Primary biliary cirrhosis （PBC） is a disease of unknown etiology leading to progressive destruction of small intrahepatic bile ducts and eventually to liver cirrhosis and failure. It is characterised by female predo...Primary biliary cirrhosis （PBC） is a disease of unknown etiology leading to progressive destruction of small intrahepatic bile ducts and eventually to liver cirrhosis and failure. It is characterised by female predominance and serum auto-antibodies to mitochondrial antigens targeting the E2 components of the 2-oxoacid dehydrogenase complex. Although they are associated with disease pathogenesis, no concrete evidence has been presented so far. Epidemiological data indicate that a geographical clustering of cases and possible environmental factors are implicated in pathogenesis. A number of genetic factors play a role in determining disease susceptibility or progression, although no definitive conclusion has been reached so far. A key factor to immune pathogenesis is considered to be the breakdown of immune tolerance, either through molecular mimicry or through the so called determinant density model. In this review, the available data regarding the pathogenesis of primary biliary cirrhosis are described and discussed. A new unifying hypothesis based on early endothelin overproduction in primary biliary cirrhosis （PBC） is presented and discussed.展开更多
OBJECTIVE The concept of double primary lung cancer （DPLC） has been generally accepted. Recently, an increasing incidence of synchronous DPLC has been reported, while the diagnostic standard and treatment strategies...OBJECTIVE The concept of double primary lung cancer （DPLC） has been generally accepted. Recently, an increasing incidence of synchronous DPLC has been reported, while the diagnostic standard and treatment strategies remain to be improved. This study was conducted to investigate effective surgical treatment and prognosis of synchronous DPLC. METHODS From January 1983 to April 2004, 31 patients with synchronous DPLC were operated in our department. Clinical data, such as surgical pattern, postoperative complications, and survival status, of all these patients were reviewed retrospectively. RESULTS The 31 patients with synchronous DPLC accounted for 0.67% of all the 4,649 patients operated for primary lung cancer in our department during the same period. Both tumors of the synchronous DPLC were resected with Iobectomy or pneumonectomy in 12 patients, while among the other 19 patients at least 1 tumor was treated with partial pulmonary resection. The postoperative morbidity was 29%（9/31）, including 1 case of respiratory insufficiency, 3 cases of atelectasis, 2 cases of atrial fibrillation, 1 case of haemoptysis, 1 case of pleural effusion, and 1 case of wound fat necrosis. No deaths occurred during the operations or within 30 days postoperatively. The postoperative 1 -, 3-, and 5-year survival rates were 52%, 29%, and 20%, respectively. CONCLUSION The incidence of synchronous DPLC is low. An aggressive and reasonable surgical approach can achieve a satisfactory outcome in patients with synchronous DPLC. The postoperative morbidity is low. Some patients might achieve long-term survival.展开更多
文摘Primary biliary cirrhosis （PBC） is a disease of unknown etiology leading to progressive destruction of small intrahepatic bile ducts and eventually to liver cirrhosis and failure. It is characterised by female predominance and serum auto-antibodies to mitochondrial antigens targeting the E2 components of the 2-oxoacid dehydrogenase complex. Although they are associated with disease pathogenesis, no concrete evidence has been presented so far. Epidemiological data indicate that a geographical clustering of cases and possible environmental factors are implicated in pathogenesis. A number of genetic factors play a role in determining disease susceptibility or progression, although no definitive conclusion has been reached so far. A key factor to immune pathogenesis is considered to be the breakdown of immune tolerance, either through molecular mimicry or through the so called determinant density model. In this review, the available data regarding the pathogenesis of primary biliary cirrhosis are described and discussed. A new unifying hypothesis based on early endothelin overproduction in primary biliary cirrhosis （PBC） is presented and discussed.
文摘OBJECTIVE The concept of double primary lung cancer （DPLC） has been generally accepted. Recently, an increasing incidence of synchronous DPLC has been reported, while the diagnostic standard and treatment strategies remain to be improved. This study was conducted to investigate effective surgical treatment and prognosis of synchronous DPLC. METHODS From January 1983 to April 2004, 31 patients with synchronous DPLC were operated in our department. Clinical data, such as surgical pattern, postoperative complications, and survival status, of all these patients were reviewed retrospectively. RESULTS The 31 patients with synchronous DPLC accounted for 0.67% of all the 4,649 patients operated for primary lung cancer in our department during the same period. Both tumors of the synchronous DPLC were resected with Iobectomy or pneumonectomy in 12 patients, while among the other 19 patients at least 1 tumor was treated with partial pulmonary resection. The postoperative morbidity was 29%（9/31）, including 1 case of respiratory insufficiency, 3 cases of atelectasis, 2 cases of atrial fibrillation, 1 case of haemoptysis, 1 case of pleural effusion, and 1 case of wound fat necrosis. No deaths occurred during the operations or within 30 days postoperatively. The postoperative 1 -, 3-, and 5-year survival rates were 52%, 29%, and 20%, respectively. CONCLUSION The incidence of synchronous DPLC is low. An aggressive and reasonable surgical approach can achieve a satisfactory outcome in patients with synchronous DPLC. The postoperative morbidity is low. Some patients might achieve long-term survival.
