Document Detail

The use of conjugated hyperbilirubinemia greater than 100 micromol/L as an indicator of irreversible liver disease in infants with short bowel syndrome.
MedLine Citation:
PMID:  17270549     Owner:  NLM     Status:  MEDLINE    
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.
Ahmed Nasr; Yaron Avitzur; Vicky L Ng; Nicole De Silva; Paul W Wales
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  42     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2007 Feb 
Date Detail:
Created Date:  2007-02-02     Completed Date:  2007-02-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  359-62     Citation Subset:  IM    
Division of General Surgery, Faculty of Medicine, The Hospital for Sick Children, University of Toronto, Toronto, Canada M5G 1X8.
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MeSH Terms
Bilirubin / blood*
Biological Markers / blood
Cohort Studies
Hyperbilirubinemia, Neonatal / complications*,  mortality
Infant, Newborn
Liver Failure / etiology*,  mortality,  surgery
Liver Transplantation
Predictive Value of Tests
Retrospective Studies
Sensitivity and Specificity
Severity of Illness Index
Short Bowel Syndrome / complications*,  diagnosis,  mortality
Survival Rate
Reg. No./Substance:
0/Biological Markers; 635-65-4/Bilirubin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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