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肌腺苷酸脱氨酶缺乏症和麦卡德尔病的缺血运动试验:血浆腺苷、肌苷和次黄嘌呤的测定

Ischaemic exercise test in myoadenylate deaminase deficiency and McArdle's disease: measurement of plasma adenosine, inosine and hypoxanthine.

作者信息

Sinkeler S P, Joosten E M, Wevers R A, Binkhorst R A, Oerlemans F T, van Bennekom C A, Coerwinkel M M, Oei T L

出版信息

Clin Sci (Lond). 1986 Apr;70(4):399-401. doi: 10.1042/cs0700399.

Abstract

Plasma adenosine, inosine and hypoxanthine concentrations were assayed in seven control subjects, five myoadenylate deaminase deficient (MADD) patients and six McArdle patients before and after ischaemic forearm exercise. The plasma adenosine increase was very low in all test groups and there were no significant differences. The MADD patients showed a significantly lower increase of plasma inosine and hypoxanthine after exercise as compared with the controls. In the McArdle patients the increase in plasma inosine and hypoxanthine after exercise did not differ significantly from the values measured in the controls. The ischaemic exercise test with measurement of plasma inosine and hypoxanthine might be of diagnostic value in MADD, but not in McArdle's disease.

摘要

在七名对照受试者、五名肌腺苷酸脱氨酶缺乏症(MADD)患者和六名麦卡德尔病患者进行缺血性前臂运动前后,测定了血浆腺苷、肌苷和次黄嘌呤浓度。所有测试组的血浆腺苷增加量都非常低,且无显著差异。与对照组相比,MADD患者运动后血浆肌苷和次黄嘌呤的增加量显著更低。在麦卡德尔病患者中,运动后血浆肌苷和次黄嘌呤的增加量与对照组测得的值无显著差异。通过测量血浆肌苷和次黄嘌呤进行的缺血性运动试验可能对MADD具有诊断价值,但对麦卡德尔病则不然。

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