title: Combined en bloc liver pancreas transplantation for children with CF.
abstract: Cystic fibrosis (CF) is an inherited genetic defect in epithelial chloride transport that results a multisystem disease affecting the sweat glands and the pulmonary and digestive systems. Although pulmonary disease remains the primary cause of morbidity and mortality in these patients, up to 8% may develop focal biliary cirrhosis and portal hypertension. Liver transplantation is an accepted therapy for these patients. About 85% of CF patients develop pancreatic exocrine insufficiency and up to 34% will develop diabetes requiring insulin. We describe a series of 9 patients who underwent liver transplantation (6 transplantation of liver only [LO], and 3 combined en bloc liver-pancreas [LP] transplantation) for CF-related liver disease at our institution,. All 9 patients had pretransplant pancreatic exocrine insufficiency requiring enzyme supplementation. Of the 9, 5 patients (55%) had preoperative insulin dependent diabetes, including all 3 patients who underwent liver pancreas transplantation. Liver-pancreas transplants were performed en bloc. One-year patient survival for both LO and LP patients was 100%. Five-year patient survival was 83% for the LO patients and 100% for the LP patients. All LP patients are off insulin and do not require pancreatic enzyme supplementation. All patients receiving LO continue to need exogenous pancreatic enzyme supplementation. In the 6 patients who received LO, 2 were insulin dependent prior to surgery, and they remain insulin dependent after transplant. Of the 4 patients who received LO who were not insulin dependent pretransplant, 3 have now developed insulin-dependent diabetes. Patients receiving LO were more likely to be diabetic and require exogenous pancreatic enzymes after transplant than those receiving LP (83% vs. 0%, P = 0.04, and 100% vs. 0%, P = 0.01, respectively). In conclusion, patients with CF and end-stage liver disease requiring liver transplantation may benefit from combined liver-pancreas transplantation.
authors.affiliation: Department of Surgery, University of Florida College of Medicine, Gainesville, FL 32610, USA.
authors.forename: Kristin L
authors.initials: KL
authors.lastname: Mekeel
authors.forename: Max R
authors.initials: MR
authors.lastname: Langham
authors.forename: Regino
authors.initials: R
authors.lastname: Gonzalez-Perralta
authors.forename: Alan
authors.initials: A
authors.lastname: Reed
authors.forename: Alan W
authors.initials: AW
authors.lastname: Hemming
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications / Q000601:surgery*; D003920:Diabetes Mellitus / Q000209:etiology; D006085:Graft Survival; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D007328:Insulin / Q000378:metabolism; D008105:Liver Cirrhosis, Biliary / Q000209:etiology / Q000601:surgery; D008107:Liver Diseases / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation / Q000009:adverse effects / Q000379:methods*; D016035:Pancreas Transplantation / Q000009:adverse effects / Q000379:methods*; D016896:Treatment Outcome
chemical_list: D007328:Insulin