Redefining short bowel syndrome in the 21st century

Abstract

In 1968, Wilmore and Dudrick reported an infant sustained by parenteral nutrition (PN) providing a potential for survival for children with significant intestinal resections. Increasing usage of TPN over time led to some patients developing Intestinal Failure Associated Liver Disease (IFALD), a leading cause of death and indication for liver/intestinal transplant. Over time, multidisciplinary teams called Intestinal Rehabilitation Programs (IRPs) began providing meticulous and innovative management. Usage of alternative lipid emulsions and lipid minimization strategies have resulted in the decline of IFALD and an increase in long-term and transplant-free survival, even in the setting of ultrashort bowel (< 20 cm). Autologous bowel reconstructive surgeries, such as the serial tapering enteroplasty procedure, have increased the likelihood of achieving enteral autonomy. Since 2007, the number of pediatric intestinal transplants performed has sharply declined and likely attributed to the newer innovations healthcare. Recent data support the need for changes in the listing criteria for intestinal transplantation given the overall improvement in outcomes. Over the last 50 y, the diagnosis of short bowel syndrome has changed from a death sentence to one of hope with a vast improvement of quality of life and survival.

Nutritional therapy complications in children with ultra‐short bowel syndrome include growth deficiency but not cholestasis

“…Intestinal failure‐associated liver failure, sepsis, thrombosis and an increased number of surgical interventions appear to make the prognosis worse for these patients, but fish‐oil lipid emulsions have been reported to open up new perspectives on the prevention of liver failure . Tremendous advances in the provision of PN have resulted in significant improvements being reported in the survival and quality of life of affected children . Cholestasis has been defined as elevated serum conjugated bilirubin of more than 2 mg/dL (34.2 μ mol/L) in parenterally fed patients .…”

Síndrome de intestino corto.

“…El SIC es una reducción en la longitud del ID inferior al mínimo requerido para una absorción adecuada de nutrientes que se debe a malformaciones congénitas o resección, dando como resultado malabsorción (1,2,3,4,5,6,7,8,9,11,12,13). Es la principal causa de falla intestinal en niños (1,2,3), su espectro clínico va desde una disfunción leve y reversible hasta una condición grave e incapacitante (2).…”

Key Determinants for Achieving Enteral Autonomy and Reduced Parenteral Nutrition Exposure in Pediatric Intestinal Failure

“…Since the first documented use of parenteral nutrition (PN) in a pediatric patient in 1968, the landscape of intestinal failure (IF) management has changed significantly . Improvements in line care, nutrition delivery, and infection prevention have led to a decrease in the incidence of IF‐associated liver disease and intestinal transplantation . Additionally, the importance of a multidisciplinary approach for intestinal rehabilitation and the need for ongoing collaboration and research has been established …”