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



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

For Condition: Smith-Lemli-Opitz Syndrome
Status: Recruiting
Sponsor(s): National Center for Research Resources (NCRR) , Oregon Health and Science University
Synopsis: 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.
Details: 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.
Eligibility:
Study Type:
  Interventional, Treatment
Minimum Age/Maximum Age: /
Genders: Both
Protocol Entry Criteria: PROTOCOL ENTRY CRITERIA: Smith-Lemli-Opitz syndrome
Total Enrollment: 5

Location and Contact Information:

Overall Study Official:
WilliamConnor,  Study Chair,  Oregon Health and Science University

Oregon Health Sciences University *Recruiting*
Portland,  Oregon,  97201-3098
United States
Recruiting Sonja  Connor 503-494-7775


Additional Information:
Study ID Numbers:
  NCRR-M01RR00334-0068;  OHSU-4019
Study Start Date: November 1995
Record last reviewed: December 2003
Additional information available at: clinicaltrials.gov
Clinicaltrials.gov Reference link: NCT00004347

Other Smith-Lemli-Opitz Syndrome Studies:
1. Prenatal Screening For Smith-Lemli-Opitz Syndrome

2. Phase II Study of Dietary Cholesterol for Smith-Lemli-Opitz Syndrome

3. Estimation of the Carrier Frequency and Incidence of Smith-Lemli-Opitz Syndrome in African Americans

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

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