Simultaneous liver-pancreas transplantation for cystic fibrosis-related liver disease: a multicenter experience.
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.
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.
R H J
RH
Bandsma
Pediatric Gastroenterology/Hepatology/Nutrition, Indiana University School of Medicine, Riley Hospital for Children, 705 Riley Hospital Drive, Indianapolis, IN 46202, USA.
M A
MA
Bozic
Department of Surgery, University of Indiana, School of Medicine, 550N University Blvd., Indianapolis, IN 46202, USA.
J A
JA
Fridell
Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Hanzeplein 1, 9700 RB Groningen, The Netherlands.
M H
MH
Crull
Pediatric Gastroenterology/Hepatology/Nutrition, Indiana University School of Medicine, Riley Hospital for Children, 705 Riley Hospital Drive, Indianapolis, IN 46202, USA.
J
J
Molleston
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.
Y
Y
Avitzur
Institute of Gastroenterology, Nutrition, and Liver Disease, Schneider Children's Medical Center of Israel, 14 Kaplan Street, Petach Tikvah 49202, Israel.
Y
Y
Mozer-Glassberg
Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Florida College of Medicine, UF Health-Shands Children's Hospital, 1600 SW Archer Road, Gainesville, FL 32608, USA.
R P
RP
Gonzalez-Peralta
Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Florida College of Medicine, UF Health-Shands Children's Hospital, 1600 SW Archer Road, Gainesville, FL 32608, USA.
M
M
Hodik
Division of Surgery, Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, Ontario M5G1X8, Canada.
A
A
Fecteau
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.
M
M
de Angelis
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; Physiology and Experimental Medicine, Research Institute, Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G1X8, Canada.
P
P
Durie
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. Electronic address: vicky.ng@sickkids.ca.
V L
VL
Ng
D000293:Adolescent; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008107:Liver Diseases / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D016035:Pancreas Transplantation / Q000379:methods*; D016896:Treatment Outcome; D055815:Young Adult
Clinical; Cohort; Cystic fibrosis; Diabetes; Retrospective; Simultaneous liver pancreas transplantation