morphine and Short-Bowel-Syndrome

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

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

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

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

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