Primary biliary cirrhosis (PBC) is a progressive cho- lestatic liver disease characterized by the immune- mediated destruction of biliary epithelial cells in small intrahepatic bile ducts. The disease is characteriz...Primary biliary cirrhosis (PBC) is a progressive cho- lestatic liver disease characterized by the immune- mediated destruction of biliary epithelial cells in small intrahepatic bile ducts. The disease is characterized by circulating antimitochondrial antibodies (AMAs) as well as disease-specific antinuclear antibodies, cholestatic liver function tests, and characteristic histological fea- tures, including granulomas. A variety of organisms are involved in granuloma formation, of which mycobacte- ria are the most commonly associated. This has led to the hypothesis that mnycobacteria may be involved in the pathogenesis of PBC, along with other infectious agents. Additionally, AMAs are found in a subgroup of patients with rnycobacterial infections, such as lep- rosy and pulmonary tuberculosis. Antibodies against species-specific mycobacterial proteins have been re- ported in patients with PBC, but it is not clear whether these antibodies are specific for the disease. In addi- tion, data in support of the involvement of the role of molecular mimicry between rnycobacterial and human mitochondrial antigens as triggers of cross-reactive im- mune responses leading to the loss of immunological tolerance, and the induction of pathological features have been published. Thus, antibodies against myco- bacterial heat shock protein appear to cross-recognize AMA-specific autoantigens, but it is not clear whether these autoantibodies are mycobacterium-species-spe- cific, and whether they are pathogenic or incidental. The view that mycobacteria are infectious triggers of PBC is intriguing, but the data provided so far are not conclusive.展开更多
Acute acalculous cholecystitis(AAC) is the inflammatory disease of the gallbladder in the absence of gallstones. AAC is estimated to represent at least 50% to 70% of all cases of acute cholecystitis during childhood. ...Acute acalculous cholecystitis(AAC) is the inflammatory disease of the gallbladder in the absence of gallstones. AAC is estimated to represent at least 50% to 70% of all cases of acute cholecystitis during childhood. Although this pathology was originally described in critically ill or post-surgical patients, most pediatric cases have been observed during several infectious diseases. In addition to cases caused by bacterial and parasitic infections, most pediatric reports after 2000 described children developing AAC during viral illnesses(such as Epstein-Barr virus and hepatitis A virus infections). Moreover, some pediatric cases have been associated with several underlying chronic diseases and, in particular, with immune-mediated disorders. Here, we review the epidemiological aspects of pediatric AAC, and we discuss etiology, pathophysiology and clinical management, according to the cases reported in the medical literature.展开更多
AIM:To investigate the feasibility and the effectiveness of ileoileostomy in the region adjacent to the ileocecal valve,which can retain the ileocecal valve in infants.METHODS:This is a retrospective review of 48 pati...AIM:To investigate the feasibility and the effectiveness of ileoileostomy in the region adjacent to the ileocecal valve,which can retain the ileocecal valve in infants.METHODS:This is a retrospective review of 48 patients who underwent ileoileostomy in the region adjacent to the ileocecal valve(group 1) and 34 patients who underwent ileocecal resections and ileotransversanastomosis(group 2).Patients were monitored for the time to flatus,resumption of eating,length of hospital stay after surgery,serum total bile acid,vitamin B12 and postoperative complications.RESULTS:The time to flatus,time until resumption of eating and post-operative length of hospital stay showed no statistically significant differences between the two groups.Serum total bile acid and vitamin B12were not significantly different between the two groups at post-operative day 1 and day 3,but were significantly decreased at 1 wk after operation in group 2.None of the patients died or suffered from stomal leak in these two groups.However,the incidence of diarrhea,intestinal infection,disturbance of acid-base balance and water-electrolytes in group 1 was lower than in group 2.CONCLUSION:Ileoileostomy in the region adjacent to the ileocecal valve is safe and results in fewer complications than ileotransversanastomosis in infants.展开更多
Fasciola hepatica,an endemic parasite in Turkey,is still a very rare cause of cholestasis worldwide.Through ingestion of contaminated water plants like watercress,humans can become the definitive host of this parasite...Fasciola hepatica,an endemic parasite in Turkey,is still a very rare cause of cholestasis worldwide.Through ingestion of contaminated water plants like watercress,humans can become the definitive host of this parasite.Cholestatic symptoms may be sudden but in some cases they may be preceeded by a long period of fever,eosinophilia and vague gastrointestinal symptoms.We report a woman with cholangitis symptoms of sudden onset which was proved to be due to Fasciola hepatica infestation by an endoscopic retrograde cholangiography.展开更多
文摘Primary biliary cirrhosis (PBC) is a progressive cho- lestatic liver disease characterized by the immune- mediated destruction of biliary epithelial cells in small intrahepatic bile ducts. The disease is characterized by circulating antimitochondrial antibodies (AMAs) as well as disease-specific antinuclear antibodies, cholestatic liver function tests, and characteristic histological fea- tures, including granulomas. A variety of organisms are involved in granuloma formation, of which mycobacte- ria are the most commonly associated. This has led to the hypothesis that mnycobacteria may be involved in the pathogenesis of PBC, along with other infectious agents. Additionally, AMAs are found in a subgroup of patients with rnycobacterial infections, such as lep- rosy and pulmonary tuberculosis. Antibodies against species-specific mycobacterial proteins have been re- ported in patients with PBC, but it is not clear whether these antibodies are specific for the disease. In addi- tion, data in support of the involvement of the role of molecular mimicry between rnycobacterial and human mitochondrial antigens as triggers of cross-reactive im- mune responses leading to the loss of immunological tolerance, and the induction of pathological features have been published. Thus, antibodies against myco- bacterial heat shock protein appear to cross-recognize AMA-specific autoantigens, but it is not clear whether these autoantibodies are mycobacterium-species-spe- cific, and whether they are pathogenic or incidental. The view that mycobacteria are infectious triggers of PBC is intriguing, but the data provided so far are not conclusive.
文摘Acute acalculous cholecystitis(AAC) is the inflammatory disease of the gallbladder in the absence of gallstones. AAC is estimated to represent at least 50% to 70% of all cases of acute cholecystitis during childhood. Although this pathology was originally described in critically ill or post-surgical patients, most pediatric cases have been observed during several infectious diseases. In addition to cases caused by bacterial and parasitic infections, most pediatric reports after 2000 described children developing AAC during viral illnesses(such as Epstein-Barr virus and hepatitis A virus infections). Moreover, some pediatric cases have been associated with several underlying chronic diseases and, in particular, with immune-mediated disorders. Here, we review the epidemiological aspects of pediatric AAC, and we discuss etiology, pathophysiology and clinical management, according to the cases reported in the medical literature.
基金Supported by National Natural Science Foundation of China,No. 81100318a grant from Nanjing Medical University,No. 2010NJMU118
文摘AIM:To investigate the feasibility and the effectiveness of ileoileostomy in the region adjacent to the ileocecal valve,which can retain the ileocecal valve in infants.METHODS:This is a retrospective review of 48 patients who underwent ileoileostomy in the region adjacent to the ileocecal valve(group 1) and 34 patients who underwent ileocecal resections and ileotransversanastomosis(group 2).Patients were monitored for the time to flatus,resumption of eating,length of hospital stay after surgery,serum total bile acid,vitamin B12 and postoperative complications.RESULTS:The time to flatus,time until resumption of eating and post-operative length of hospital stay showed no statistically significant differences between the two groups.Serum total bile acid and vitamin B12were not significantly different between the two groups at post-operative day 1 and day 3,but were significantly decreased at 1 wk after operation in group 2.None of the patients died or suffered from stomal leak in these two groups.However,the incidence of diarrhea,intestinal infection,disturbance of acid-base balance and water-electrolytes in group 1 was lower than in group 2.CONCLUSION:Ileoileostomy in the region adjacent to the ileocecal valve is safe and results in fewer complications than ileotransversanastomosis in infants.
文摘Fasciola hepatica,an endemic parasite in Turkey,is still a very rare cause of cholestasis worldwide.Through ingestion of contaminated water plants like watercress,humans can become the definitive host of this parasite.Cholestatic symptoms may be sudden but in some cases they may be preceeded by a long period of fever,eosinophilia and vague gastrointestinal symptoms.We report a woman with cholangitis symptoms of sudden onset which was proved to be due to Fasciola hepatica infestation by an endoscopic retrograde cholangiography.