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Friday 18 November 2011

Primary biliary cirrhosis (PBC)

- interlobular bile ducts are damaged by chronic granulomatous inflammation causing progressive cholestasis, cirrhosis and portal hypertension
- 90% are women in age range 40-50 years
- serum AMA (anti-mitochondrial antibodies) subtype M2 in 95%
- associated with autoimmune disorders (Sjogren, scleroderma, thyroid disorder)
- pruritus is earliest symptom, preceding jaundice by a few years
- back pain can be prominent in advanced PBC due to osteomalacia from malabsorption or hepatic osteodystrophy. Osteoporosis is common.
- palmar xanthoma, tuberous xanthomas are seen
- raised ALP, increased serum cholesterol, raised IgM levels
- liver biopsy : granulomas surrounding bile ducts in early disease later progressing to cirrhosis
- treatment: ursodeoxycholic acid and replacement of vitamin A,D,E and K
- median survival overall is 7-10years, reduced to 2 years in presence of jaundice


2 comments:

julia Franklin said...

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MATINA said...


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liver already present. I started on antiviral medications which
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