Primary biliary cirrhosis (PBC) is a chronic, cholestatic liver disease that is characterized by progressive immune mediated destruction of the intrahepatic bile ducts. Over time, fibrosis extends out of the portal tracts and progresses to cirrhosis. Neither the etiology nor the pathogenesis are well understood; however, most of the current evidence suggests that it is an autoimmune condition t...

Primary biliary cirrhosis is worldwide in its distribution with a low prevalence of 3.7 14 cases per 100,000 population. The disease is rarely diagnosed in the tropics. The incidence and prevalence is not known but if a thorough search is made for the diagnosis of hepatitis B Surface Antigen negative cirrhosis the diagnosis of this condition would probably increase. The following case report cl...

Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure. Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Annual incidence is estimated between 0.7 and 49 cases per million...

BACKGROUND Several case reports, but only a few studies, have examined the coexistence of coeliac disease and primary biliary cirrhosis. AIM To estimate the risk of primary biliary cirrhosis in two national cohorts of patients with coeliac disease in Denmark and Sweden. METHODS Through record linkage all Danish patients hospitalised with coeliac disease were followed for possible occurrence...

In a prospective survey positive antimitochondrial antibodies have been detected in 69/4200 (1.64%) of all sera submitted to a routine immunology laboratory. Of the 69, only nine patients had uniquivocal primary biliary cirrhosis, six others had chronic active hepatitis, 10 had abnormal liver function tests without evidence of primary biliary cirrhosis, while the remaining 44 had no clinical or...

In a series of 218 patients diagnosed as having primary biliary cirrhosis only nine exhibited a negative serum mitochondrial antibody. On examining additional specimens from these patients, seven were found to be positive, giving a final incidence of greater than 99%. The two patients whose sera remained negative for the mitochondrial antibody had liver histology compatible with the diagnosis o...

OBJECTIVES many patients with primary biliary cirrhosis present for the first time aged over 65, but it is unclear whether the disease is different in older patients. We have examined presentation and mortality in relation to age at which primary biliary cirrhosis was first suspected clinically. DESIGN we identified 1023 patients from our regional primary biliary cirrhosis database with defin...

The intestinal absorption of (3H)cholecalciferol was studied in five patients with alcoholic liver disease, six patients with primary biliary cirrhosis, and 15 healthy subjects. The rate of appearance in plasma of (3H)cholecalciferol after oral ingestion and the subsequent appearance of (3H) polar metabolites in the alcoholic subjects were similar to those in the healthy subjects. In subjects w...

In a retrospective review of 85 patients with primary biliary cirrhosis (PBC), 10 (11.8%) were noted to have extrahepatic malignant neoplasm. In seven female patients the tumour developed within a mean of 3.5 yr after the clinical onset of PBC. This observed number of tumours, 3.5 times more common than the expected age-adjusted incidence, was statistically significant at the 0.5% level.