Addisons' other disease: primary biliary cirrhosis as a model autoimmune disease
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چکیده
منابع مشابه
Addison's other disease: primary biliary cirrhosis as a model autoimmune disease.
Thomas Addison described three of the classical autoimmune diseases, so can justifiably be regarded as one of the fathers of the study of autoimmunity. The least well recognised of these conditions, the autoimmune liver disease primary biliary cirrhosis (PBC), is in some ways the most interesting. As a result of the relatively advanced state of knowledge of its immunopathogenesis, it represents...
متن کاملa patient with primary biliary cirrhosis accompanied by wilson’s disease
conclusions co-occurrence of pbc with wd is rare, which can cause diffusely intrahepatic copper deposition. early liver biopsy and genetic testing are necessary for the diagnosis. the combination of ursodeoxycholic acid with zinc and sodium dimercaptopropane sulfonate is effective. introduction both primary biliary cirrhosis (pbc) and wilson’s disease (wd) can cause copper retention in the live...
متن کاملNOD.c3c4 congenic mice develop autoimmune biliary disease that serologically and pathogenetically models human primary biliary cirrhosis
Primary biliary cirrhosis (PBC) is an autoimmune disease with a strong genetic component characterized by biliary ductular inflammation with eventual liver cirrhosis. The serologic hallmark of PBC is antimitochondrial antibodies that react with the pyruvate dehydrogenase complex, targeting the inner lipoyl domain of the E2 subunit (anti-PDC-E2). Herein we demonstrate that NOD.c3c4 mice congenic...
متن کاملA Patient With Primary Biliary Cirrhosis Accompanied by Wilson’s Disease
INTRODUCTION Both primary biliary cirrhosis (PBC) and Wilson's disease (WD) can cause copper retention in the liver, which is an important factor for liver cellular damage. Copper chelation may preserve liver cell function. It is challenging to distinguish WD from copper accumulation in patients with PBC. There have been few case reports of PBC co-occurrence with WD. CASE PRESENTATION Here we...
متن کاملPrimary biliary cirrhosis and other ductopenic diseases.
Several distinct conditions are characterized by a reduction in the number of small and medium-sized intrahepatic bile ducts. These diseases are associated with progressive cholestasis, which in turn leads to biliary fibrosis and ultimately cirrhosis. The best-characterized ductopenic condition in adulthood is primary biliary cirrhosis (PBC) for which there is now strong evidence of an autoimmu...
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ژورنال
عنوان ژورنال: Clinical Medicine
سال: 2003
ISSN: 1470-2118,1473-4893
DOI: 10.7861/clinmedicine.3-4-351