Document Detail


Sclerosing cholangitis with neonatal onset.
MedLine Citation:
PMID:  3612394     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Sclerosing cholangitis is characterized by irregular narrowing of extrahepatic or intrahepatic bile ducts, and in adults is defined mainly by radiologic findings. We describe eight children with cholestasis from the first week of life, followed by early cirrhosis and portal hypertension. Histologic examination of the liver showed absence of interlobular bile ducts in the early cholestatic phase in two patients and biliary cirrhosis later in all patients. Radiologic examination by percutaneous cholecystography under ultrasound guidance, carried out at age 8 months to 9 years, disclosed abnormal intrahepatic bile ducts with rarefaction of segmental branches, stenosis, and focal dilation. The extrahepatic ducts were involved in six patients. No intestinal disease has been found in these patients.
Authors:
O Amedee-Manesme; O Bernard; F Brunelle; M Hadchouel; C Polonovski; J J Baudon; P Beguet; D Alagille
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of pediatrics     Volume:  111     ISSN:  0022-3476     ISO Abbreviation:  J. Pediatr.     Publication Date:  1987 Aug 
Date Detail:
Created Date:  1987-09-10     Completed Date:  1987-09-10     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  225-9     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Bile Ducts / pathology*
Child
Child, Preschool
Cholangiography
Cholangitis / diagnosis*,  metabolism,  pathology
Female
Humans
Infant
Infant, Newborn
Liver / pathology
Liver Cirrhosis / pathology
Male
Sclerosis

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


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