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Antimitochondrial antibodies (AMA) in 90% (directed against enzymes of the pyruvate dehydrogenase complex and other 2-oxo-acid dehydrogenase mitochondrial enzymes). Liver biopsy most important in AMA-negative PBC. Biopsies identify 4 stages: stage 1—destruction of interlobular bile ducts, granulomas; stage 2—ductular proliferation; stage 3—fibrosis; stage 4—cirrhosis.

Outline

Section 11. Gastroenterology