Plasma carnitine insufficiency and effectiveness ofL-carnitine therapy in patients with mitochondril myopathy

Plasma carnitine “insufficiency,” (plasma esterified carnitine to free carnitine ratio above 0.25) was found in 21 48 (43.8%) patients with mitochondrial myopathy, of whom 4 also showed both total and free carnitine deficiencies in plasma. In addition, plasma levels of SCAC and LCAC were higher in patients with mitochondrial myopathy than in controls ( P < 0.001 and P <0.01, respectively). Patients diagnosed as having plasma carnitine...