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  • Title: The use of conjugated hyperbilirubinemia greater than 100 micromol/L as an indicator of irreversible liver disease in infants with short bowel syndrome.
    Author: Nasr A, Avitzur Y, Ng VL, De Silva N, Wales PW.
    Journal: J Pediatr Surg; 2007 Feb; 42(2):359-62. PubMed ID: 17270549.
    Abstract:
    BACKGROUND: The introduction of parenteral nutrition resulted in improved survival of neonates with short bowel syndrome. It is unclear why some may deteriorate to end-stage liver disease (ESLD). Knowledge of when to refer such children for evaluation for transplantation is crucial. A commonly used criterion is conjugated hyperbilirubinemia greater than 100 micromol/L (CB100). OBJECTIVES: The aim of this study is to evaluate if CB100 is a reliable marker for identifying which infants with short bowel syndrome will subsequently develop ESLD. METHODS: All neonates from our short bowel registry (1997-2003) were reviewed. Conjugated hyperbilirubinemia greater than 100 micromol/L was defined as a sustained CB100 for at least 2 weeks with no concurrent sepsis. The sensitivity, specificity, as well as positive and negative predictive values for predicting an outcome of ESLD were calculated. RESULTS: Seventy short gut infants were identified (25 males; mean gestational age of 32.5 +/- 4.9 weeks and weight of 1902 +/- 888 g). Twenty-three patients (33%) developed CB100. Seventeen patients (24%) developed ESLD. Conjugated hyperbilirubinemia greater than 100 micromol/L had a sensitivity of 94% and a specificity of 87% in determining which patients would advance to ESLD. The positive and negative predictive values were 70% and 98%, respectively. The median time from CB100 to ESLD was 60 days (range, 10-365 days). CONCLUSION: A positive predictive value of 70% ensures a safe level of over-triage to the transplant service for assessment; however, the short duration from CB100 to ESLD (60 days) implies a late detection of advanced liver disease, which raises concern about the use of this test in the clinical setting.
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