Document Detail

Variant forms of cholestatic diseases involving small bile ducts in adults.
MedLine Citation:
PMID:  10811317     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Cholestasis may result from diverse etiologies. We review chronic cholestatic disorders involving small intrahepatic bile ducts in the adult ambulatory care setting. Specifically, we discuss variant forms of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) as well as other conditions that may present diagnostic and therapeutic difficulties. METHODS: We conducted a MEDLINE search of the literature (1981-1997) and reviewed the experiences at the Mayo Clinic. All articles were selected that discussed antimitochondrial antibody (AMA)-negative PBC, small-duct PSC (formerly pericholangitis), and idiopathic adulthood ductopenia. RESULTS: The most common chronic cholestatic liver diseases affecting adults are PBC and PSC. Patients without the hallmarks of either syndrome are diagnosed according to their clinical and histological characteristics. Autoimmune cholangitis is diagnosed if clinical and histological features are compatible with PBC but autoantibodies other than AMA are present. Isolated small duct PSC is diagnosed if patients have inflammatory bowel disease, biopsy features compatible with PSC, but a normal cholangiogram. If ductopenia (absence of interlobular bile ducts in small portal tracts) is found histologically in the absence of PSC, inflammatory bowel disease, and other specific cholestatic syndromes such as drug reaction or sarcoidosis, the most likely diagnosis is idiopathic adulthood ductopenia. CONCLUSIONS: Based on these definitions, an algorithm for diagnosis and therapy in patients with laboratory evidence of chronic cholestasis may be constructed, pending results of further investigations into the etiopathogenesis of these syndromes.
W R Kim; J Ludwig; K D Lindor
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  The American journal of gastroenterology     Volume:  95     ISSN:  0002-9270     ISO Abbreviation:  Am. J. Gastroenterol.     Publication Date:  2000 May 
Date Detail:
Created Date:  2000-05-30     Completed Date:  2000-05-30     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  0421030     Medline TA:  Am J Gastroenterol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1130-8     Citation Subset:  IM    
Department of Laboratory Medicine and Pathology, Mayo Clinic and Foundation, Rochester, Minnesota 55905, USA.
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MeSH Terms
Autoantibodies / analysis
Autoimmune Diseases / diagnosis,  pathology
Cholangitis, Sclerosing / etiology,  pathology
Cholestasis, Intrahepatic* / classification,  diagnosis,  etiology,  immunology
Chronic Disease
Diagnosis, Differential
Liver Cirrhosis, Biliary / etiology,  pathology
Mitochondria / immunology
Reg. No./Substance:
Comment In:
Am J Gastroenterol. 2000 May;95(5):1115-6   [PMID:  10811312 ]

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

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