Congenital enteropathy and intestinal transplantation

Abstract

Intestinal failure (IF) requires the use of parenteral nutrition (PN). Causes of severe protracted IF include short bowel syndrome, severe motility disorders (total or sub-total aganglionosis or chronic intestinal pseudo-obstruction syndrome) and congenital diseases of enterocyte development. Severe liver disease may develop in patients with IF as a consequence of both underlying digestive disease and unadapted PN. Catheter-related sepsis and/or extensive vascular thrombosis may impede the continuation of safe and efficient PN. Thus management of patients with IF requires early recognition of the condition and the analysis of its risk of irreversibility. Timing of referral for intestinal transplantation remains a crucial issue. As a consequence, management should include therapies adapted to each stage of IF based on a multi-disciplinary approach in centers involving pediatric gastroenterology, PN expertise, home-PN program, pediatric surgery, liver-intestinal transplantation program. Copyright © 2006 Nestec Ltd., Vevey/S. Karger AG.