Phase II Study of Cholesterol- and Cholestanol-Free Diet, Lovastatin, and Chenodeoxycholic Acid for Cerebrotendinous Xanthomatosis

National Center for Research Resources (NCRR)

Status and phase: Unknown; Phase 2

Conditions: Cerebrotendinous Xanthomatosis

Treatments: Drug: lovastatin; Drug: chenodeoxycholic acid

Study type: Interventional

Funder types: Other; NIH

Identifiers

NCT00004346

NCRR-M01RR00334-0067

OHSU-4008

Details and patient eligibility

About

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.

Full description

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.

Sex: All

Volunteers: No Healthy Volunteers

Inclusion and exclusion criteria

PROTOCOL ENTRY CRITERIA: Cerebrotendinous xanthomatosis