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| ID | Type | Description | Link |
|---|---|---|---|
| OHSU-4008 |
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| Name | Class |
|---|---|
| Oregon Health and Science University | OTHER |
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.
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| chenodeoxycholic acid | Drug | |||
| lovastatin | Drug |
PROTOCOL ENTRY CRITERIA: Cerebrotendinous xanthomatosis
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| Name | Affiliation | Role |
|---|---|---|
| William Connor | Oregon Health and Science University | Study Chair |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Oregon Health and Science University | Recruiting | Portland | Oregon | 97239-3098 | United States |
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| ID | Term |
|---|---|
| D019294 | Xanthomatosis, Cerebrotendinous |
| D008661 | Metabolism, Inborn Errors |
| D035583 | Rare Diseases |
| ID | Term |
|---|---|
| D008052 | Lipid Metabolism, Inborn Errors |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D052439 | Lipid Metabolism Disorders |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| D014973 | Xanthomatosis |
| D020969 | Disease Attributes |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
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| ID | Term |
|---|---|
| D002635 | Chenodeoxycholic Acid |
| D008148 | Lovastatin |
| ID | Term |
|---|---|
| D003840 | Deoxycholic Acid |
| D002793 | Cholic Acids |
| D001647 | Bile Acids and Salts |
| D013256 | Steroids |
| D000072473 | Fused-Ring Compounds |
| D011083 | Polycyclic Compounds |
| D002757 | Cholanes |
| D009281 | Naphthalenes |
| D011084 | Polycyclic Aromatic Hydrocarbons |
| D006841 | Hydrocarbons, Aromatic |
| D006844 | Hydrocarbons, Cyclic |
| D006838 | Hydrocarbons |
| D009930 | Organic Chemicals |
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