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Diagnosis and treatment of primary biliary cirrhosis
Anonymous Original Site Updated: September 3, 2008 primary biliary cirrhosis (PBC) is a mainly affects middle-aged women with chronic progressive cholestatic liver disease characterized by serum anti-mitochondrial antibody positive, pathological features of non-interlobular bile duct damage and bile duct inflammation suppurative granuloma formation. Interlobular bile duct damage leading to chronic progressive cholestasis, fibrosis, cirrhosis and eventually liver failure.
1. Clinical manifestations
Primary biliary cirrhosis (PBC) of the typical symptoms of fatigue and itching. Scratching lesions, jaundice and increased liver and spleen and other signs though it is specific but were more common. Jaundice usually appears late in the disease process, but can also be found in 20% to 40% of patients in early stages. 90% to 95% of patients are female, age of onset is usually 30 to 65 years. Other symptoms are abdominal pain, anorexia and so on. Itching in the disease process can occur at any time, with the progress of the disease may occasionally be eased. On the contrary, jaundice is usually persistent, but also the most important indicator of prognosis. Primary biliary cirrhosis (PBC) other clinical manifestations include skin pigmentation, macular tumor. Can occur in patients with advanced ascites, esophageal variceal bleeding, hepatic encephalopathy and other complications of cirrhosis of the performance. Because


the intestine common bile salt deficiency leads to steatorrhea. Patients with severe osteoporosis fractures may occur spontaneously.
2. Laboratory
Anti-mitochondrial antibody (AMA) is the primary biliary cirrhosis-positive (PBC) serological characteristics, can be found in 9O% ~ 95% of patients, and in the clinical symptoms and abnormal liver function for several years before the onset of emergence. 2 AMA focused on a mitochondrial oxygen acid dehydrogenase complex (2-OADC) of the acyl domain. Serum biochemical examination revealed changes of cholestasis, serum alkaline phosphatase (ALP) with Bu glutamyl GGT (r-GT) increased (3-5 times) is the primary biliary cirrhosis (PBC) the most common biochemical abnormalities. Aspartate aminotransferase (AST) and alanine aminotransferase (ALT) was elevated significantly elevated rare. Serum total cholesterol and IgM levels. Serum bilirubin levels increased with the slow progress of the disease can be more than 20mg/dl, direct bilirubin and indirect bilirubin were increased. Hyperbilirubinemia, hypoalbuminemia and prolonged prothrombin time were indicators of poor prognosis.
Histologically, interlobular / interval destructive granulomatous inflammation of bile duct with lymphocyte infiltration in this lesion characteristics, showing inflammation of portal area, around the damaged bile duct cells and basement membrane and invasion into the bile duct bile duct epithelial cells, and bile duct epithelial cells vacuolar degeneration. Damaged bile duct surrounded by epithelioid cells and multinucleated giant cells of the granuloma, granuloma is characterized by numerous organizations within the cytoplasm of foam cells, so called lipid (yellow tumor-like) granulomas. Expansion of the portal area lesions, diffuse lymphocytic overflow into the hepatic lobule, the debris is tantamount to necrosis. The destruction of the cell basement membrane of bile duct proliferation after bile duct and the formation of granuloma with necrosis of the debris fragments called biliary necrosis, unlike lymphocytes in autoimmune hepatitis, necrosis of debris.
3. Recommended diagnostic
Currently primary biliary cirrhosis (PBC) diagnosis based on the following three criteria: AMA-positive serum, liver enzymes (ALP increased to the most common) increased more than 6 months and the corresponding histological findings. Who meet the above two as a possible diagnosis, and diagnostic uncertainty existed simultaneously required. Some scholars believe that evidence of liver histology is not required, but the liver biopsy examination can provide staging information for evaluation of drug efficacy. 54th AASLD (AASLD) on primary biliary cirrhosis (PBC) diagnosis of recommendation include: unexplained elevated serum ALP (normal ultrasound examination of bile duct), required serum AMA; AMA high titer ( 1:40) in particular, AMA-M2 and liver biochemical tests positive for no other interpretation of cholestasis can be identified with primary biliary cirrhosis (PBC) diagnosis; AMA positive (
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