Status:
COMPLETED
Nutritional Therapy of the Deficits of Oxidation Mitochondrial of the Fatty Acids
Lead Sponsor:
Assistance Publique - Hôpitaux de Paris
Conditions:
Inborn Errors of Metabolism
Eligibility:
All Genders
Phase:
NA
Brief Summary
Usual dietary therapies of mitochondrial fatty acid oxidation disorders (FAO) are based on 3 strategies: * limitation of lipid intake in the diet; * supplementation of the diet with medium-chain trig...
Detailed Description
The main specific aim of this study will be to determine the efficiency of odd-chain MCT: TRIHEPTANOIN (Tri-C7) and its metabolites, BETA-HYDROXYPENTANOATE (BHP) and BETA-KETOPENTANOATE (BKP), as pote...
Eligibility Criteria
Inclusion
- Patient with an enzyme deficiency of carnitine palmitoyltransferase 1 (CPT1), carnitine-acylcarnitine translocase (CAT), carnitine palmitoyltransferase 2 (CPT2), very-long chain acyl-CoA dehydrogenase (VLCAD), L-3-hydroxy-acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (MTP).
- Covered by health and social insurance
- Written informed consent
Exclusion
- Patient affected with FAO dysfunction secondary to other cause (e.g. mitochondrial respiratory chain disorders)
- Patient with suspected FAO disorder that has not been proven (by enzymatic or molecular test)
Key Trial Info
Start Date :
June 1 2006
Trial Type :
INTERVENTIONAL
Allocation :
ACTUAL
End Date :
June 1 2010
Estimated Enrollment :
20 Patients enrolled
Trial Details
Trial ID
NCT00328159
Start Date
June 1 2006
End Date
June 1 2010
Last Update
February 17 2011
Active Locations (1)
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1
Necker University Hospital - Metabolism Unit
Paris, France, 75743