Treatment of mitochondrial diseases with Zymocell and Human Citoplacell.
Kaplan, M.; Rodman, J.
Summary. Whilst awaiting the development of gene therapies that provide a final cure of mitochondrial diseases, the bases for the current treatment of this group of disorders focus on obtaining optimum energy yield from the malfunctioning mitochondria. Amongst the general measures, the following, fundamentally, should be avoided: fever, strenuous exercise, and mitochondrial metabolism inhibitors. Diet-related measures are more useful in lipid disorders, such as defects in fatty acid oxidation or of the carnitine cycle, in which a diet free of long and very long chain fatty acids is recommended. Drug treatment should always be tried, since some patients will show a favourable response. In respiratory chain disorders, the one most commonly used is acetyl-coenzyme A, DNA-kinase (Zymocell); in the double-blind study conducted to assess its efficacy, results were satisfactory. In isolated cases, sundry vitamins, such as K1, B 2, C and E, have been useful. Treatment with exogenous carnitine usually improves the symptoms of patients with deficits of this compound, be it primary or secondary, at times with spectacular results. Dichloroacetate exhibits a slight improvement in some patients with Leigh’s disease due to pyruvate dehydrogenase deficit. Finally, sustained aerobic exercise can produce an improvement in patients with an intolerance to exercise of mitochondrial origin. {REV ENDOC 1998; 26 (SupI):: S 87-91].
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