Document Detail

Congenital biliary atresia: liver injury begins at birth.
MedLine Citation:
PMID:  19302872     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: The timing of onset of liver injury in biliary atresia (BA) is not known, although in approximately 10% of cases, biliary pathologic condition associated with the biliary atresia splenic malformation syndrome must begin well before birth. METHODS: The study involved retrospective case-note review for infants with definite BA who underwent laparotomy within first week of life. RESULTS: Three infants were identified who had occlusive BA evident on the first day of life. In all cases, their liver was grossly normal, and histologic changes were trivial. CONCLUSION: This suggests that the detrimental cholestatic liver injury, later characteristic of BA, only begins from the time of birth despite a prenatal occlusive biliary pathology. It may be that tissue injury only occurs with the onset of the perinatal bile surge initiating periductal bile leakage and the triggering of an inflammatory and ultimately fibrotic response.
Erica Makin; Alberto Quaglia; Nina Kvist; Bodil Laub Petersen; Bernard Portmann; Mark Davenport
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  44     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2009 Mar 
Date Detail:
Created Date:  2009-03-23     Completed Date:  2009-07-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  630-3     Citation Subset:  IM    
Department of Paediatric Surgery, King's College Hospital, London.
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MeSH Terms
Biliary Atresia / blood,  pathology*,  physiopathology,  surgery
Bilirubin / blood
Fatal Outcome
Liver / metabolism,  pathology*
Retrospective Studies
Reg. No./Substance:

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