Clinical Trial: Phase II Study of Dietary Cholesterol for Smith-Lemli-Opitz Syndrome

Study Status: Completed
Recruit Status: Unknown status
Study Type: Interventional

Official Title: The Effects of Dietary Cholesterol in the Smith-Lemli-Opitz Syndrome

Brief Summary:

OBJECTIVES: I. Examine the intestinal absorption of dietary cholesterol in patients with Smith-Lemli-Opitz syndrome.

II. Measure the effect of dietary cholesterol on plasma sterol composition. III. Quantify basal cholesterol synthesis, turnover of cholesterol and 7-dehydrocholesterol, and the effects of dietary cholesterol on these parameters.

IV. Identify fecal bile acid excretion quantitatively and qualitatively in these patients.

V. Compare the incorporation of deuterated water into plasma cholesterol, 7-dehydrocholesterol, and other intermediates, and assess the effect of dietary cholesterol on this incorporation.

Detailed Summary:

PROTOCOL OUTLINE:

Patients are treated with 2 dietary regimens for 1 to 3 weeks: high cholesterol and cholesterol-free. Cholesterol is provided in the form of egg yolk. The absorption and metabolism of cholesterol are evaluated with plasma and fecal studies. Deuterated water is given to patients followed by analysis of deuterium in plasma cholesterol and other intermediates, including 7-dehydrocholesterol.

Sponsor: National Center for Research Resources (NCRR)

Current Primary Outcome:

Original Primary Outcome:

Current Secondary Outcome:

Original Secondary Outcome:

Information By: National Center for Research Resources (NCRR)

Dates:
Date Received: October 18, 1999
Date Started: November 1995
Date Completion:
Last Updated: June 23, 2005
Last Verified: December 2003