Intestinal adaptation in pediatric patients with short-bowel syndrome.

The purpose of this study is to evaluate 12 pediatric short-bowel syndrome (SBS) patients experienced at Osaka University Hospital and its affiliated hospitals and to study the intestinal length for achieving intestinal adaptation and the metabolic characteristics. The length of the residual small intestine ranged from 0 to 75 cm with an average of 47 cm and an ileocecal valve had been resected in five cases. Total parenteral nutrition (TPN) was started immediately after operation and was gradually substituted by enteral nutrition. No patient died during the follow-up period. Eight of 12 patients could be weaned from TPN with residual intestinal length of 27 to 75 cm (mean 57 cm). Four patients with the residual intestine of 0 to 45 cm (mean 22 cm) were unable to achieve intestinal adaptation. The rate of catheter-related sepsis per 1000 catheter days was 0.63. Fatty liver was detected in two cases, but no patient developed progressive liver failure. Plasma arginine and citrulline were decreased in patients who were unable to achieve intestinal adaptation. Our nutritional support program provided excellent survival for pediatric SBS patients primarily due to the low incidence of catheter-related sepsis and no episode of severe liver disease. Patients with more than 16 cm of residual intestinal length can be expected to be weaned from TPN.