Treatment of mitochondrial cytopathies

Mitochondrial cytopathies are clinically and biochemically heterogeneous di sorders affecting energy production. Because of the diverse symptoms spanni ng organ systems, the large number of biochemical and genetic defects, and an unpredictable clinical course, there are limited data regarding proven e ffective therapies. In general, treatments for mitochondrial cytopathies ar e intended to augment energy production as well as reduce the production of free radicals and other toxic metabolites that further limit the generatio n of cellular energy. Theoretically, treatment can be aimed at increasing r espiratory chain activity by supplementing relative deficiencies of cofacto rs required for proper functioning. Possible strategies to consider may inc lude dietary management, supplemental vitamins and cofactors, and/or specif ic medications aimed at a particular symptom.