Combined heterotopic liver–pancreas transplantation as a curative treatment for liver cirrhosis and diabetes mellitus in cystic fibrosis

Abstract

Cystic fibrosis (CF) is an inherited disease with a defect in epithelial chloride transport that results in a multisystem disease. Although pulmonary disease remains the primary cause of morbidity and mortality, focal biliary cirrhosis and portal hypertension may develop in up to 8% of these patients. Liver transplantation (TX) is an accepted therapy and shows good results. We report on a patient with cystic fibrosis homozygous for the most common CFTR mutation delta F 508 who received a combined heterotopic liver and pancreas transplantation at the age of 18 yr. He suffered from CFRD, which untypically required high doses of insulin. In addition, the patient had pulmonary complications, was chronically colonized with multiresistant Pseudomonas aeruginosa (MBL) and had an allergic bronchopulmonary aspergillosis (ABPA). The patient remained in stable health for 54 months post-TX and was able to live a nearly normal life. With a follow-up of five yr, the function of the liver and pancreas allografts was excellent. However, and sadly, his pulmonary function continued to deteriorate from progression of his CF, and he died of respiratory failure due to a severe pneumonia and septicemia at the age of 23 yr and five months.

Combined lung-liver-pancreas transplantation in a recipient with cystic fibrosis

“…Pancreas transplantation improves quality of life and nutritional status, resulting in enhanced growth and weight gain. The limited experience to date suggests that pancreas graft survival is excellent, especially when combined with liver or kidney transplantation [7][8][9][10][11][12]14]. Fridell et al reported three patients who received simultaneous lung and pancreas transplants, all of whom achieved insulin and enzyme independence [13].…”

“…Although several reports highlight the potential benefits of pancreas transplantation in CFRD, it remains rare with only 28 transplants documented in the United States between 1987 and 2014 [6]. The majority of these pancreas transplants were combined with a liver transplant, but kidney-pancreas, lung-pancreas, and pancreas-alone transplants have also been successfully performed for CFRD [7][8][9][10][11][12][13]. Herein, we report the first successful combined lung, liver, and pancreas transplantation.…”

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

“…There is at least one case series that reported proximal enteric drainage in combined pancreas–lung transplantation for CF patients were able to discontinue all pancreatic enzyme supplements . Similarly, there are multiple case reports of recipients’ en bloc liver–pancreas transplant recipients who became independent of pancreatic enzyme supplements . Given these findings, it may be beneficial to attempt proximal enteric drainage in pancreas transplants performed in the CF population.…”

“…Pancreas transplantation could improve quality of life as well as improve diabetes control and nutritional status through adequate pancreatic exocrine function. Case series are limited to single‐center reports of patients undergoing pancreas transplantation in combination with lung (two series) or liver (six series) (Table , ).…”

A simplified multivisceral transplantation procedure for patients with combined end‐stage liver disease and type 2 diabetes mellitus

“…Combined heterotopic liver and pancreas transplantation, a standard OLT and heterotopic pancreas transplantation in the right lower quadrant, is an infrequent option for patients with end‐stage liver disease and concurrent DM . Patients may not benefit from the transplantation because the procedure is associated with a higher incidence of complications and a lower longterm pancreas graft survival rate.…”