title: Natural history of pediatric intestinal failure: initial report from the Pediatric Intestinal Failure Consortium.
abstract: OBJECTIVE
  To characterize the natural history of intestinal failure (IF) among 14 pediatric centers during the intestinal transplantation era.
  
  
  METHODS
  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.
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA 15224, USA. squiresr@upmc.edu
authors.forename: Robert H
authors.initials: RH
authors.lastname: Squires
authors.forename: Christopher
authors.initials: C
authors.lastname: Duggan
authors.forename: Daniel H
authors.initials: DH
authors.lastname: Teitelbaum
authors.forename: Paul W
authors.initials: PW
authors.lastname: Wales
authors.forename: Jane
authors.initials: J
authors.lastname: Balint
authors.forename: Robert
authors.initials: R
authors.lastname: Venick
authors.forename: Susan
authors.initials: S
authors.lastname: Rhee
authors.forename: Debra
authors.initials: D
authors.lastname: Sudan
authors.forename: David
authors.initials: D
authors.lastname: Mercer
authors.forename: J Andres
authors.initials: JA
authors.lastname: Martinez
authors.forename: Beth A
authors.initials: BA
authors.lastname: Carter
authors.forename: Jason
authors.initials: J
authors.lastname: Soden
authors.forename: Simon
authors.initials: S
authors.lastname: Horslen
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Rudolph
authors.forename: Samuel
authors.initials: S
authors.lastname: Kocoshis
authors.forename: Riccardo
authors.initials: R
authors.lastname: Superina
authors.forename: Sharon
authors.initials: S
authors.lastname: Lawlor
authors.forename: Tamara
authors.initials: T
authors.lastname: Haller
authors.forename: Marcia
authors.initials: M
authors.lastname: Kurs-Lasky
authors.forename: Steven H
authors.initials: SH
authors.lastname: Belle
mesh_terms: D020345:Enterocolitis, Necrotizing / Q000453:epidemiology; D005260:Female; D020139:Gastroschisis / Q000453:epidemiology; D006627:Hirschsprung Disease / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007409:Intestinal Atresia / Q000453:epidemiology; D007410:Intestinal Diseases / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery / Q000628:therapy*; D045822:Intestinal Volvulus / Q000453:epidemiology; D007422:Intestines / Q000637:transplantation; D008297:Male; D010288:Parenteral Nutrition; D011379:Prognosis; D012189:Retrospective Studies