GROWTH-HORMONE THERAPY MAY BENEFIT PROTEIN-METABOLISM IN MITOCHONDRIAL ENCEPHALOMYOPATHY

Mitochondrial encephalomyopathy is a genetic disorder for which there is at present no cure, Conventional treatment regimes may not be effec tive in preventing weight loss and muscle wasting in many patients, Re combinant human GH has been shown to have anabolic effects on protein metabolism and to reduce muscle wasting in various diseases. We have t reated a patient known to have myoclonus, epilepsy with ragged red fib res (MERRF) with a high protein diet for 1 month followed by a high pr otein diet and GH therapy for 1 month. To assess the benefit of these treatments the patient underwent whole body protein turnover, myometri c and body composition studies at baseline, following the high protein diet (100 g/day) and following GH therapy, Whole body protein synthes is (and protein breakdown) increased following a high protein intake a nd was further enhanced by treatment with GH and a high protein diet, Body composition did not change significantly following treatment with either the high protein diet or GH but there was an improvement in mu scle performance following GH treatment. Mitochondrial encephalomyopat hy, a wasting disorder, may be a disease in which the known protein an abolic effect of GH may have a therapeutic benefit.