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Primary Biliary Cirrhosis      pbc2007.pdf  

Primary biliary cirrhosis (PBC),


Approximately 30% of newly diagnosed patients are asymptomatic, but most develop symptoms within 2 to 4 years. Early symptoms are fatigue and pruritus, whereas jaundice, steatorrhea, osteoporosis, and xanthomata develop later. Muscle wasting and bleeding from portal hypertension are end-stage manifestations and indications for referral for liver transplantation. The most troublesome symptom, itching, usually responds to cholestyramine, 4 g three times daily, initially. Cholestyramine appears to bind the itch-promoting substance in the intestine and to lead to its excretion in feces, a process that presumably decreases its concentration in bodily fluids. Rifampin relieves itching in some patients, but often elevates the serum bilirubin. UVB light, methyltestosterone, opioid antagonists such as naloxone, and plasmapheresis have been successful in refractory pruritus. Blood levels of fat-soluble vitamins should be checked, particularly in jaundiced patients, and supplemented orally if low.

There is no proven, generally accepted treatment for the underlying disorder. No medical treatment has been shown to alter the natural history of PBC.

ACP Library on Disk 2- (c) 1997 - American College of Physicianss