Cerebrotendinous Xanthomatosis Clinical Trial
Official title:
Effects of Diet and Medication in Patients With Cerebrotendinous Xanthomatosis (CTX)
OBJECTIVES: I. Assess the biosynthesis of cholesterol and cholestanol, and measure the
turnover of individual sterols and bile acids in patients with cerebrotendinous
xanthomatosis before and after a cholesterol- and cholestanol-free diet.
II. Assess the biosynthesis of cholesterol and cholestanol, and measure the turnover of
individual sterols and bile acids in these patients before and after lovastatin and
chenodeoxycholic acid.
PROTOCOL OUTLINE: Following a 2-week baseline assessment, patients participate in a feeding
study for up to 3 weeks. The diet is free of cholesterol and cholestanol.
For the next 4 weeks, patients return to their typical diet and are medicated with daily
lovastatin and chenodeoxycholic acid.
The feeding study is repeated for an additional 3 weeks, with the patient taking either
lovastatin or chenodeoxycholic acid.
;
Primary Purpose: Treatment
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