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首页> 外文期刊>Neuromuscular disorders: NMD >Evaluation of muscle glycogen content by 13C NMR spectroscopy in adult-onset acid maltase deficiency.
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Evaluation of muscle glycogen content by 13C NMR spectroscopy in adult-onset acid maltase deficiency.

机译：成年酸性麦芽糖酶缺乏症中13 C NMR光谱法评估肌肉糖原含量。

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Muscle glycogen storage was measured by in vivo, natural abundance 13C nuclear magnetic resonance spectroscopy in distal and proximal lower limb segments of patients suffering from adult-onset acid maltase deficiency. Interleaved T1-weighted acquisitions of glycogen and creatine served to quantify glycogen excess. For acid maltase deficient patients (n=11), glycogen:creatine was higher than controls (n=12), (1.20+/-0.39 vs. 0.83+/-0.18, P=0.0005). Glycogen storage was above the normal 95% confidence limits in at least one site for 7/11 patients. The intra-individual coefficient of reproducibility was 12%. This totally atraumatic measurement of glycogen allows repeated measurement at different muscle sites of acid maltase deficient patients, despite selective fatty replacement of tissue. This could provide an additional parameter to follow the development of disease in individual patients, including in the perspective of forthcoming therapeutic trials. It may also offer an appropriate tool to study the role of glycogen accumulation in progression of the pathology.
机译：通过体内，自然丰度13C核磁共振波谱法在患有成人性酸性麦芽糖酶缺乏症的患者的远端和近端下肢节段中测量了肌肉糖原的存储量。糖原和肌酸的交错T1加权采集可量化糖原过量。对于酸性麦芽糖酶缺乏症患者（n = 11），糖原：肌酸高于对照组（n = 12）（1.20 +/- 0.39 vs.0.83 +/- 0.18，P = 0.0005）。在7/11的患者中，至少一个部位的糖原存储量高于正常的95％置信限。个体内可重复性系数为12％。糖原的完全无创伤性测量允许对酸性麦芽糖酶缺乏症患者的不同肌肉部位进行重复测量，尽管可以选择性地脂肪替代组织。这可以提供额外的参数来跟踪个体患者的疾病发展，包括即将进行的治疗试验。它也可能提供合适的工具来研究糖原积累在病理过程中的作用。

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