A 33-year-old woman was evaluated for symptoms of severe intense generalized pruritus. Her physical examination reveals multiple sites of excoriation. Laboratory investigations confirm the clinical diagnosis of primary biliary cirrhosis (PBC). She is now inquiring about available treatments for this condition, including if there is a cure for the disease. Which of the following treatments is most likely to “cure” her PBC?
Correct Answer: liver transplantation
Description: In patients with end-stage liver failure, liver transplantation is "curative," since recurrence after transplantation is rare. There is no known effective therapy to prevent progression of liver disease in PBC. The other therapies listed have been reported as effective in small case series but not in controlled trials. Ursodeoxycholic acid seems effective in providing at least symptomatic improvement, and may even delay liver transplantation. Replacement of fat-soluble vitamins (eg, vitamin A to prevent night blindness) is an important part of therapy, as is replacement of calcium and vitamin D to prevent osteoporosis.
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