Natural history of pediatric intestinal failure: Initial report from the pediatric intestinal failure consortium

Robert H. Squires, Christopher Duggan, Daniel H. Teitelbaum, Paul W. Wales, Jane Balint, Robert Venick, Susan Rhee, Debra Sudan, David Mercer, J. Andres Martinez, Beth A. Carter, Jason Soden, Simon Horslen, Jeffrey A. Rudolph, Samuel Kocoshis, Riccardo Superina, Sharon Lawlor, Tamara Haller, Marcia Kurs-Lasky, Steven H. Belle

Research output: Contribution to journalArticlepeer-review

250 Scopus citations

Abstract

Objective: To characterize the natural history of intestinal failure (IF) among 14 pediatric centers during the intestinal transplantation era. Study design: The Pediatric Intestinal Failure Consortium performed a retrospective analysis of clinical and outcome data for a multicenter cohort of infants with IF. Entry criteria included infants <12 months receiving parenteral nutrition (PN) for >60 continuous days. Enteral autonomy was defined as discontinuation of PN for >3 consecutive months. Values are presented as median (25th, 75th percentiles) or as number (%). Results: 272 infants with a gestational age of 34 weeks (30, 36) and birth weight of 2.1 kg (1.2, 2.7) were followed for 25.7 months (11.2, 40.9). Residual small bowel length in 144 patients was 41 cm (25.0, 65.5). Diagnoses were necrotizing enterocolitis (71, 26%), gastroschisis (44, 16%), atresia (27, 10%), volvulus (24, 9%), combinations of these diagnoses (46, 17%), aganglionosis (11, 4%), and other single or multiple diagnoses (48, 18%). Prescribed medications included oral antibiotics (207, 76%), H2 blockers (187, 69%), and proton pump inhibitors (156, 57%). Enteral feeding approaches varied among centers; 19% of the cohort received human milk. The cohort experienced 8.9 new catheter-related blood stream infections per 1000 catheter days. The cumulative incidences for enteral autonomy, death, and intestinal transplantation were 47%, 27%, and 26%, respectively. Enteral autonomy continued into the fifth year after study entry. Conclusions: Children with IF endure significant mortality and morbidity. Enteral autonomy may require years to achieve. Improved medical, nutritional, and surgical management may reduce time on PN, mortality, and need for transplantation.

Original languageEnglish (US)
Pages (from-to)723-728.e2
JournalJournal of Pediatrics
Volume161
Issue number4
DOIs
StatePublished - Oct 2012

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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