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

• Published in: Journal of cystic fibrosis Vol. 13; no. 4; pp. 471 - 477
• Main Authors: Bandsma, R.H.J., Bozic, M.A., Fridell, J.A., Crull, M.H., Molleston, J., Avitzur, Y., Mozer-Glassberg, Y., Gonzalez-Peralta, R.P., Hodik, M., Fecteau, A., de Angelis, M., Durie, P., Ng, V.L.
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.07.2014
• Subjects: Adolescent; Child; Clinical; Cohort; Cystic fibrosis; Cystic Fibrosis - complications; Cystic Fibrosis - surgery; Diabetes; Female; Follow-Up Studies; Humans; Liver Diseases - etiology; Liver Diseases - surgery; Liver Transplantation - methods; Male; Pancreas Transplantation - methods; Pulmonary/Respiratory; Retrospective; Simultaneous liver pancreas transplantation; Treatment Outcome; Young Adult; Clinical; Cystic fibrosis; Simultaneous liver pancreas transplantation; Diabetes; Cohort; Retrospective
• ISSN: 1569-1993; 1873-5010; 1873-5010
• DOI: 10.1016/j.jcf.2013.12.010

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. 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. 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 38months. 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). Patients with CFLD undergoing initial assessment for liver transplantation may benefit from consideration of simultaneous liver–pancreas transplantation.