All of the following are true about Primary Biliary Cirrhosis except?
Primary biliary cirrhosis- Characterized by chronic inflammation and fibrous obliteration of intrahepatic bile ductules. Associated with autoimmune disorders- CREST syndrome, Sicca syndrome, Autoimmune thyroiditis, Type-1 Diabetes mellitus and IgA deficiency. Mostly seen in females Age is 35-60 yrs C/F-Pruritis, jaundice, steatorrhoea, xanthelasma, hepatomegaly, splenomegaly, ecchymosis, osteomalacia. Associated with cholesterol stones (not pigment stones) due to reduced bile acid synthesis . Lab findings: | Serum 5' nucleotidase activity | Gamma-glutamyl transpeptidase activity | Serum bilirubin levels | Serum lipid levels | ALP Confirmation test- Liver biopsy Treatment of choice is liver transplantation.
Increase 5'- nucleotidase
Median age of presentation is 50 years
Most common cause of cholangitis in children
PBC frequently associated with CREST syndrome
Cirrhosis