Topics: Adolescent; Adult; Case-Control Studies; Child; Cystic Fibrosis; Female; Genetic Variation; Humans; Ileus; Male; Meconium; Mucin 5AC; Mucin-1; Mucin-2; Repetitive Sequences, Nucleic Acid; Young Adult

The coexistence of duodenal atresia (DA) may mask the antenatal ultrasound findings of meconium ileus (MI) and delay its postnatal diagnosis. We report a rare case of MI in a newborn infant diagnosed antenatally to have trisomy 21 and DA. The diagnosis of MI was only established intraoperatively after the patient showed persistent signs of intestinal obstruction following the surgical repair of the DA.

Topics: Cystic Fibrosis; Diagnosis, Differential; Down Syndrome; Duodenal Obstruction; Duodenum; Fatal Outcome; Humans; Ileus; Infant, Newborn; Intestinal Atresia; Male; Meconium; Radiography

Cystic fibrosis (CF) is an autosomal recessive disease caused by a mutation in the CFTR gene, resulting in an alteration of a protein involved in sodium and chloride transport in the apical plasma membrane of epithelial cells in respiratory and intestinal tracts. It primarily presents respiratory compromise, affecting other systems in different ways. Meconium ileus is a gastrointestinal manifestation that occurs in 10-20% of patients, which is not entirely attributable to a specific CFTR mutation.. To report a case of monozygotic twins diagnosed with CF (F508) in whom phenotypic variation is evident based on the expression of meconium ileus, showing that there are external modifiers in the development of this complication.. monoamniotic monochorionic twin pregnancy which resulted in preterm births. One of the patient presented meconium ileus at birth leading to CF suspicion and establishing the diagnosis by (F508/F508) molecular analysis in both twins.. Phenotypic variability in these twins supports the hypothesis proposed by different authors that there are other gene expression-modulation factors of the disease as well as environmental modifiers that must be taken into account when dealing with this disease.

Topics: Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diseases in Twins; Humans; Ileus; Infant, Newborn; Male; Meconium; Mutation; Phenotype; Twins, Monozygotic

Cystic fibrosis (CF) pigs develop disease with features remarkably similar to those in people with CF, including exocrine pancreatic destruction, focal biliary cirrhosis, micro-gallbladder, vas deferens loss, airway disease, and meconium ileus. Whereas meconium ileus occurs in 15% of babies with CF, the penetrance is 100% in newborn CF pigs. We hypothesized that transgenic expression of porcine CF transmembrane conductance regulator (pCFTR) cDNA under control of the intestinal fatty acid-binding protein (iFABP) promoter would alleviate the meconium ileus. We produced 5 CFTR-/-;TgFABP>pCFTR lines. In 3 lines, intestinal expression of CFTR at least partially restored CFTR-mediated anion transport and improved the intestinal phenotype. In contrast, these pigs still had pancreatic destruction, liver disease, and reduced weight gain, and within weeks of birth, they developed sinus and lung disease, the severity of which varied over time. These data indicate that expressing CFTR in intestine without pancreatic or hepatic correction is sufficient to rescue meconium ileus. Comparing CFTR expression in different lines revealed that approximately 20% of wild-type CFTR mRNA largely prevented meconium ileus. This model may be of value for understanding CF pathophysiology and testing new preventions and therapies.

Topics: Animals; Animals, Genetically Modified; Animals, Newborn; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Fatty Acid-Binding Proteins; Female; Gene Expression; Humans; Ileum; Ileus; Infant, Newborn; Lung; Male; Meconium; Pancreas; Phenotype; Promoter Regions, Genetic; Radiography; Rats; Sus scrofa; Trachea

Bronchiectasis develops early in the course of cystic fibrosis, being detectable in infants as young as 10 weeks of age, and is persistent and progressive. We sought to determine risk factors for the onset of bronchiectasis, using data collected by the Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) intensive surveillance program.. We examined data from 127 consecutive infants who received a diagnosis of cystic fibrosis after newborn screening. Chest computed tomography (CT) and bronchoalveolar lavage (BAL) were performed, while the children were in stable clinical condition, at 3 months and 1, 2, and 3 years of age. Longitudinal data were used to determine risk factors associated with the detection of bronchiectasis from 3 months to 3 years of age.. The point prevalence of bronchiectasis at each visit increased from 29.3% at 3 months of age to 61.5% at 3 years of age. In multivariate analyses, risk factors for bronchiectasis were presentation with meconium ileus (odds ratio, 3.17; 95% confidence interval [CI], 1.51 to 6.66; P=0.002), respiratory symptoms at the time of CT and BAL (odds ratio, 2.27; 95% CI, 1.24 to 4.14; P=0.008), free neutrophil elastase activity in BAL fluid (odds ratio, 3.02; 95% CI, 1.70 to 5.35; P<0.001), and gas trapping on expiratory CT (odds ratio, 2.05; 95% CI, 1.17 to 3.59; P=0.01). Free neutrophil elastase activity in BAL fluid at 3 months of age was associated with persistent bronchiectasis (present on two or more sequential scans), with the odds seven times as high at 12 months of age and four times as high at 3 years of age.. Neutrophil elastase activity in BAL fluid in early life was associated with early bronchiectasis in children with cystic fibrosis. (Funded by the National Health and Medical Research Council of Australia and Cystic Fibrosis Foundation Therapeutics.)

Topics: Bronchiectasis; Bronchoalveolar Lavage Fluid; Child, Preschool; Cystic Fibrosis; Female; Humans; Ileus; Infant; Infant, Newborn; Leukocyte Elastase; Longitudinal Studies; Lung; Male; Meconium; Multivariate Analysis; Prevalence; Risk Factors

Enterostomy formation is a common outcome in emergency neonatal laparotomy. No consensus exists regarding optimal stoma site. This study aims to identify incidence of complications and closure details related to position of stomas.. This study is a retrospective case note review of emergency neonatal enterostomy formation over 11 years at a single institution. Patients were separated into two groups: stomas created through the laparotomy wound and stomas created through a separate incision. Demographic details, complications and closure details were ascertained. Differences between groups were analysed (Mann-Whitney test for continuous variables, Chi-squared test or Fisher's exact test for categorical variables).. One hundred and thirteen stoma formations were examined in 106 patients (71 within laparotomy wound, 42 through a separate incision). Age, gestation, weight, wound-related and stoma-related complications were not significantly different between the groups. A trend towards a higher rate of full laparotomy at closure with stomas through the wound (p = 0.09) was seen. If stomas were sited adjacently, there was no difference in avoidance of full laparotomy at closure (p = 0.97).. Stomas sited adjacently within the laparotomy wound are not related to increased complications and offer the same advantage of circumexcision at closure as stomas sited through a separate wound, without an additional abdominal wound.

Topics: Biliary Atresia; Emergencies; Enterocolitis, Necrotizing; Enterostomy; Hirschsprung Disease; Humans; Ileus; Infant, Newborn; Laparotomy; Meconium; Postoperative Complications; Retrospective Studies

Postpartum abdominal distention and meconium ileus may occur due to intestinal obstruction, Hirschprung disease or cystic fibrosis. However, other rare and challenging etiologies such as congenital chloride diarrhea (CCD) should be included in differential diagnosis of such presentation. We present a premature baby girl who had distended abdomen and lack of meconium immediately after birth. Surgical etiology was excluded and she was mistakenly suspected of having cystic fibrosis due to meconium ileus. CCD was diagnosed by recognition of watery diarrhea in association with hyponatremic, hypochloremic metabolic acidosis. Mutation analysis confirmed the diagnosis.

Topics: Cystic Fibrosis; Diagnosis, Differential; Diarrhea; DNA Mutational Analysis; Feces; Female; Humans; Ileus; Infant, Newborn; Infant, Premature; Meconium; Metabolism, Inborn Errors; Risk Assessment

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

The stability of extemporaneously prepared acetylcysteine 1% and 10% solutions for treatment of meconium ileus was evaluated.. Acetylcysteine 1% (10-mg/mL) and 10% (100-mg/mL) solutions were prepared by mixing 3 and 10 mL, respectively, of commercially available 20% acetylcysteine solution with a sufficient quantity of bacteriostatic 0.9% sodium chloride for injection to make a final volume of 60 mL. Three identical samples of each concentration were prepared, placed in 2-oz amber plastic prescription bottles, and stored at 20-25 °C. Samples were assayed in duplicate using high-performance liquid chromatography and inspected for changes in color, odor, and pH immediately after preparation and at 7, 14, 30, 60, and 90 days. Stability was defined as retention of at least 90% of the initial concentration.. At least 90% of the initial concentration of acetylcysteine was retained in both formulations for 60 days. No appreciable change from the initial pH occurred in the acetylcysteine 1% or 10% solution during the first 60 days, but there was a notable change in pH after 90 days in both formulations. Neither solution was stable at day 90. There was no detectable change in color at 90 days; however, the odor of hydrogen sulfide was more pungent than on previous study days.. Extemporaneously prepared solutions of acetylcysteine 1% (10 mg/mL) and 10% (100 mg/mL) prepared with bacteriostatic 0.9% sodium chloride for injection were stable for at least 60 days when stored in plastic amber bottles at room temperature.

Topics: Acetylcysteine; Administration, Oral; Chromatography, High Pressure Liquid; Drug Stability; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Meconium; Solutions

A nationwide survey on neonatal surgery conducted by the Japanese Society of Pediatric Surgeons has demonstrated that the mortality of neonatal intestinal perforation has risen over the past 15 years. The incidence of intestinal perforation in extremely low-birthweight (ELBW) neonates has been increasing as more ELBW neonates survive and as the live-birth rate of ELBW has increased. In contrast to necrotizing enterocolitis (NEC) and focal intestinal perforation (FIP), the pathogenesis of meconium-related ileus, defined as functional bowel obstruction characterized by delayed meconium excretion and microcolon, remains unclarified.. The histology of 13 ELBW neonates with intestinal perforation secondary to meconium-related ileus was reviewed, and the radiology of 33 cases of meconium-related ileus diagnosed on contrast enema was reviewed. Specimens obtained from 16 ELBW neonates without gastrointestinal disease served as age-matched controls for histological assessment.. The size of the ganglion cell nucleus in meconium-related ileus and in control subjects was 47.3 ± 22.0 µm(2) and 37.8 ± 11.6 µm(2), respectively, which was not significantly different. In all cases of meconium-related ileus, contrast enema demonstrated a microcolon or small-sized colon, with a gradual caliber change in the ileum and filling defects due to meconium in the ileum or colon, showing not-identical locations of caliber changes and filling defects.. Morphological immaturity of ganglia was not suggested to be the pathogenesis of meconium-related ileus. Impaction of inspissated meconium is not the cause of obstruction, but the result of excessive water absorption in the hypoperistaltic bowel before birth, although the underlying mechanism responsible for the fetal hypoperistalsis remains unclear.

Topics: Colon; Colonic Diseases; Contrast Media; Diagnosis, Differential; Enema; Ganglia, Sympathetic; Gestational Age; Humans; Ileus; Infant, Newborn; Infant, Newborn, Diseases; Infant, Very Low Birth Weight; Laparotomy; Meconium; Prognosis; Radiography, Abdominal

Various surgical methods are used to treat meconium ileus (MI), including resection with enterostomy (RES), primary anastomosis (RPA), and purse-string enterotomy with intra-operative lavage (PSI). The aim of this study is to discuss the surgical treatment of MI, based on our experience.. Of the 41 MI patients treated at our institution between 1984 and 2007, 18 had simple MI and 23 had complex MI. These groups were analyzed according to treatment modality, concentrating on length of hospital stay, complications [peritonitis, septicemia, adhesive small bowel obstruction (ASBO), and malabsorption/diarrhea], need for additional surgical procedures, mortality.. Of the 18 patients with simple MI, 7 (39%) were successfully treated with diluted Gastrografin® enema. The remaining 11 patients were treated surgically: two underwent RPA, of whom one died; five had RES, of whom one developed ASBO; four underwent PSI, of whom two developed peritonitis. In the complex MI group, 14 patients underwent RPA, with peritonitis occurring in three (one died); nine underwent RES, of whom two developed ASBO.. In patients with simple MI, conservative treatment with diluted Gastrografin® enema is an effective initial treatment in our hands. In case of failure, RES is advisable. Patients with complex MI are candidates for RES. RPA and PSI seem to have higher complication rates.

Topics: Female; Humans; Ileal Diseases; Ileus; Infant, Newborn; Male; Meconium; Retrospective Studies; Severity of Illness Index

Meconium abnormalities are characterized by a wide spectrum of severity, from the meconium plug syndrome to the complicated meconium ileus associated with cystic fibrosis. Meconium Related Ileus in absence of Cystic Fibrosis includes a combination of highly viscid meconium and poor intestinal motility, low grade obstruction, benign systemic and abdominal examination, distended loops without air fluid levels. Associated risk factors are severe prematurity and low birth weight, Caesarean delivery, Maternal MgSO4 therapy, maternal diabetes. In the last 20 yrs a new specific type of these meconium related obstructions has been described in premature neonates with low birth weight. Its incidence has shown to increase while its management continues to be challenging and controversial for the risk of complicated obstruction and perforation.. Among 55 newborns admitted between 1992-2008 with Meconium Related Ileus as final diagnosis, data about Low Birth Weight infants (LBW < 1500 g) were extracted and compared to those of patients ≥ 1500 g. Hischsprung's Diseases and Cystic Fibrosis were excluded by rectal biopsy and genetic probe before discharge. A softening enema with Gastrografin was the first option whenever overt perforation was not present. Temporary stoma or trans appendiceal bowel irrigation were elected after unsuccessful enema while prompt surgical exploration was performed in perforated cases. NEC was excluded in all operated cases. Data collected were perinatal history and neonatal clinical data, radiological signs, clinical course and complications, management and outcome.. 30 cases with BW ≥ 1500 g had an M/F ratio 16/14, Mean B.W. 3052 g, Mean G.A. 37 w Caesarean section rate 40%. There were 10 meconium plug syndrome, 4 small left colon syndromes, and 16 meconium ileus without Cystic Fibrosis. Five cases were born at our institution (inborn) versus 25 referred after a mean of 2, 4 Days (1-7) after birth in another Hospital (outborn). They were managed, after a Gastrografin enema with 90% success rate, by 1 temporary Ileostomy and 2 trans appendiceal irrigation. 25 cases with BW< 1500 g (LBW) had M/F ratio 11/14, Mean B.W. 818 g, Mean G.A. 27 w, Caesarean section rate 70%, assisted ventilation 16/25. There were 8 inborn and 17 outborn. Gastrografin enema was successful in 6 out 8 inborn infants only, all referred within one week from birth. There were 12 perforations mainly among late referred LBW outborn.. Meconium Related Ileus without Cystic Fibrosis responds to conservative management and softening enema in most of mature infants. In LBW clinical course is initially benign but as any long standing bowel obstruction management may present particular challenges. Clinical and plain radiographic criteria are reliable for making diagnosis and testing for Cystic Fibrosis may not be indicated. Enema may be resolutive when performed in a proper environment. Perforated cases may be confused with NEC which is excluded by clinical history, no signs of sepsis, lab signs missing, abdominal signs missing, typical radiological signs missing. The higher complication rate is recorded among cases delivered and initially managed in Neonatal Units without co-located Surgical Facilities. Early diagnosis and aggressive medical therapy may lead to higher success rate and help avoiding surgical interventions. Surgical therapy in uncomplicated cases, unresponsive to medical management, should be minimally aggressive.

Topics: Cystic Fibrosis; Female; Follow-Up Studies; Humans; Ileus; Incidence; Infant, Low Birth Weight; Infant, Newborn; Infant, Newborn, Diseases; Infant, Premature; Infant, Premature, Diseases; Italy; Male; Meconium; Prognosis; Retrospective Studies; Survival Rate

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

Patients with cystic fibrosis (CF) presenting with meconium ileus (MI) tend to have worse outcomes than those without MI. We evaluated the clinical characteristics and survival rates among Israeli patients with CF with and without MI after a prolonged follow-up (15-30 years).. A multicenter retrospective study. Forty-nine patients with CF, representing 13.8% of all patients with CF in Israel, presented with MI (current age 17.4 +/- 7.9 years) between 1975 and 2006. They were compared with 38 patients with CF (current age 19.3 +/- 6.5 years) without MI matched by sex and CF transmembrane conductance regulator mutation.. A total of 66.2% of patients with MI and 73.6% without MI were followed for a prolonged period (24.9 +/- 2.7 years). Of the patients with MI, 31 were managed operatively, whereas 18 were treated successfully with gastrograffin enema, with similar clinical outcomes. Five patients in the MI group and 3 in the control group died during the study period. Bacterial colonization, z score of body mass index, and pulmonary function tests were similar in patients with and without MI in the long term. In younger patients, many clinical parameters were more prevalent in patients with MI (P = 0.004). However, these differences disappeared after the long-term follow-up (up to 31-years).. Patients with CF presenting with MI had similar pulmonary function and nutritional status, as well as survival rates as did the control patients without MI. The distinct genetic mutation found in our population may explain in part the favorable results compared with other studies. In addition, it seems that early diagnosis and treatment of MI in patients with CF may be beneficial, subsequently lowering morbidity, and increasing survival.

Topics: Adolescent; Adult; Age Factors; Body Mass Index; Child; Cystic Fibrosis; Diatrizoate Meglumine; Disease Progression; Enema; Female; Humans; Ileus; Infant; Israel; Lung; Male; Meconium; Respiratory Function Tests; Retrospective Studies; Risk Factors; Survival Analysis; Treatment Outcome; Young Adult

The aims of this study were to determine prevalence, risk factors and treatment of constipation in patients with Cystic Fibrosis (CF), as well as the diagnostic value of abdominal radiography.. A cohort of 214 pediatric CF patients was investigated. Furthermore, 106 abdominal radiographs of CF patients with or without constipation were independently assessed by three observers on two separate occasions using the Barr and Leech scores.. The prevalence of constipation was 47%. Low total fat absorption and meconium ileus were independent risk factors for constipation in CF, while fiber and fluid intake were not associated. In CF patients the inter and intraobserver variabilities of the Barr and Leech scores were poor to moderate.. Constipation is a significant medical issue in CF and was associated with low total fat absorption and a history of meconium ileus. Finally, abdominal radiography seems of little value in the regular follow-up of CF patients.

Topics: Adolescent; Child; Child, Preschool; Constipation; Cystic Fibrosis; Dietary Fats; Female; Humans; Ileus; Infant, Newborn; Intestinal Absorption; Malabsorption Syndromes; Male; Meconium; Observer Variation; Prevalence; Radiography, Abdominal; Risk Factors; Steatorrhea

In Cftr-/- mice that mostly die because of intestinal obstruction, intestinal expression of Clca3 is decreased, whereas upregulation of Clca3 results in amelioration of intestinal disease. The aim of the study was to investigate whether the p.S357N variant in CLCA1, the human orthologue of Clca3, acts as a modifier gene in a cohort of 682 European patients with cystic fibrosis (CF)-99 patients with meconium ileus. The 357SS genotype was significantly overrepresented in both patients with meconium ileus and also with a severe CFTR genotype (P = 0.009) and in p.F508del homozygotes (P = 0.002). This suggests that CLCA1 has similar important functions in CF-related intestinal obstruction in humans as in Cftr-/- mice.

Topics: Adolescent; Adult; Child; Chloride Channels; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Europe; Female; Genetic Variation; Genotype; Humans; Ileus; Infant, Newborn; Male; Meconium; Young Adult

The present study compares the clinical presentation and diagnostic features of meconium ileus (MI) in newborns with and without cystic fibrosis (CF). A retrospective study of 43 patients treated in the Pediatric Surgical Center of Amsterdam was performed. Twenty-three of the patients (53.5%) were diagnosed as having CF. Complex MI was significantly more frequent in patients without CF, and these patients had lower gestational ages and birth weights than patients with CF. All of the patients with complex MI had homozygous DF508 mutations, whereas the patients with simple MI also had other mutations. None of the patients with other mutations had complex MI. Therefore, we conclude that the clinical entity of MI represents a spectrum of underlying pathologies.

Topics: Birth Weight; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Genotype; Gestational Age; Humans; Ileus; Incidence; Infant, Newborn; Meconium; Mutation; Retrospective Studies

Lung disease causes most of the morbidity and mortality in cystic fibrosis (CF). Understanding the pathogenesis of this disease has been hindered, however, by the lack of an animal model with characteristic features of CF. To overcome this problem, we recently generated pigs with mutated CFTR genes. We now report that, within months of birth, CF pigs spontaneously developed hallmark features of CF lung disease, including airway inflammation, remodeling, mucus accumulation, and infection. Their lungs contained multiple bacterial species, suggesting that the lungs of CF pigs have a host defense defect against a wide spectrum of bacteria. In humans, the temporal and causal relations between inflammation and infection have remained uncertain. To investigate these processes, we studied newborn pigs. Their lungs showed no inflammation but were less often sterile than controls. Moreover, after introduction of bacteria into their lungs, pigs with CF failed to eradicate bacteria as effectively as wild-type pigs. These results suggest that impaired bacterial elimination is the pathogenic event that initiates a cascade of inflammation and pathology in CF lungs. Our finding that pigs with CF have a host defense defect against bacteria within hours of birth provides an opportunity to further investigate CF pathogenesis and to test therapeutic and preventive strategies that could be deployed before secondary consequences develop.

Topics: Animals; Animals, Newborn; Cystic Fibrosis; Disease Models, Animal; Ileus; Inflammation; Lung; Meconium; Mucus; Pancreatic Diseases; Radiography, Thoracic; Survival Analysis; Swine; Time Factors

It is a matter of debate as to what extent the long-term outcome of cystic fibrosis (CF) is affected by presenting with meconium ileus (MI). We compared long-term clinical outcomes of CF children who presented with MI, to those presenting with other symptoms (non-MI) in an era of non new-born-screening (NBS).We collected annual lung function data between the ages of 8-15 years in terms of percent predicted first second forced expired volume (FEV1%pr), percent predicted forced vital capacity (FVC%pr), and between the ages of 2-15 years annual height and weight Z-scores (HtZ and WtZ respectively) for children attending the Royal Brompton Hospital CF clinic. To be included in the study, subjects had to have at least five pulmonary function tests and five anthropometric measurements recorded over this period.Thirty-eight MI and 76 non-MI subjects were compared. There were no significant differences in genotype, sex, chronic Pseudomonas infection, or pancreatic enzyme use between the two groups. The median age of diagnosis was 1 day (MI) versus 7 months (non-MI). There was a decline in spirometry and anthropometric variables over the study period for both MI and non-MI groups apart from WtZ score in the non-MI group. Mixed model analysis adjusting for potential confounders including genotype, pancreatic status, sex, chronic Pseudomonas aeruginosa lung infection, and age of diagnosis revealed no difference between the two groups in terms of lung function and growth during the time period of the study, however there was a non-significant trend for subjects presenting with MI to do better in all four parameters.We conclude that babies presenting with MI have no worse long-term outcome than those presenting symptomatically later in infancy, despite having undergone invasive procedures in the newborn period. This underscores the importance of early diagnosis and treatment in CF.

Topics: Adolescent; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Ileus; Male; Meconium; Prognosis; Respiratory Function Tests

Cystic fibrosis (CF) is a monogenic disease due to mutations in the CFTR gene. Yet, variability in CF disease presentation is presumed to be affected by modifier genes, such as those recently demonstrated for the pulmonary aspect. Here, we conduct a modifier gene study for meconium ileus (MI), an intestinal obstruction that occurs in 16-20% of CF newborns, providing linkage and association results from large family and case-control samples. Linkage analysis of modifier traits is different than linkage analysis of primary traits on which a sample was ascertained. Here, we articulate a source of confounding unique to modifier gene studies and provide an example of how one might overcome the confounding in the context of linkage studies. Our linkage analysis provided evidence of a MI locus on chromosome 12p13.3, which was segregating in up to 80% of MI families with at least one affected offspring (HLOD = 2.9). Fine mapping of the 12p13.3 region in a large case-control sample of pancreatic insufficient Canadian CF patients with and without MI pointed to the involvement of ADIPOR2 in MI (p = 0.002). This marker was substantially out of Hardy-Weinberg equilibrium in the cases only, and provided evidence of a cohort effect. The association with rs9300298 in the ADIPOR2 gene at the 12p13.3 locus was replicated in an independent sample of CF families. A protective locus, using the phenotype of no-MI, mapped to 4q13.3 (HLOD = 3.19), with substantial heterogeneity. A candidate gene in the region, SLC4A4, provided preliminary evidence of association (p = 0.002), warranting further follow-up studies. Our linkage approach was used to direct our fine-mapping studies, which uncovered two potential modifier genes worthy of follow-up.

Topics: Adolescent; Chromosome Mapping; Chromosomes, Human, Pair 12; Cystic Fibrosis; Female; Genetic Linkage; Humans; Ileus; Infant, Newborn; Male; Meconium; Receptors, Adiponectin; Sodium-Bicarbonate Symporters

Cystic fibrosis (CF) is a multisystem disorder intrinsically associated with inflammation of mucosal surfaces. Because inflammation can result in enteric neuromuscular dysfunction we hypothesized that terminal ileitis in patients with CF may predispose to distal ileal obstruction syndrome (DIOS).. Full-thickness terminal ileal tissues from 6 children with CF and severe DIOS, 6 infants with complicated meconium ileus (MI), and 6 children with non-CF intestinal atresia were studied.. Lymphocyte-predominant mucosal and transmural ileal inflammation was present in 6 of 6 patients with DIOS. Lymphocytic ganglionitis was present in 4 of 6 although numbers of myenteric neurons were not decreased (5/5). Myocyte proteins were preserved (6/6). Mild submucosal fibrosis was common in DIOS (5/6) and transformation of submucosal fibroblasts to a myofibroblastic phenotype was noted in 4 of 6. Inflammatory changes were distinct from those described in fibrosing colonopathy. Antroduodenal manometry in an individual who had experienced MI/DIOS was consistent with a neuropathic pseudo-obstructive process. Submucosal or transmural lymphocyte predominant inflammation was also present in 6 of 6 infants with complicated MI, which, when coupled with submucosal myofibroblast proliferation (5/6), appeared highly predictive of CF rather than non-CF atresia. Histological findings at birth were similar, although milder, than those seen in DIOS, suggesting that these changes are a primary abnormality in CF.. Submucosal or transmural inflammation of the ileum is common in newborns with CF and MI and older children with DIOS. Severe recurrent DIOS should be investigated with seromuscular and mucosal biopsy of the ileum to seek a transmural ileitis potentially amenable to anti-inflammatory therapies.

Topics: Adolescent; Child; Child, Preschool; Crohn Disease; Cystic Fibrosis; Duodenum; Female; Fibroblasts; Ganglion Cysts; Humans; Ileum; Ileus; Infant; Inflammation; Intestinal Atresia; Intestinal Mucosa; Intestinal Pseudo-Obstruction; Lymphocytes; Male; Manometry; Meconium; Muscle Cells; Myenteric Plexus; Retrospective Studies

Cystic Fibrosis (CF) is a common autosomal recessive disease that affects multiple organs. The lack of an animal model with manifestations like those typically found in humans has slowed understanding of its pathogenesis. Therefore, because of the similarities between human and swine anatomy, biochemistry, physiology, size, and genetics, we chose to develop a porcine model of CF. We used homologous recombination in primary cultures of porcine fibroblasts to disrupt the CFTR gene and then used those cells as nuclear donors for somatic cell nuclear transfer. After crossing heterozygous pigs, we produced CFTR-/- pigs. The newborn CFTR null piglets manifested meconium ileus, pancreatic destruction, early focal biliary cirrhosis, and gall bladder abnormalities that were very similar to those observed in humans with CF. At birth, there were no abnormalities in the airway epithelium or submucosal glands and no evidence of inflammation, consistent with findings in the newborn human. We hope that this porcine model will help elucidate the pathogenesis of CF and thereby lead to the development of new mechanism-based therapies.

Topics: Animals; Animals, Genetically Modified; Animals, Newborn; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Disease Models, Animal; Gallbladder Diseases; Gene Targeting; Humans; Ileus; Infant, Newborn; Liver Diseases; Meconium; Models, Biological; Mutation; Pancreatic Diseases; Phenotype; Respiratory System; Species Specificity; Swine

To identify cystic fibrosis (CF) patients with a history of meconium ileus (MI) and to compare long-term outcome of these patients with CF patients without MI.. 370 CF patients who were treated at the Munich CF centre in 2006, mean age 17.1 years (range: 0-48), were classified with regard to a history of MI.. Retrospective chart analysis regarding history of MI, age, and lung function and first detection of Pseudomonas aeruginosa as the primary outcome variables.. We identified 60 patients with MI (16.2% of all patients with a mean age of 16.2 +/- 9 years (0-41) who did not differ in age from patients without MI (17.3 +/- 10; 0-48), whereas the mean age of CF diagnosis was different (0.4 vs. 21.8 months, P < 0.001). Cross sectional analysis of best Forced expiratory volume (FEV% predicted) in 2006 was not different between patients with MI (89 +/- 23%, 17-134) and those without (88 +/- 27%, 21-148, P = 0.73). Longitudinal analysis using FEV1 values of more than 9,000 lung function tests also showed no difference in FEV1 although first detection of P. aeruginosa in patients with MI was significantly earlier.. MI was associated with hospitalization, surgery, and early infection with P. aeruginosa. However, the long-term outcome of CF patients with or without MI was the same. This finding might be explained by the considerably earlier CF diagnosis in CF patients with MI including the benefit of an early initiation of appropriate CF therapy.

Topics: Adolescent; Adult; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Ileus; Infant; Infant, Newborn; Male; Meconium; Middle Aged; Retrospective Studies; Time Factors

Contrast enema is the initial study of choice for simple meconium ileus to confirm diagnosis and to relieve obstruction. Despite favorable historically published results, our clinical impression suggests decreased effectiveness of the contrast enema resulting in more surgical interventions in contemporary practice.. A retrospective multiinstitutional review for a 12-year period was conducted for neonates diagnosed with meconium ileus by contrast enema. The neonates were divided into 2 groups-historic group (HG = before 2002) and contemporary group (CG = after 2002). T test was used for comparison of continuous variables and chi(2) for categorical data.. Thirty-seven total patients were identified (21 females and 16 males). Obstruction was relieved in 8 neonates (22% overall success rate). Average enema attempt per patient was decreased in the CG group compared to HG (1.4 vs 1.9). The success rate in the CG group was 5.5% (1/18) compared to 39% (7/18) in HG.. In this review, success of contrast enema for relief of meconium ileus has significantly decreased over time. These findings may be because of reluctance to repeat enemas, change in radiologist experience, or use of contrast agent. As a result, higher rates of operative intervention are now observed. In stable patients, surgeons should recommend repeat enemas before exploration.

Topics: Birth Weight; Cystic Fibrosis; Diatrizoate Meglumine; Enema; Female; Gestational Age; Humans; Ileus; Infant, Newborn; Male; Meconium; Preoperative Care; Radiography; Retrospective Studies; Treatment Outcome

We are reporting the case of a neonate which died on 16th day of life due to a fatal evolution of a rare association of cystic fibrosis and Hirschsprung's disease.

Topics: Colonic Diseases; Cystic Fibrosis; Fatal Outcome; Hirschsprung Disease; Humans; Ileal Diseases; Ileus; Infant, Newborn; Male; Meconium

We discuss an infant with MI secondary to cystic fibrosis, who was managed surgically by a double barrel ileostomy for mid - small bowel atresia and developed severe faecal impaction in the post - operative period. The faecal impaction was treated successfully with oral NAC and 0.2% NAC contrast enemas. The patient's liver function tests revealed a dramatic increase in transaminases and bilirubin contemporaneous with the administration of the enemas. The levels showed a spontaneous improvement after discontinuation. This is only the second reported case of hepatotoxicity secondary to NAC enemas in the literature. While our experience offers modest support for the use of NAC, its efficacy is not yet proven and paediatric surgeons using NAC in the enema form need to closely monitor liver function contemporaneous with this agent's administration and adjust their treatment accordingly.

Topics: Acetylcysteine; Chemical and Drug Induced Liver Injury; Cystic Fibrosis; Enema; Fecal Impaction; Free Radical Scavengers; Humans; Ileus; Infant, Newborn; Meconium

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

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

Although administration of a water-soluble contrast enema has been recognized to be effective for meconium-related ileus, there have been no definitive management guidelines for very low-birthweight infants.. Between 1998 and 2004, 10 infants without cystic fibrosis were treated for meconium-related ileus at Toyohashi Municipal Hospital. Their treatment and clinical course were reviewed retrospectively.. The average gestational age and birthweight of the 10 infants was 27 weeks and 788 g, respectively. The average age at initiation of treatment with a water-soluble contrast enema was 6.8 days. Intestinal obstruction was relieved by the enema in eight of 10 patients, while one underwent laparotomy and one died without any improvement of obstruction. In both neonates for whom the enema failed, rectal examination and rectal irrigation had been performed for several days before the enema was administered at the age of 14 and 15 days, respectively. In contrast, the enema was administered at the age of 1-11 days in neonates for whom this treatment was successful. Obstruction was relieved if the contrast medium reached the distal ileum, but enemas without reflux into the distal ileum failed to improve the obstruction. Contrast medium passed through the ileocecal valve to reach the distal ileum in all procedures done under fluoroscopy, but the medium failed to reach the ileum in most of the procedures done without fluoroscopy.. Although administration of water-soluble contrast enemas can be effective for meconium-related ileus, reflux into the terminal ileum is essential for bowel obstruction to improve, so it is desirable to perform the procedure under fluoroscopic guidance.

Topics: Contrast Media; Female; Fluoroscopy; Humans; Ileus; Infant, Newborn; Infant, Very Low Birth Weight; Male; Meconium

Various options are available for the surgical treatment of meconium ileus (MI). This paper examines the use of resection and primary anastomosis as the favoured option for the treatment of complicated meconium ileus. This was a retrospective study. All patients (13 children) with MI treated with primary resection and anastomosis (RA) in the 10-year period (1996-2005) at St Mary's Hospital in Manchester were identified. The case notes were retrieved. The gestational age, type of surgery, length of bowel resection and complications were recorded. Out of 13 (3 males, 10 females), 7 had severely dilated bowels, 5 had perforation and 1 had volvulus. Mean length of bowel resection was 33.8 cm (range 20-50 cm). Overall survival in this group was 85%. Seven patients (54%) developed complications. Four (31%) had surgical complications: two anastomotic strictures with adhesions, one adhesive intestinal obstruction and one intra-abdominal drain retraction. Primary resection and anastomosis is a safe option in the treatment of complicated meconium ileus. It has the advantage of less hospital stay and avoids a secondary laparotomy for closure of the stoma.

Topics: Anastomosis, Surgical; Cause of Death; Cystic Fibrosis; Female; Follow-Up Studies; Humans; Ileus; Infant, Newborn; Intestines; Laparotomy; Length of Stay; Male; Meconium; Retrospective Studies; Survival Rate; Treatment Outcome; United Kingdom

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

Serum immunoreactive trypsin (IRT) is used as a screening test for cystic fibrosis (CF) in neonates in many countries. Variations in IRT levels are observed in healthy and cystic neonates within the first few weeks of life. Fifteen percentage of CF neonates present with meconium ileus (MI). We hypothesised that there may be differences in serum IRT levels in cystic babies with simple and complicated MI. The aim of this study was to investigate the serum levels of IRT in neonates with CF presenting with MI. IRT levels were sequentially measured in neonates (n = 29) with CF with intestinal obstruction due to simple or complicated MI. These were compared to levels obtained from non-cystic neonates/controls admitted with a variety of other intra-abdominal pathologies (n = 49) IRT levels were significantly higher in the CF-MI group than the non-cystic controls (P < 0.001). There was no statistical difference in IRT levels between the simple or complicated MI groups. In the MI group there was no statistical difference between those who required operation, no difference between the pre- and post-operative IRT levels and no significant relationship between IRT levels and birth weight or gestation. Serum IRT levels are significantly elevated in neonates with CF and MI compared with non-cystic, non-MI neonates. The results of this observational study highlight that a single raised level of IRT in a neonate should prompt the analysis for CF regardless of any underlying surgical pathology.

Topics: Biomarkers; Cystic Fibrosis; Diagnosis, Differential; Follow-Up Studies; Humans; Ileus; Infant, Newborn; Meconium; Retrospective Studies; Severity of Illness Index; Trypsin

Meconium ileus is one of the gastrointestinal manifestations of cystic fibrosis (CF), and affects 15% of neonates. The condition results from the accumulation of sticky inspissated meconium. Both nonoperative and operative therapies may be effective in relieving obstruction. The treatment of choice for uncomplicated meconium ileus is the use of enteral N-acetylcysteine or Gastrografin enemata. Once such therapy fails, surgery is indicated. A number of operative procedures are in use, including Bishop-Koop enterostomy, T-tube irrigation, resection and primary anastomosis, and enterotomy with irrigation and primary closure. During the period 1991-2003, five newborns required surgical intervention for uncomplicated meconium ileus. None responded to conservative management. All were males, including one set of twins. All underwent laparotomy, enterotomy, appendectomy, irrigation and closure of enterotomy. None required a second surgical procedure. CF was confirmed in all, and in each case, both parents were found to be genetic carriers of a mutational form of CF. A single surgical intervention is preferable in these patients, in view of the high rate of pulmonary involvement in CF patients. Enterotomy, irrigation and primary closure are the treatment of choice for uncomplicated meconium ileus.

Topics: Humans; Ileus; Infant, Newborn; Male; Meconium; Retrospective Studies; Treatment Outcome

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

Information concerning long-term operative outcomes in patients with cystic fibrosis (CF) is relatively sparse in the operative literature.. A retrospective review of CF patients with operative conditions was performed (1972-2004) at a tertiary children's hospital to analyze outcomes including long-term morbidity and survival.. A total of 226 patients with CF presented with an operative diagnosis (113 men, 113 women). A total of 422 operations were performed in 213 patients (94%). The mean age at operation was 4.1 +/- 6.2 years (range, 1 d to 26 y) and 109 were neonates. Fifteen of 42 (36%) babies with simple meconium ileus (MI) were treated nonoperatively with hypertonic enemas, 27 of 42 and all 45 patients with complicated MI required operation, including 15 with jejunoileal atresia (17%). Seventeen of 27 (63%) patients with meconium ileus equivalent had MI as neonates; 7 of 27 (26%) required operation. Eight of 9 (89%) with fibrosing colonopathy required operation. Organ transplantation was required in 21 patients. Follow-up evaluation was possible in 204 of 213 (96%) patients. The duration of follow-up evaluation was 14.9 +/- 8.5 years (range, 2 mo to 35 y). Operative morbidity was 11% at 1 year, 2% at 2 to 4 years, 1% at 5 to 10 years, and less than 1% at more than 10 years. There were 24 deaths (11%); 22 followed CF-related pulmonary complications and included 8 of 16 (50%) children with pneumothorax.. Long-term survival in CF patients has improved significantly (89%), with many surviving into the fourth decade. MI may predispose to late complications including meconium ileus equivalent and fibrosing colonopathy. Pneumothorax in CF patients is an ominous predictor of mortality. Children with CF are living longer and are good candidates for operation, but require long-term follow-up evaluation because of ongoing exocrine dysfunction.

Topics: Abdomen; Adolescent; Adult; Bile Duct Diseases; Child; Child, Preschool; Cystic Fibrosis; Female; Humans; Ileus; Infant; Infant, Newborn; Intestinal Diseases; Intussusception; Liver Diseases; Male; Meconium; Pneumothorax; Postoperative Complications; Retrospective Studies; Survival Analysis; Thoracic Surgical Procedures; Treatment Outcome

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

To describe the prevalence of liver disease in a cohort of 241 cystic fibrosis (CF) patients.. 241 CF patients were followed-up every 3 months with clinical and biological assessment, and every year with ultrasonography of the liver. The presence of liver disease was studied using a multivariate Cox's regression analysis including variables such as history of meconium ileus, pulmonary function, pancreatic insufficiency and CFTR gene mutations.. The prevalence of liver disease was 18, 29, and 41% after 2, 5 and 12 years, respectively, and did not increase thereafter. In multivariate analysis, the probability of liver disease was independently associated with history of meconium ileus (P = 0.001) and pancreatic insufficiency (P = 0.004). CFTR mutations and severity of pulmonary disease were not associated with liver disease. Cirrhosis occurred in 19 (7.8%) patients at a median age of 10 years, and liver transplantation was required in five patients.. This study shows that CF related-liver disease occurs mainly in the first decade of life with a prevalence of 41% of patients at 12 years of age. A history of meconium ileus and pancreatic insufficiency are predictive of liver disease. Preventive treatment with ursodesoxycholic acid could be considered in patients with meconium ileus.

Topics: Adolescent; Age Distribution; Child; Child, Preschool; Cohort Studies; Cystic Fibrosis; Disease Progression; Exocrine Pancreatic Insufficiency; Female; Humans; Ileus; Incidence; Infant; Liver Diseases; Longitudinal Studies; Male; Meconium; Prevalence; Prognosis; Survival Analysis

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

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

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

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

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

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

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; 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; 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; 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; Fetal Diseases; Humans; Ileus; Infant, Newborn; Meconium; Polysaccharides

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

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