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强直性肌营养不良患者缺血运动后血浆钾离子过度升高。

Excessive plasma K+ increase after ischemic exercise in myotonic muscular dystrophy.

作者信息

Wevers R A, Joosten M G, van de Biezenbos J B, Theewes G M, Veerkamp J H

机构信息

Institute of Neurology, University Hospital Nijmegen, The Netherlands.

出版信息

Muscle Nerve. 1990 Jan;13(1):27-32. doi: 10.1002/mus.880130107.

DOI:10.1002/mus.880130107
PMID:2325699
Abstract

Changes in plasma electrolyte levels upon ischemic forearm exercise were studied in myotonic muscular dystrophy (MyD) patients, disease control groups, and healthy volunteers. Significant differences were observed in the pH and the concentrations of creatine kinase and Na+ before exercise between healthy volunteers and MyD patients. In comparison with healthy volunteers a lower pH and higher concentrations of both CK and Na+ were found in MyD patients. The concentrations of K+, inorganic phosphate, lactate, and ammonia increase upon exercise in all groups. The mean increase in plasma K+ for healthy volunteers amounted to 0.8 mM (= 23%). In MyD patients a significantly higher increase in plasma K+ was found [mean 2.2 mM (= 65%)]. No abnormal release of K+ from muscular tissue was found in the disease control groups. Data on the postexercise increase in the concentration of other muscular constituents such as creatine kinase, inorganic phosphate, or creatine exclude the possibility of a generally increased membrane permeability in MyD. The abnormally high increase of plasma K+ upon muscular exercise seems to be specific for MyD and may be related to the biochemical defect in this disease.

摘要

我们研究了强直性肌营养不良(MyD)患者、疾病对照组和健康志愿者在进行缺血性前臂运动后血浆电解质水平的变化。在运动前,健康志愿者和MyD患者之间的pH值、肌酸激酶和Na+浓度存在显著差异。与健康志愿者相比,MyD患者的pH值较低,CK和Na+浓度较高。所有组在运动后K+、无机磷酸盐、乳酸和氨的浓度均升高。健康志愿者血浆K+的平均升高幅度为0.8 mM(=23%)。在MyD患者中,血浆K+的升高幅度明显更高[平均2.2 mM(=65%)]。在疾病对照组中未发现肌肉组织有异常的K+释放。关于运动后其他肌肉成分如肌酸激酶、无机磷酸盐或肌酸浓度升高的数据排除了MyD中普遍膜通透性增加的可能性。肌肉运动后血浆K+异常大幅升高似乎是MyD特有的,可能与该疾病的生化缺陷有关。

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Muscle Nerve. 1990 Jan;13(1):27-32. doi: 10.1002/mus.880130107.
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Exertional rhabdomyolysis in a patient with calcium adenosine triphosphatase deficiency.
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J Neurol Neurosurg Psychiatry. 1993 Jul;56(7):823-6. doi: 10.1136/jnnp.56.7.823.
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Br J Ophthalmol. 1992 Oct;76(10):594-7. doi: 10.1136/bjo.76.10.594.
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