J Pediatr. 2021 Jun 18;S0022-3476(21)00555-2. doi: 10.1016/j.jpeds.2021.06.025.Online ahead of print.

Daniela Gattini ¹, Amin J Roberts ², Paul W Wales ³, Sue V Beath ⁴, Helen M Evans ⁵, Jonathan Hind ⁶, David Mercer ⁷, Theodoric Wong ⁴, Jason Yap ⁸, Christina Belza ⁹, Koen Huysentruyt ¹, Yaron Avitzur ¹⁰

Author information

• ¹Group for Improvement of Intestinal Function and Treatment (GIFT), Transplant Centre, Toronto, Ontario, Canada; Division of Gastroenterology, Hepatology & Nutrition, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
• ²Group for Improvement of Intestinal Function and Treatment (GIFT), Transplant Centre, Toronto, Ontario, Canada; Department of Paediatric Gastroenterology, Starship Child Health, University of Auckland, Auckland, New Zealand.
• ³Group for Improvement of Intestinal Function and Treatment (GIFT), Transplant Centre, Toronto, Ontario, Canada; Division of General & Thoracic Surgery, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
• ⁴Department of Gastroenterology and Nutrition, Nutrition Support and Intestinal Failure team, Birmingham Women's and Children's Hospital, Birmingham, United Kingdom.
• ⁵Department of Paediatric Gastroenterology, Starship Child Health, University of Auckland, Auckland, New Zealand.
• ⁶King's College Hospital, Paediatric Liver, GI & Nutrition Centre, London, United Kingdom.
• ⁷Organ Transplant Center, University of Nebraska Medical Center, Omaha, Nebraska, USA.
• ⁸Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
• ⁹Group for Improvement of Intestinal Function and Treatment (GIFT), Transplant Centre, Toronto, Ontario, Canada.
• ¹⁰Group for Improvement of Intestinal Function and Treatment (GIFT), Transplant Centre, Toronto, Ontario, Canada; Division of Gastroenterology, Hepatology & Nutrition, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. Electronic address: yaron.avitzur@sickkids.ca.

Abstract

Objectives: To assess the natural history and outcomes of children with intestinal failure in a large, multicenter and geographically diverse contemporary cohort (2010-2015) from 6 pediatric intestinal failure programs.

Study design: Retrospective analysis of a multicenter intestinal failure cohort (N=443). Competing risk analysis was used to obtain cumulative incidence rates for the primary outcome (enteral autonomy, transplantation or death). Chi-square test and Cox Proportional Hazards regressions were used for bivariate and multivariable analysis.

Results: 443 patients (male - 61.2%) constituted the study group. Primary etiologies included short bowel syndrome (SBS) (84.9%), dysmotility disorder (7.2%), and mucosal enteropathy (7.9%). Cumulative incidences for enteral autonomy, transplantation and death at 6-year of follow up were 53.0%, 16.7% and 10.5% respectively. Enteral autonomy was associated with SBS, ≥ 50% of small bowel length, presence of ileocecal valve (ICV), absence of portal hypertension, and follow-up in a non-high transplant volume center. The composite outcome transplantation/death was associated with persistent advanced cholestasis and hypoalbuminemia; age at diagnosis < 1 year, ICV, and intact colon were protective.

Conclusion: The rates of death and transplantation in children with intestinal failure have decreased, however, the number of children achieving enteral autonomy has not changed significantly, and a larger proportion of patients remain parenteral nutrition dependent. New strategies to achieve enteral autonomy are needed to improve patient outcome.