title: Restoration of exocrine pancreatic function following pancreas-liver-kidney transplantation in a cystic fibrosis patient.
abstract: Pancreatic transplantation for endocrine replacement is well-established for insulin-dependent diabetes mellitus. Exocrine pancreatic function after pancreas transplantation has been maintained after orthotopic cluster transplants for malignancy, and restoration of adequate exocrine function in a previously deficient patient has been reported in a patient with chronic pancreatitis who developed labile diabetes and steatorrhea after pancreatectomy. We performed a triple organ transplant (pancreas, liver and kidney) in a patient with exocrine pancreatic insufficiency and insulin-dependent diabetes related to cystic fibrosis (CF) after he developed hepatic and renal failure. Pancreatic exocrine secretions were drained enterically to the jejunum. At 24-month follow-up, malabsorption is absent. The 3-day stool fat, stool trypsin and chymotrypsin are normal. Serum carotene is within the normal range. Exocrine pancreatic insufficiency in CF patients can be corrected by pancreas transplantation. However, routine use in CF is precluded by the risks of surgery and immunosuppression. For diabetic patients with pancreatic exocrine insufficiency who require another organ transplant (e.g., lung, liver, or kidney), simultaneous pancreas transplantation with the exocrine secretions directed into the upper gastrointestinal tract should be considered.
authors.affiliation: Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio.
authors.forename: R C
authors.initials: RC
authors.lastname: Stern
authors.forename: J T
authors.initials: JT
authors.lastname: Mayes
authors.forename: F L
authors.initials: FL
authors.lastname: Weber
authors.forename: E W
authors.initials: EW
authors.lastname: Blades
authors.forename: J A
authors.initials: JA
authors.lastname: Schulak
mesh_terms: D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications* / Q000503:physiopathology; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000601:surgery; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications / Q000601:surgery; D016030:Kidney Transplantation; D008103:Liver Cirrhosis / Q000150:complications / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D010179:Pancreas / Q000378:metabolism*; D016035:Pancreas Transplantation; D010193:Pancreaticojejunostomy; D010195:Pancreatitis / Q000150:complications