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.
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