Primary biliary cirrhosis
Primary biliary cirrhosis 1001
Leonard M. Miller School of Medicine
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This 2 page Study Guide was uploaded by Hanyao Foong on Monday January 4, 2016. The Study Guide belongs to 1001 at Leonard M. Miller School of Medicine taught by in Winter2015. Since its upload, it has received 35 views. For similar materials see Medcine in Military Science at Leonard M. Miller School of Medicine.
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Date Created: 01/04/16
Primary biliary cirrhosis Primary biliary cirrhosis is a chronic liver disorder typically seen in middle-aged females (female: male ratio of 9:1). The aetiology is not fully understood although it is thought to be an autoimmune condition. Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis. The classic presentation is itching in a middle-aged woman. Associations with other autoimmune conditions: Sjogren's syndrome (seen in up to 80% of patients) rheumatoid arthritis systemic sclerosis (CREST) thyroid disease celiac disease Diagnosis anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients and are highly specific smooth muscle antibodies in 30% of patients raised serum IgM Management pruritus: cholestyramine fat-soluble vitamin supplementation ursodeoxycholic acid liver transplantation e.g. if bilirubin > 100 (PBC is a major indication) - recurrence in graft can occur but is not usually a problem Primary biliary cirrhosis the M rule IgM antiMitochondrial antibodies, M2 subtype Middle aged females
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