Status:

COMPLETED

Phase III Trial of Coenzyme Q10 in Mitochondrial Disease

Lead Sponsor:

University of Florida

Collaborating Sponsors:

FDA Office of Orphan Products Development

Food and Drug Administration (FDA)

Conditions:

Mitochondrial Diseases

Eligibility:

All Genders

12-17 years

Phase:

PHASE3

Brief Summary

To show that oral CoQ10 is a safe and effective treatment for children with inborn errors of mitochondrial energy metabolism due to defects in specific respiratory chain (RC) complexes or mitochondria...

Detailed Description

In many patients mitochondrial disease leads to progressive nerve and muscle damage that may be associated with problems with thinking, talking, remembering, walking or balancing. Sometimes it may als...

Eligibility Criteria

Inclusion

  • Age 12 m - 17 y
  • Biochemical proof of a deficiency of complex I, III or IV of the RC or a molecular genetic proof of a mutation in mtDNA, or an nDNA mutation in a gene known to be associated with dysfunction of the electron transport chain (e.g., SURF1)
  • Willingness to stop all other medication regimens and supplements other than what the Steering and Planning Committee deems medically necessary

Exclusion

  • A genetic mitochondrial disease other than those stipulated under inclusion criteria
  • Intractable epilepsy, defined as grand mal seizures occurring with a frequency \> 4/month, despite treatment with conventional antiepileptic drugs
  • Primary, defined organic acidurias other than lactic acidosis (e.g., propionic aciduria
  • Primary disorders of amino acid metabolism
  • Primary disorders of fatty acid oxidation
  • Secondary lactic acidosis due to impaired oxygenation or circulation (e.g., due to severe cardiomyopathy or congenital heart defects)
  • Severe anemia, defined as a hematocrit \<30%
  • Malabsorption syndromes associated with D-lactic acidosis
  • Renal insufficiency, defined as (1) a requirement for chronic dialysis or (2) serum creatinine ≥ 1.2 mg/dl or creatinine clearance \<60 ml/min
  • Primary hepatic disease unrelated to mitochondrial disease
  • Allergy to CoQ10 or placebo ingredients
  • Pregnancy

Key Trial Info

Start Date :

January 1 2007

Trial Type :

INTERVENTIONAL

Allocation :

ACTUAL

End Date :

May 1 2013

Estimated Enrollment :

24 Patients enrolled

Trial Details

Trial ID

NCT00432744

Start Date

January 1 2007

End Date

May 1 2013

Last Update

September 11 2017

Active Locations (3)

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Page 1 of 1 (3 locations)

1

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States, 45267

2

Case Western Reserve University

Cleveland, Ohio, United States, 44106

3

Hospital for Sick Children

Toronto, Ontario, Canada, M5G 1X8