Send to

Choose Destination
Liver Transpl. 2007 Mar;13(3):406-9.

Combined en bloc liver pancreas transplantation for children with CF.

Author information

Department of Surgery, University of Florida College of Medicine, Gainesville, FL 32610, USA.


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.

[Indexed for MEDLINE]
Free full text

Supplemental Content

Full text links

Icon for Wiley
Loading ...
Support Center