Focal ischemic necrosis in advanced biliary atresia cirrhosis.

Details

Serval ID
serval:BIB_98DF6BF46DFB
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Title
Focal ischemic necrosis in advanced biliary atresia cirrhosis.
Journal
Pediatric Transplantation
Author(s)
Wildhaber B.E., Rubbia-Brandt L., Majno P., Mentha G., Schäppi M.G., Anooshiravani M., Belli D., Chardot C.
ISSN
1399-3046 (Electronic)
ISSN-L
1397-3142
Publication state
Published
Issued date
2008
Volume
12
Number
4
Pages
487-491
Language
english
Notes
Publication types: Case Reports ; Journal Article Publication Status: ppublish
Abstract
This report correlates the clinical and biological findings, liver hemodynamics and histological features of focal INL in an infant with BA cirrhosis. An eight month old boy with BA, with previous successful porto-enterostomy, was admitted with signs of cholangitis and ascites. He was treated with antibiotics and diuretics with subsequent clinical improvement. Eight days later, while being fed with hyper-osmolar milk, he became febrile again: ASAT/ALAT climbed (9000/2300 IU/L), liver function deteriorated. Infectious work-up was negative. Liver-ultrasound showed reversed portal flow and a negative arterial diastolic flow. The patient recovered within five days under supportive treatment. A similar event recurred five days later. INL was suspected and semi-urgent living-related liver transplantation was performed, with uneventful post-operative course. Histology of the explanted liver showed extensive foci of INL of different ages. This report illustrates how the association of reversed portal and arterial diastolic flows, with subsequent liver hypoperfusion, may repeatedly cause foci of INL in BA cirrhosis, and lead to rapid progression to liver failure. Because of precarious hepatic blood supply in such patients, close monitoring of portal and diastolic arterial flows is recommended.
Keywords
Biliary Atresia/pathology, Graft Survival, Humans, Infant, Ischemia/pathology, Liver/metabolism, Liver/pathology, Liver Cirrhosis/pathology, Liver Failure/therapy, Liver Transplantation/methods, Male, Necrosis/diagnosis, Necrosis/pathology, Treatment Outcome, Ultrasonography, Doppler/methods
Pubmed
Web of science
Create date
21/02/2015 13:07
Last modification date
20/08/2019 15:00
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