Health Worlds

A, PBC's History
Are the first documented description of PBC may be associated with the same disease, now is the of Addison and Gull, 1851 in Guys Hospital years they yellow one case of skin tumors were reported when patients with the disease [1]. 1875 Hanot recognize cholestasis is the essence of the disease, so after some scholars call it Hanot's xanthomatous cirrhosis or liver cirrhosis, cholestatic liver disease because the yellow tumors on the skin [2]. The name of primary biliary cirrhosis is established in 1949, when the purpose is to boldly and extrahepatic duct obstruction secondary to cirrhosis of the liver caused by the phase difference [3]. 1950 Ahrens et al [4] a detailed description of primary biliary cirrhosis clinical manifestations, was that this is a rare disease, reported worldwide before 1950, no more than 100 cases. Initially named as a cirrhosis of the liver, so the name is often misunderstood, many patients are diagnosed when the PBC is not actually true cirrhosis, patients with this name will cause psychological discomfort. Some scholars try to use various other names, such as chronic non-suppurative granulomatous diseases such as intrahepatic bile duct, the end has not been universally accepted. Therefore, PBC is still used as the name of this disease are still in use.
Second, PBC describes the initial clinical manifestations
1959 Sheila Sherlock describes her personal follow-up from 1944 to 1959 of the 42 cases PBC [5], 20 patients prese


nted with skin itching, or even significant jaundice in 11 years. However, there were 14 cases of jaundice occurred after the itching. There are several cases of liver complaints but no symptoms of a large (possibly the first description of asymptomatic PBC). At the time, no effective way to distinguish intrahepatic and extrahepatic bile duct obstruction, the group of patients no patient had symptoms of fever or abdominal pain. The color change of the stool more, the majority of normal, small white stool. Since there was no ultrasound, so often out to establish the diagnosis of bile duct exploration after scientific examination or liver biopsy determined. 42 patients, only 5 cases have not been confirmed after exploratory surgery.
Sherlock xanthoma cases reported skin changes are very common. 42 cases, 16 cases had xanthoma, xanthoma some flat, others are deposited in the eyelid nodular surface of the inner canthus, can also appear in the wrist, hip, knee and ankle, never occurs in the tendon sheath before. These patients are not all caused due to high cholesterol deposits in the skin caused by cholesterol. Patients by autopsy findings in 15 cases of vascular atherosclerosis in these cases are rare. She reported that the degree of hyperbilirubinemia and the percentage of dietary fat absorption correlation. Spine and ribs X-ray examination showed that some cases of the disease, and spinal vertebrae, rib fractures degradation. Some patients in the supplemented patients after exogenous VitD bone is still thin, so PBC patients are often associated with the presence of osteoporosis.
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What is primary biliary cirrhosis performance and how to diagnose? Medical history and symptoms of the disease often associated with other autoimmune diseases such as rheumatoid joint class, Sjogren's syndrome, scleroderma, chronic lymphocytic thyroiditis and other co-exist, more common in middle-aged women, insidious onset, after a slow early mild symptoms, the patient generally in good condition, no significant loss of appetite and weight declined more than about 10% of patients may have no symptoms. Of unexplained chronic progressive obstructive jaundice patients, especially those associated with steatorrhea, the incentive should learn more about the onset and progression of the situation, whether the existence of other autoimmune diseases, attention and secondary biliary cirrhosis cause jaundice and other liver cirrhosis were identified. Experience found that the skin, sclera jaundice shows multiple scratches and scaling. Liver, splenomegaly surface is still smooth, no tenderness. Auxiliary examination lipids, serum bile acids, conjugated bilirubin, AKP, and GGT enzymes such as micro-duct was significantly increased, transfer of nitrogen enzymes normal or mild to moderate increase. Anti-mitochondrial antibody-positive blood, IgM increased, prothrombin time prolonged. Positive for urinary bilirubin, urine bile per normal or reduced. Imaging ultrasound, ERCP, CT, PTC, etc. to understand whether the extrahepatic bile duct dilatation and obstructive jaundice caused by extrahepatic disease.


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