Glucose infusion abolishes the excessive ATP degradation in working muscles of a patient with McArdle's disease.
A 23-year-old woman with McArdle's disease performed mild leg exercise on a bicycle ergometer. Saline or 10% glucose solution was infused throughout exercise. After exercise with saline infusion, her plasma concentrations of ammonia, hypoxanthine and creatine kinase increased greatly. Conversely, after exercise with glucose infusion, there were no appreciable changes in these plasma substances. In addition, she noticed that glucose infusion relieved her from muscle symptoms during exercise. These findings suggest that glucose infusion to patients with McArdle's disease ameliorates excessive ATP degradation in exercising muscles.
Mineo
I
I
Second Department of Internal Medicine, Osaka University Medical School, Japan.
Kono
N
N
Yamada
Y
Y
Hara
N
N
Kiyokawa
H
H
Hamaguchi
T
T
Kawachi
M
M
Yamasaki
T
T
Nakajima
H
H
Kuwajima
M
M
D000255:Adenosine Triphosphate / Q000378:metabolism*; D000328:Adult; D000641:Ammonia / Q000097:blood; D003402:Creatine Kinase / Q000097:blood; D005080:Exercise Test; D005260:Female; D005947:Glucose / Q000008:administration & dosage / Q000494:pharmacology*; D006012:Glycogen Storage Disease Type V / Q000378:metabolism*; D006801:Humans; D019271:Hypoxanthine; D007042:Hypoxanthines / Q000097:blood; D007262:Infusions, Intravenous; D009119:Muscle Contraction; D009132:Muscles / Q000187:drug effects* / Q000201:enzymology / Q000378:metabolism; D005082:Physical Exertion
D007042:Hypoxanthines; D019271:Hypoxanthine; D000641:Ammonia; D000255:Adenosine Triphosphate; D003402:Creatine Kinase; D005947:Glucose