Intestinal failure in childhood

Abstract

Intestinal failure (IF) requires the use of parenteral nutrition (PN) for as long as it persists and in case of irreversible IF may be an indication for intestinal transplantation (ITx). Biological evaluation of IF is becoming possible with the use of plasma citrulline as a marker of intestinal mass. Short bowel syndrome (SBS) is the leading cause of intestinal failure in infants while few epidemiological data are to date available. Data on morbidity and mortality in paediatric patients with SBS are very limited but long-term outcomes seem to be improving. Other causes of intestinal failure include neuro-muscular intestinal disease and congenital disease of enterocyte development. The management of IF should include therapies adapted to each type and stage of IF based on a multidisciplinary approach in centres involving paediatric surgery, paediatric gastroenterology, parenteral nutrition expertise, home-parenteral nutrition programme, liver-intestinal transplantation experience. Timing for referral of patients in specialised centres remains a crucial issue.

Key words:

• citrulline
• congenital enteropathy
• home parenteral nutrition chronic intestinal pseudoobstruction
• intestinal failure
• intestinal transplantation
• liver transplantation
• parenteral nutrition
• short bowel syndrome