Review: The Long and Short of IT: intestinal failure-associated liver disease (IFALD) in adults—recommendations for early diagnosis and intestinal transplantation

摘要

Intestinal failure-associated liver disease (IFALD) often presents in adults unexpectedly with advanced disease. Non-invasive tests can be falsely reassuring. Patients with ‘ultrashort’ intestine (<20 cm) ending in a stoma are at particular risk of developing IFALD, which may occur rapidly. Recent experience and studies suggest that IFALD can be reversed by isolated intestine transplant occurring before the development of high grade fibrosis or cirrhosis. Post-transplant survival is superior for isolated intestinal grafts compared with liver containing intestinal grafts; waiting time and waiting list mortality is higher for a combined graft, and donor liver supply is limited. Therefore, the aim of clinicians treating patients with intestinal failure should be to identify IFALD early and refer to an intestinal transplant centre while isolated intestine transplantation can be contemplated and before the liver disease has progressed to a stage requiring consideration of combined liver and intestinal transplantation.