title: The optimal timing of referral to an intestinal failure program: the relationship between hyperbilirubinemia and mortality.
abstract: OBJECTIVE
  Multidisciplinary treatment of pediatric intestinal failure has shown promising results. However, there are limited data as to the optimal time frame for referral of patients to intestinal failure programs. The aim of this study was to explore the relationship of hyperbilirubinemia at referral with patient outcomes in a multidisciplinary program.
  
  
  METHODS
  A retrospective analysis was performed of a prospectively collected database from a multidisciplinary intestinal failure program. Multivariable logistic regression adjusted for age at referral was used to model the association between the conjugated bilirubin at referral and risk of mortality. Median values with range are reported.
  
  
  RESULTS
  Sixty-two patients were referred from 2005 to 2009. Patients presented at age 6.4 months (0.4-261.4 months) and were followed up for 16.8 (0.3-53.0) months. Nine subjects (14.5%) died, and 12 subjects (19.4%) were listed for combined liver-intestine transplant. A 50% mortality was seen in patients referred with a conjugated bilirubin ≥ 7.2 mg/dL (n = 12), whereas mortality at referral bilirubin levels <7.2 mg/dL was 6%. After adjusting for age at referral, patients with a conjugated bilirubin ≥ 7.2 mg/dL at referral were 15.4 times more likely to die than patients who presented with lower bilirubin levels (P = .001; 95% confidence interval, 2.8-83.4).
  
  
  CONCLUSIONS
  Within a pediatric intestinal failure program, mortality is associated with the degree of hyperbilirubinemia at time of referral. These data strongly suggest that these patients should be referred to a multidisciplinary program early in the evolution of their liver disease.
authors.affiliation: Division of Pediatric General Surgery, Seattle Children's Hospital and University of Washington, Seattle, WA 98105, USA. patrick.javid@seattlechildrens.org
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: Javid
authors.forename: Frances R
authors.initials: FR
authors.lastname: Malone
authors.forename: Rachel
authors.initials: R
authors.lastname: Bittner
authors.forename: Patrick J
authors.initials: PJ
authors.lastname: Healey
authors.forename: Simon P
authors.initials: SP
authors.lastname: Horslen
mesh_terms: D000046:Academic Medical Centers; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D016208:Databases, Factual; D018450:Disease Progression; D005260:Female; D006776:Hospitals, Pediatric; D006801:Humans; D006932:Hyperbilirubinemia / Q000175:diagnosis* / Q000401:mortality* / Q000601:surgery; D007223:Infant; D007231:Infant, Newborn; D007410:Intestinal Diseases / Q000175:diagnosis / Q000401:mortality* / Q000601:surgery*; D053208:Kaplan-Meier Estimate; D008107:Liver Diseases / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery; D016015:Logistic Models; D008297:Male; D009102:Multiple Organ Failure / Q000401:mortality / Q000503:physiopathology / Q000601:surgery; D015999:Multivariate Analysis; D016377:Organ Transplantation / Q000379:methods / Q000401:mortality; D015397:Program Evaluation; D012017:Referral and Consultation / Q000458:organization & administration*; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D016019:Survival Analysis; D015996:Survival Rate; D014481:United States