Il trattamento della sindrome da intestino corto: vecchie e nuove terapie

Abstract

Intestinal failure (IF) is an acute or chronic condition characterized by a so severe reduced intestinal function that does not allow a stable nutritional status and a normal child growth. The “so-called” chronic benign intestinal failure (CBIF) is mainly caused by neonatal post-surgical short bowel syndrome (SBS) and less frequently by congenital motility disorders or by structural abnormalities of the intestinal epithelium. The correct management of CBIF requires a multidisciplinary approach whose target is the enteral autonomy that means the weaning from parenteral nutrition, a rescue therapy for these patients. In the last few years, a lot of attention has been paid to the identification of drugs able to stimulate the intestinal adaptation. Among these drugs teduglutide, a glucagon-like peptide-2 analogue, approved in Italy for children from January 2021, showed to be effective for the treatment of SBS in children aged one year or more. This intestinotrophic peptide improves intestinal capacity for fluid and nutrients absorption through the induction of mucosal growth and reduction of gastrointestinal motility, thus reducing the need of parenteral support. The paper reports the principal experiences in paediatrics by collecting the results in 104 patients. At present the working group of the Italian Society for Paediatric Gastroenterology, Hepatology and Nutrition (SIGENP) is trying to standardize and share how to use teduglutide in Italian specialized setting.