title: Growth hormone therapy may benefit protein metabolism in mitochondrial encephalomyopathy.
abstract: Mitochondrial encephalomyopathy is a genetic disorder for which there is at present no cure. Conventional treatment regimes may not be effective in preventing weight loss and muscle wasting in many patients. Recombinant human GH has been shown to have anabolic effects on protein metabolism and to reduce muscle wasting in various diseases. We have treated a patient known to have myoclonus, epilepsy with ragged red fibres (MERRF) with a high protein diet for 1 month followed by a high protein diet and GH therapy for 1 month. To assess the benefit of these treatments the patient underwent whole body protein turnover, myometric and body composition studies at baseline, following the high protein diet (100 g/day) and following GH therapy. Whole body protein synthesis (and protein breakdown) increased following a high protein intake and was further enhanced by treatment with GH and in 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 muscle performance following GH treatment. Mitochondrial encephalomyopathy, a wasting disorder, may be a disease in which the known protein anabolic effect of GH may have a therapeutic benefit.
authors.affiliation: Division of Medicine, St. Thomas' Hospital, London. p.carroll@umds.ac.uk
authors.forename: P V
authors.initials: PV
authors.lastname: Carroll
authors.forename: A M
authors.initials: AM
authors.lastname: Umpleby
authors.forename: E
authors.initials: E
authors.lastname: Albany
authors.forename: N C
authors.initials: NC
authors.lastname: Jackson
authors.forename: J A
authors.initials: JA
authors.lastname: Morgan-Hughes
authors.forename: P H
authors.initials: PH
authors.lastname: Sonksen
authors.forename: D L
authors.initials: DL
authors.lastname: Russell-Jones
mesh_terms: D001823:Body Composition / Q000187:drug effects; D003131:Combined Modality Therapy; D004044:Dietary Proteins / Q000008:administration & dosage*; D013006:Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D007334:Insulin-Like Growth Factor I / Q000032:analysis; D008297:Male; D008875:Middle Aged; D017237:Mitochondrial Encephalomyopathies / Q000188:drug therapy* / Q000378:metabolism; D018482:Muscle, Skeletal / Q000503:physiopathology; D011506:Proteins / Q000378:metabolism*
chemical_list: D004044:Dietary Proteins; D011506:Proteins; D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone