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

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

This study is currently recruiting participants.
Verified by National Center for Research Resources (NCRR), December 2003

Sponsors and Collaborators:	National Center for Research Resources (NCRR) Oregon Health and Science University
Information provided by:	National Center for Research Resources (NCRR)
ClinicalTrials.gov Identifier:	NCT00004346

Purpose

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.

Condition	Intervention	Phase
Cerebrotendinous Xanthomatosis	Drug: chenodeoxycholic acid Drug: lovastatin	Phase II

Genetics Home Reference related topics:

cerebrotendinous xanthomatosis cholesteryl ester storage disease Farber lipogranulomatosis long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency mitochondrial trifunctional protein deficiency primary carnitine deficiency

MedlinePlus related topics:

Cholesterol

ChemIDplus related topics:

Cholest-5-en-3-ol (3beta)- Lovastatin Chenodeoxycholic acid Cholestanol

U.S. FDA Resources

Study Type:	Interventional
Study Design:	Treatment

Official Title:	Effects of Diet and Medication in Patients With Cerebrotendinous Xanthomatosis (CTX)

Further study details as provided by National Center for Research Resources (NCRR):

Estimated Enrollment:	5
Study Start Date:	January 1996

Detailed 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.

Eligibility

Genders Eligible for Study:	Both
Accepts Healthy Volunteers:	No

Criteria

PROTOCOL ENTRY CRITERIA: Cerebrotendinous xanthomatosis

Contacts and Locations

Please refer to this study by its ClinicalTrials.gov identifier: NCT00004346

Locations


United States, Oregon
	Oregon Health and Science University	Recruiting
	Portland, Oregon, United States, 97239-3098
	Contact: Sonja Connor 503-494-7775 connors@ohsu.edu

Sponsors and Collaborators

National Center for Research Resources (NCRR)

Oregon Health and Science University

Investigators


Study Chair:	William Connor	Oregon Health and Science University

More Information

Study ID Numbers:	NCRR-M01RR00334-0067, OHSU-4008
First Received:	October 18, 1999
Last Updated:	June 23, 2005
ClinicalTrials.gov Identifier:	NCT00004346
Health Authority:	United States: Federal Government

Keywords provided by National Center for Research Resources (NCRR):

cerebrotendinous xanthomatosis

inborn errors of metabolism

rare disease

Study placed in the following topic categories:

Lipid Metabolism, Inborn Errors

Xanthomatosis

Metabolism, Inborn Errors

Metabolic Diseases

Genetic Diseases, Inborn

Chenodeoxycholic Acid

Cerebrotendinous xanthomatosis

Rare Diseases

Xanthomatosis, Cerebrotendinous

Metabolic disorder

Lovastatin

Lipid Metabolism Disorders

Additional relevant MeSH terms:

Antimetabolites

Molecular Mechanisms of Pharmacological Action

Antilipemic Agents

Therapeutic Uses

Gastrointestinal Agents

Enzyme Inhibitors

Cathartics

Anticholesteremic Agents

Hydroxymethylglutaryl-CoA Reductase Inhibitors

Pharmacologic Actions

ClinicalTrials.gov processed this record on October 10, 2008