Simultaneous liver-pancreas transplantation for cystic fibrosis-related liver disease: a multicenter experience

J Cyst Fibros. 2014 Jul;13(4):471-7. doi: 10.1016/j.jcf.2013.12.010. Epub 2014 Jan 16.

Abstract

Background: Diabetes is associated with increased morbidity and mortality in patients with cystic fibrosis (CF). While liver transplantation is well established for CF-related liver disease (CFLD), the role of simultaneous liver-pancreas transplantation is less understood.

Methods: We polled 81 pediatric transplantation centers to identify and characterize subjects who had undergone simultaneous liver-pancreas transplantation and obtain opinions about this procedure in CFLD.

Results: Fifty (61.7%) polled transplant centers responded and 94% reported that they would consider simultaneous liver-pancreas transplantation for CFLD and diabetes. A total of 8 patients with simultaneous liver-pancreas transplantation were identified with median follow up of 38 months. All patients had pre-existing diabetes. Exocrine and endocrine pancreatic function was initially restored in all patients with later functional loss in one patient. Body mass index Z-score increased between one year pre-transplantation and one year post-transplantation (P=0.029).

Conclusions: Patients with CFLD undergoing initial assessment for liver transplantation may benefit from consideration of simultaneous liver-pancreas transplantation.

Keywords: Clinical; Cohort; Cystic fibrosis; Diabetes; Retrospective; Simultaneous liver pancreas transplantation.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Child
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / surgery
  • Female
  • Follow-Up Studies
  • Humans
  • Liver Diseases / etiology
  • Liver Diseases / surgery*
  • Liver Transplantation / methods*
  • Male
  • Pancreas Transplantation / methods*
  • Treatment Outcome
  • Young Adult