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
Division of Pediatric Gastroenterology, Hepatology and Nutrition, SickKids Transplant Centre, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G1X8, Canada.
Pediatric Gastroenterology/Hepatology/Nutrition, Indiana University School of Medicine, Riley Hospital for Children, 705 Riley Hospital Drive, Indianapolis, IN 46202, USA.
J Cyst Fibros. 2014 Jul;13(4):471-7. doi: 10.1016/j.jcf.2013.12.010. Epub 2014 Jan 16.
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 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).
Patients with CFLD undergoing initial assessment for liver transplantation may benefit from consideration of simultaneous liver-pancreas transplantation.
糖尿病与囊性纤维化（CF）患者的发病率和死亡率增加相关。虽然肝移植对于CF相关肝病（CFLD）已确立，但同期肝胰联合移植的作用了解较少。
我们对81个儿科移植中心进行了调查，以确定和描述接受同期肝胰联合移植的受试者，并获取关于CFLD中该手术的意见。
50个（61.7%）接受调查的移植中心做出了回应，94%报告称他们会考虑对CFLD和糖尿病患者进行同期肝胰联合移植。共确定了8例接受同期肝胰联合移植的患者，中位随访时间为38个月。所有患者均有糖尿病史。所有患者的外分泌和内分泌胰腺功能最初均得以恢复，但有1例患者后来出现功能丧失。移植前1年至移植后1年期间，体重指数Z评分升高（P=0.029）。
接受肝移植初始评估的CFLD患者可能会从同期肝胰联合移植的考虑中获益。
