Genetics of Glycogen-Storage Disease Type V Treatment & Management

Updated: Nov 10, 2015
  • Author: Edward J Cupler, MD, FAAN; Chief Editor: Maria Descartes, MD  more...
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Treatment

Medical Care

Therapies in patients with McArdle disease (glycogen-storage disease type V) are used in an attempt to bypass the metabolic block by providing glucose or fructose yield inconsistent results. Similarly, injection of glucagons yields inconsistent results.

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Consultations

Acute renal failure may occur with rhabdomyolysis, necessitating consultation with a nephrologist.

Monitor renal function in all patients with McArdle disease. This may be performed in conjunction with a nephrologist.

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Diet

A high carbohydrate diet may improve maximal work capacity and exercise tolerance. A randomized controlled trial comparing a carbohydrate rich (20% fat, 15% protein, 65% carbohydrate) and a protein rich diet (15% fat, 55% protein, 30% carbohydrate) surprisingly showed that subjects on the carbohydrate rich diet improved exercise tolerance and maximum oxidative capacity compared with subjects on the protein rich diet. [11]

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Activity

Regular, moderate aerobic activity has been shown to improve exercise capacity in patients with McArdle disease. Patients should exercise using a heart rate monitor, keeping the heart rate to 60-70% of maximum. [12] Exercise should be preceded by a warm-up period and dose of sucrose prior to exertion if not contraindicated. [13]

Strenuous isometric exercises may cause symptoms or rhabdomyolysis.

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