morphine and Biliary-Tract-Diseases

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

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

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

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