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The optimal timing of referral to an intestinal failure program: the relationship between hyperbilirubinemia and
Patrick J Javid1, Frances R Malone, Rachel Bittner
1Division of Pediatric General Surgery, Seattle Children's Hospital and University of Washington, Seattle, WA 98105, USA. patrick.javid@seattlechildrens.org
Insights
Early referral to pediatric intestinal failure programs is crucial. High hyperbilirubinemia at referral significantly increases mortality risk, underscoring the need for timely intervention in liver disease management.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Intestinal Failure Management
Background:
- Multidisciplinary treatment improves outcomes for pediatric intestinal failure.
- Optimal referral timing to intestinal failure programs remains unclear.
- Hyperbilirubinemia is a potential indicator of disease severity in these patients.
Purpose of the Study:
- To investigate the association between hyperbilirubinemia at referral and patient outcomes.
- To determine if conjugated bilirubin levels predict mortality in pediatric intestinal failure.
- To inform optimal referral strategies for intestinal failure programs.
Main Methods:
- Retrospective analysis of a prospectively collected database.
- Inclusion of 62 patients referred between 2005 and 2009.
- Multivariable logistic regression adjusted for age at referral to assess mortality risk based on conjugated bilirubin levels.
Main Results:
- Mortality was 50% for patients with conjugated bilirubin ≥ 7.2 mg/dL versus 6% for those with lower levels.
- Patients with higher bilirubin levels were 15.4 times more likely to die.
- 19.4% of patients were listed for combined liver-intestine transplant.
Conclusions:
- Mortality in pediatric intestinal failure programs is linked to the degree of hyperbilirubinemia at referral.
- Elevated conjugated bilirubin at referral is a significant predictor of mortality.
- Early referral to multidisciplinary programs is recommended for pediatric patients with evolving liver disease.
Purpose:
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).
Conclusion:
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.
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