Sclerosing Cholangitis and Primary Biliary Cirrh原发性硬化性胆管炎原发性胆汁性肝硬变ppt课件.pptVIP

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Sclerosing Cholangitis and Primary Biliary Cirrh原发性硬化性胆管炎原发性胆汁性肝硬变ppt课件.ppt

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Sclerosing Cholangitis and Primary Biliary Cirrh原发性硬化性胆管炎原发性胆汁性肝硬变ppt课件

临床医学于波心房选择性钠通道阻滞剂在房颤中的应用颅内非肿瘤性病变的影像学表现基础医学慢性鼻窦炎鼻息肉围手术期综合治疗的临床指引泌尿生殖道感染衣原体检测和支原体培养及药敏结果分析 Primary Sclerosing Cholangitis and Primary Biliary Cirrhosis Registrar teaching July 2007 Paul Frankish Primary Biliary Cirrhosis PBC-introduction Slowly progressive autoimmune liver disease 90% females Peak incidence in 40’s Portal inflammation and autoimmune destruction of intrahepatic bile ducts Leads to cirrhosis and liver failure 90-95% have antimitochondrial antibody Clinical features ~50% asymptomatic at diagnosis Fatigue and pruritus most commonn symptoms~20% Hyperlipidaemia,hypothyroidism,osteopenia,autoimmune diseases Portal hypertension ,liver failure,HCC Physical examination Often normal Spiders and skin excoriations Xanthelasmas Hepatomegaly ~70% Jaundice (late) Diagnosis 3 criteria Positive AMA Abnormal LFT Compatible biopsy Pathological Stages (4) 1 Destruction of bile ducts in portal tracts 2 Inflammation beyond portal tracts 3 fibrous septa link portal triads Cirrhosis Epidemiology and Genetic factors Most prevalent in Nth Europe.10 fold variation More common in first degree relatives Molecular mimicry to certain bacteria or viruses Environmental chemical exposure Autoimmune responses Targets of antimitichondrial antibodies 4 autoreactive mitochondrial antigens Pyruvate dehydrogenase E2 complex PDC-E2 E-3 binding protein E3-BP Ketoglutaric acid dehydrogenase E2 complex OGDC-E2 2 oxo-aciddehydrogenaseE-2 complex BCKD-E2 T cell response T cells infiltrating the liver are specific for PDC-E2 Nature of bile duct injury not fully elucidated Treatment:-Ursodeoxycholic acid UDCA Given in dose 12-15 mg/kg Reduces bilirubin,ALP,AST,ALT cholesterol and IgM Meta-analysis of 3 trials 548 patients UDCA reduced risk of liver transplantation or death over 4 years Delays fibrosis and varices Does not work in advanced disease Other drugs Colchicine Methotrexate Budesoide Liver transplantation Only effective Rx for liver failure Survival is excellent 85% at 5 years CAN R