Early Coenzyme Q10 Supplementation in Primary Coenzyme Q10 Deficiency

Primary coenzyme Q10 deficiency is considered to be the only treatable mitochondrial disorder, since patients have a response\udto oral coenzyme Q10 supplementation. The disease\udusually manifests with nephropathy and encephalomyopathy.\ud1 It has been shown that oral coenzyme Q10 may stop the progression of encephalopathy, but no benefit from this therapy\udhas been noted with respect to the evolution of renal disease associated with this deficiency.1,2 We now describe the results of long-term coenzyme Q10 supplementation in two patients\udwith coenzyme Q10 deficiency caused by a homozygous\udmissense mutation in the COQ2 gene.3,