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Paper Information

Journal:   IRANIAN JOURNAL OF CHILD NEUROLOGY (IJCN)   2013 , Volume 7 , Number 4 (SUPPL.1); Page(s) 16 To 17.
 
Paper: 

TREATMENT OF MITOCHONDRIAL CYTOPATHIES

 
 
Author(s):  SHAKIBA MARJAN*
 
* MOFID CHILDREN HOSPITAL, SHARIATI AVE, TEHRAN, IRAN
 
Abstract: 

Mitochondrial cytopathies are clinically and biochemically heterogeneous disorders affecting energy production. Because of the heterogeneity of disorders, the large number of biochemical and genetic defects, and wide spectrum of clinical course, there are limited data about proven effective therapies. Overall, treatments for mitochondrial cytopathies are intended to augment energy production and reduce the production of free radicals and other toxic metabolites that further limit the generation of cellular energy. Treatment can be aimed at increasing respiratory chain activity by supplementing relative deficiencies of cofactors required for proper functioning. Possible strategies to consider may include dietary management, supplemental vitamins and cofactors, and specific medications aimed at a particular symptom.
The clinical benefits for cofactor and vitamin therapy can include improved strength and endurance, although patients report a variety of benefits. Coenzyme Q10 can be given at dosages ranging from 5 to15 mg/kg/d in two to three divided doses. Levocarnitine is advocated at dosages of 30 mg/kg/d in two to three divided doses. Riboflavin (vitamin B2) at dosages of 100 to 400 mg/d has been shown to be beneficial in some patients. The use of creatine has been shown to improve strength in patients with mitochondrial myopathies, although its long-term use should be consider with caution Because of the potential for renal toxicity. The use of antioxidants. (<-lipoic acid, vitamin E, vitamin C, ®-carotene, selenium, vitamin K and N-acetylcysteine) to lessen free radical damage to the mitochondrial membrane has a scientific rationale, but again proof of effectiveness does not exist.

 
Keyword(s): CHILDREN, MITOCHONDRIAL CYTOPATHIES, TREATMENT
 
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