Under Utilization of Pancreas Transplants in Cystic Fibrosis Recipients in the United Network Organ Sharing (UNOS) Data 1987–2014

Abstract

Despite a high prevalence of pancreatic endocrine and exocrine insufficiency in cystic fibrosis (CF), pancreas transplantation is rarely reported. United Network for Organ Sharing (UNOS) data were used to examine utilization of pancreas transplant and posttransplant outcomes in CF patients. Between 1987–2014, CF patients (N = 4600) underwent 17 liver–pancreas, three lung–pancreas, one liver–lung pancreas, four kidney–pancreas, and three pancreas-only transplants. Of the 303 CF patients who received liver transplantation, 20% had CF-related diabetes (CFRD) before transplantation, and nine of those received a liver–pancreas transplant. Of 4241 CF patients who underwent lung transplantation, 33% had CFRD before transplantation, and three of those received a pancreas transplant. Of 49 CF patients who received a liver–lung transplant, 57% had CFRD before transplantation and one received a pancreas transplant. Posttransplantation diabetes developed in 7% of CF pancreas transplant recipients versus 24% of CF liver and 29% of CF lung recipients. UNOS has no data on pancreas exocrine insufficiency. Two-year post-transplantation survival was 88% after liver–pancreas transplant, 33% after lung–pancreas transplant, and 100% after pancreas–kidney and pancreas-only transplants. Diabetes is common pretransplantation and posttransplantation in CF solid organ transplant recipients, but pancreas transplantation remains rare. Further consideration of pancreas transplant in CF patients undergoing other solid organ transplant may be warranted.

A Multidisciplinary Approach to Pretransplant and Posttransplant Management of Cystic Fibrosis–Associated Liver Disease

“…Two single‐center series reported 92% and 89% 1‐year survival after LT with deaths beyond 3 years primarily from progression of pulmonary disease . More recently, LP transplants have been described . In 8 children who had undergone LP with a median follow‐up of 38 months, exocrine and endocrine function were restored in all patients as defined by cessation of pancreatic enzymatic supplementation and exogenous insulin while maintaining normal glucose concentrations.…”

“…(45) More recently, LP transplants have been described. (46,47) In 8 children who had undergone LP with a median follow-up of 38 months, exocrine and endocrine function were restored in all patients as defined by cessation of pancreatic enzymatic supplementation and exogenous insulin while maintaining normal glucose concentrations. Median body mass index (BMI) z score also increased from pretransplant levels of -0.95 (range, 2.49) to -0.07 (range, 1.16) 1 year after transplant.…”

Diabetes mellitus in pediatric solid organ recipients without and with cystic fibrosis: An analysis from the German-Austrian diabetes database (Diabetes Patienten Verlaufsdokumentation)

“…PTDM patients with CF had the lowest weight and BMI z‐scores. From our data on CF patients needing SOT, however, it is difficult to determine whether increased disease severity affected weight development or low weight was a risk factor for SOT …”

“…From our data on CF patients needing SOT, however, it is difficult to determine whether increased disease severity affected weight development or low weight was a risk factor for SOT. 11,26 Hyperglycemia and diabetes development in SOT is associated with a higher percentage of cardiovascular risk factors. 27 In our study, 30 The effect of steroids and steroid withdrawal on height was investigated by Bhakta et al 31 One year after transplantation, mean height-z-score was −0.8 and there was no significant difference in height SD between the steroid-free and steroidtreated groups of kidney-transplanted children, bringing into focus other factors than steroids, for example, bodyweight.…”

Combined Pancreatic Islet-Lung-Liver Transplantation in a Pediatric Patient with Cystic Fibrosis-Related Diabetes

“…IT results in decreased insulin needs and improved metabolic control in patients with previous or simultaneous lung transplantation [7-9]. While pancreas transplantations, in combination with liver and/or lung transplantations, have previously been described [10, 11], we report the first case of a combined islet-lung-liver transplantation in a pediatric patient who developed CFRD unusually early, possibly due to a genetic predisposition and in whom islets were infused into the donor liver.…”