Pharmacological and nutritional treatment trials in McArdle disease.
A systematic review of evidence for randomised controlled trials using pharmacologic and nutritional therapies in McArdle disease was undertaken. Primary outcome measures included any objective assessment of exercise endurance. Secondary outcome measures included changes in metabolic parameters, subjective measures such as quality of life scores and adverse outcomes. Ten randomised controlled trials were identified. Two trials low dose creatine (60 mg/kg/day) and oral sucrose 75 g prior to exercise demonstrated a positive effect.
Quinlivan
R M
RM
The Wolfson Centre for Inherited Neuromuscular Diseases (CIND), Robert Jones and Agnes Hunt, NHS Trust, Oswestry, UK. Rcmq37@aol.com
Beynon
R J
RJ
D003401:Creatine / Q000627:therapeutic use; D004311:Double-Blind Method; D024982:Glycogen Phosphorylase, Muscle Form / Q000235:genetics; D006012:Glycogen Storage Disease Type V / Q000178:diet therapy* / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D009154:Mutation; D016032:Randomized Controlled Trials as Topic; D012266:Ribose / Q000009:adverse effects / Q000627:therapeutic use; D013395:Sucrose / Q000627:therapeutic use; D016896:Treatment Outcome; D025101:Vitamin B 6 / Q000009:adverse effects / Q000627:therapeutic use
D013395:Sucrose; D012266:Ribose; D025101:Vitamin B 6; D024982:Glycogen Phosphorylase, Muscle Form; D003401:Creatine