title: Simultaneous liver and pancreas transplantation in patients with cystic fibrosis.
abstract: BACKGROUND
  Improved survival in patients with cystic fibrosis (CF) has led to an increased incidence of extrapulmonary complications of this disease. Of these, cirrhosis and pancreatic insufficiency, including CF-related diabetes (CFRD) and exocrine insufficiency, are significant causes of morbidity and mortality. Liver transplantation is the treatment of choice for cirrhosis in this setting, but the addition of an isolated simultaneous pancreas transplant in patients with CFRD has not been reported.
  
  
  METHODS
  Two female patients with CF underwent simultaneous pancreas and liver transplantation. Both had pancreatic insufficiency, CFRD, cirrhosis, and preserved renal function. In each case, the liver and pancreas were procured from a single cadaveric donor. The liver transplant was performed first. A lower midline extension was added for improved exposure of the iliac vessels. The donor pancreas transplant was performed with systemic venous drainage and enteric exocrine drainage. Immunosuppression included rabbit anti-thymocyte globulin, tacrolimus, mycophenolate mofetil, and early steroid withdrawal.
  
  
  RESULTS
  Both patients recovered well with normal liver function, resolution of portal hypertension, and normal blood glucoses independent of insulin. As a result of the enteric exocrine drainage of the pancreas, they are now independent of supplemental pancreatic enzymes.
  
  
  CONCLUSIONS
  Simultaneous liver and pancreas transplantation in CF patients provides the advantages of normalization of glucose and improved nutrition for patients requiring liver transplantation and should be considered in CF patients with CFRD who require liver transplants.
authors.affiliation: Department of Surgery, Indiana University, 550 N. University Boulevard #4258, Indianapolis, IN 46202, USA. jfridell@iupui.edu
authors.forename: J A
authors.initials: JA
authors.lastname: Fridell
authors.forename: R
authors.initials: R
authors.lastname: Vianna
authors.forename: P Y
authors.initials: PY
authors.lastname: Kwo
authors.forename: M
authors.initials: M
authors.lastname: Howenstine
authors.forename: A
authors.initials: A
authors.lastname: Sannuti
authors.forename: J P
authors.initials: JP
authors.lastname: Molleston
authors.forename: M D
authors.initials: MD
authors.lastname: Pescovitz
authors.forename: A J
authors.initials: AJ
authors.lastname: Tector
mesh_terms: D000293:Adolescent; D000328:Adult; D003550:Cystic Fibrosis / Q000150:complications / Q000601:surgery*; D003920:Diabetes Mellitus / Q000209:etiology / Q000601:surgery; D005260:Female; D006801:Humans; D016031:Liver Transplantation / Q000379:methods*; D016035:Pancreas Transplantation / Q000379:methods*; D016896:Treatment Outcome