Primary biliary cirrhosis (PBC) is a chronic, progressive cholestatic liver disease that usually affects middle-aged women and eventually leads to liver failure (Kaplan, 1996).
See also Reynolds syndrome (613471), in which primary biliary cirrhosis is a ... Primary biliary cirrhosis (PBC) is a chronic, progressive cholestatic liver disease that usually affects middle-aged women and eventually leads to liver failure (Kaplan, 1996). See also Reynolds syndrome (613471), in which primary biliary cirrhosis is a feature.
Jaup and Zettergen (1980) studied familial incidence of primary biliary cirrhosis. In the study of patients with PBC and their relatives, Miller et al. (1983) used a method based on the finding that the in vitro addition of ... Jaup and Zettergen (1980) studied familial incidence of primary biliary cirrhosis. In the study of patients with PBC and their relatives, Miller et al. (1983) used a method based on the finding that the in vitro addition of concanavalin A to pokeweed mitogen-stimulated lymphocytes activates suppressor cells, which in turn inhibit immunoglobulin synthesis. Significant impairment of IgG suppression was observed in 13 of 16 patients with PBC and 6 of 23 healthy relatives; all 6 relatives were females. No abnormal suppression was found in unrelated household contacts, patients with other forms of cirrhosis, or healthy controls. They suggested that the finding is not a result of the PBC but a genetic marker of susceptibility to the disorder. Hirakata et al. (1988) described 2 unrelated patients with a combination of the CREST syndrome (181750) and primary biliary cirrhosis. Tsuji et al. (1992) studied 18 healthy first-degree relatives of patients with primary biliary cirrhosis in 2 families. In each of these 2 families, there were 2 persons with PBC: 2 sisters in one family and a brother and sister in the other. Kaplan (1996) reviewed all aspects of primary biliary cirrhosis, including the genetics.