Primary biliary cirrhosis in a son and his father.

A father and his son with systemic AL amyloidosis.

criminate between driver and passenger mutations, as was also shown recently in the context of FLT3. In the MOLT-4 and RPMI-8402 cell lines, we identified another transcript variant, which lacks exons 7 and 8 (nucleotides 648-1178). This results in a shift in the open reading frame with generation of a premature stop codon in exon 9. Consequently, a truncated form of JAK1 is expressed in these ...

Asymptomatic primary biliary cirrhosis.

Primary biliary cirrhosis.

Primary biliary cirrhosis is a chronic liver disease characterised by intrahepatic bile-duct destruction, cholestasis, and, in some cases, cirrhosis. Evidence supporting the autoimmune nature of this disorder includes the appearance of highly specific antimitochondrial antibodies (AMAs) and autoreactive T cells. Concordance rates in monozygotic twins, familial prevalence, and genetic associatio...

Primary Biliary Cirrhosis

Primary Biliary Cirrhosis (PBC) is the result of long-standing damage to intra-hepatic biliary channels as a result of immune mechanisms leading to cholestasis and its associated complications. It can end up in chronic liver disease or cirrhosis. It usually manifests as pruritis that then proceeds to fatigue, jaundice and other features of chronic liver disease. It is detected by raised serolog...

Primary biliary cirrhosis.

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...