Chibisov I V, Karmanskiĭ I M, Sheliapina V V, Leont'ev A F, Rozenfel'd E L
Vopr Med Khim. 1978 Jul-Aug;24(4):555-9.
Distinct accumulation of glycogen, anomalous in structure, and absence of amylo-1,6-glucosidase activity were observed in studies of material obtained by biopsy from liver and muscle tissues of a patient with generalized form of glycogenosis type III. Anamalous glycogen (limitdextrin) was also found in erythrocytes. Concentration of lipoproteins, especially of low density lipoproteins 12.20 S and 0-12 S, was increased in blood serum. Spectrum of lipoproteins acquired a tendency to normalization simultaneously with clinical improvement after intravenous administration of glucose and treatment with cholesterolamine per os.
在对一名III型糖原贮积症全身性患者的肝脏和肌肉组织活检材料进行研究时，观察到糖原的独特积聚、结构异常以及缺乏淀粉-1,6-葡萄糖苷酶活性。在红细胞中也发现了异常糖原（极限糊精）。血清中脂蛋白的浓度，尤其是低密度脂蛋白12.20 S和0 - 12 S的浓度升高。静脉注射葡萄糖和口服胆固醇胺治疗后，随着临床症状改善，脂蛋白谱有趋于正常化的趋势。
