New Frontiers in the treatment of intestinal failure in short bowel syndrome
1Great Ormond Street Hospital, London
Short gut or short bowel syndrome (SBS) can be defined as insufficient small intestinal mass to facilitate sufficient intestinal absorption to support homeostasis and child normal growth and development. Children are usually dependent on parenteral nutrition (PN). Currently the most successful treatment strategy is for the patient to be managed in a specialist intestinal failure (IF) rehabilitation centre by a multi disciplinary team. A new treatment available for children who are stable on PN and unable to wean onto enteral nutrition is a glucagon-like peptide 2(GLP-2) analogue, teduglutide or revestive that showed to be effective in reducing PN need. Despite there has been a marked improvement in the outcome of these patient streated in specialist IF centres there is still a limited role for Intestinal Transplant in children who ‘fail’ PN treatment but it has to be considered that the outcome for PN is about 95% long-term/10 year survival. In contrast the worldwide 5-year survival for intestinal transplant is just 75%. With newer treatments such as ‘teduglutide’ there is an even greater chance that children with SBS will successfully wean off PN. A child born today who develops SBS is most likely to survive through out childhood and into adult life with a good quality of life even if she/he remains dependent on PN.