Severe short-bowel syndrome in children. Clinical experience

introduction: Innovative surgical and pharmacological therapeutic measures In short-bowel syndrome (SBS) are constantly changing the prognosis of this devastating condition. The aim of this paper is to present our most recent experience in the treatment of this disease, with particular emphasis on t he impact of home parenteral nutrition (HPN) and the use of growth hormone (GH). Methods: A group of 8 patients with severe SBS have been studied for the past 4 years. intestinal length of less than 25% normal at the time of bowel resection was the criterion far inclusion in this study. Results: Mea n age at the time of diagnosis was 2 years (ranging from 1 day to 9 years). The etiology of the SBS was Hirchsprung's disease (n = 3), midgut volvulus (n = 2), gastroschisis (n = 1), omphalocele with ileal atresia and necroti zing enterocolitis (n = 1) and Crohn's disease (n = 1). Length of the resid ual bowel was 8 and SO cm with ileocecal valve (ICV) preservation and 23, 2 7, 30, 50, 70, 100 cm without ICV. Sixty percent of the patients survived. Two patients died due to fulminant gram-negative sepsis and one due to card iac malformation. Two patients are still on parenteral nutrition (PN) provi ding 30 and 60% of total calories. Human GH (0.3 U/kg/day) was used in two patients over a period of 28 days. In these patients, an increased toleranc e to enteral feeding was observed. HPN was provided in 5 cases, allowing re gular school attendance in 3 patients. In 3 cases, discontinuation of the P N was achieved at 24, 25 and 35 months respectively. Conclusions: Human GH can improve tolerance of enteral feeding. HPN has a beneficial effect on ch ild behaviour. Intestinal transplantation must be considered when no other surgical or medical measures are available.