Literature DB >> 14695410

The effect of oral sucrose on exercise tolerance in patients with McArdle's disease.

John Vissing1, Ronald G Haller.   

Abstract

BACKGROUND: Energy metabolism in muscles relies predominantly on the breakdown of glycogen early in exercise. In patients with McArdle's disease, blocked glycogenolysis in muscles results in low exercise tolerance and can lead to muscle injury, particularly in the first minutes of exercise. We hypothesized that ingesting sucrose before exercise would increase the availability of glucose and would therefore improve exercise tolerance in patients with McArdle's disease.
METHODS: In a single-blind, randomized, placebo-controlled crossover study, 12 patients with McArdle's disease drank 660 ml of a beverage that had been sweetened with artificial sweeteners (placebo) or with 75 g of sucrose after an overnight fast. Thirty to 40 minutes later, the patients rode a stationary bicycle at a constant workload for 15 minutes while the heart rate, level of perceived exertion, and venous blood glucose levels were monitored.
RESULTS: Supplemental sucrose increased the mean plasma glucose level by more than 36 mg per deciliter (2.0 mmol per liter) and resulted in a marked improvement in exercise tolerance in all patients. The mean (+/-SE) heart rate dropped by a maximum of 34+/-3 beats per minute (P<0.001), and the level of perceived exertion fell dramatically when the patients ingested glucose as compared with when they received the placebo.
CONCLUSIONS: This study suggests that the ingestion of sucrose before exercise can markedly improve exercise tolerance in patients with McArdle's disease. The treatment takes effect during the time when muscle injury commonly develops in these patients. In addition to increasing the patients' exercise capacity and sense of well-being, the treatment may protect against exercise-induced rhabdomyolysis. Copyright 2003 Massachusetts Medical Society

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Year:  2003        PMID: 14695410     DOI: 10.1056/NEJMoa031836

Source DB:  PubMed          Journal:  N Engl J Med        ISSN: 0028-4793            Impact factor:   91.245


  39 in total

Review 1.  Pharmacological and nutritional treatment for McArdle disease (Glycogen Storage Disease type V).

Authors:  Rosaline Quinlivan; Andrea Martinuzzi; Benedikt Schoser
Journal:  Cochrane Database Syst Rev       Date:  2014-11-12

Review 2.  A diagnostic algorithm for metabolic myopathies.

Authors:  Andres Berardo; Salvatore DiMauro; Michio Hirano
Journal:  Curr Neurol Neurosci Rep       Date:  2010-03       Impact factor: 5.081

Review 3.  Myopathies Related to Glycogen Metabolism Disorders.

Authors:  Mark A Tarnopolsky
Journal:  Neurotherapeutics       Date:  2018-10       Impact factor: 7.620

4.  Muscle molecular adaptations to endurance exercise training are conditioned by glycogen availability: a proteomics-based analysis in the McArdle mouse model.

Authors:  Carmen Fiuza-Luces; Alejandro Santos-Lozano; Francisco Llavero; Rocío Campo; Gisela Nogales-Gadea; Jorge Díez-Bermejo; Carlos Baladrón; África González-Murillo; Joaquín Arenas; Miguel A Martín; Antoni L Andreu; Tomàs Pinós; Beatriz G Gálvez; Juan A López; Jesús Vázquez; José L Zugaza; Alejandro Lucia
Journal:  J Physiol       Date:  2018-02-14       Impact factor: 5.182

Review 5.  Molecular genetics of McArdle's disease.

Authors:  G Nogales-Gadea; J Arenas; A L Andreu
Journal:  Curr Neurol Neurosci Rep       Date:  2007-01       Impact factor: 5.081

Review 6.  Treatment of glycogenosys type V (McArdle disease) with creatine and ketogenic diet with clinical scores and with 31P-MRS on working leg muscle.

Authors:  M Vorgerd; J Zange
Journal:  Acta Myol       Date:  2007-07

Review 7.  Muscle glycogenoses: an overview.

Authors:  S Di Mauro
Journal:  Acta Myol       Date:  2007-07

Review 8.  Pharmacological and nutritional treatment trials in McArdle disease.

Authors:  R M Quinlivan; R J Beynon
Journal:  Acta Myol       Date:  2007-07

9.  Chronic therapy for McArdle disease: the randomized trial with ACE inhibitor.

Authors:  A Martinuzzi; A Liava; E Trevisi; L Antoniazzi; M Frare
Journal:  Acta Myol       Date:  2007-07

10.  Myophosphorylase deficiency (McArdle disease) in a patient with normal pregnancy and normal pregnancy outcome.

Authors:  Warwick Giles; Catherine Maher
Journal:  Obstet Med       Date:  2011-07-26
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