The use of conjugated hyperbilirubinemia greater than 100 μmol/L as an indicator of irreversible liver disease in infants with short bowel syndrome

BackgroundThe 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 μmol/L (CB100).ObjectivesThe 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.MethodsAll neonates from our short bowel registry (1997-2003) were reviewed. Conjugated hyperbilirubinemia greater than 100 μmol/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.ResultsSeventy 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 μmol/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).ConclusionA 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.