| 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. |
| Authors | Ahmed Nasr, Yaron Avitzur, Vicky L Ng, Nicole De Silva, Paul W Wales
(Affiliation: Division of General Surgery, Faculty of Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Canada M5G 1X8.)
|
| Journal | Journal of pediatric surgery
(J Pediatr Surg)
Vol. 42
Issue 2
Pg. 359-62
(Feb 2007)
ISSN: 1531-5037 United States |
| PMID | 17270549
(Publication Type: Journal Article)
|
| Chemical References |
- Biological Markers
- Bilirubin
|
| Topics |
- Bilirubin
(blood)
- Biological Markers
(blood)
- Cohort Studies
- Female
- Humans
- Hyperbilirubinemia, Neonatal
(complications, mortality)
- Infant
- Infant, Newborn
- Liver Failure
(etiology, mortality, surgery)
- Liver Transplantation
- Male
- Predictive Value of Tests
- Probability
- Prognosis
- Retrospective Studies
- Sensitivity and Specificity
- Severity of Illness Index
- Short Bowel Syndrome
(complications, diagnosis, mortality)
- Survival Rate
|
