Bundschu H D, Hauger W, Lang H D
Dtsch Med Wochenschr. 1975 Jun 13;100(24):1337-41. doi: 10.1055/s-0028-1106383.
Dilatation of the renal pelvis, ureters and bladder appeared in two brothers with myotonic dystrophy (aged 26 and 29 years), probably as a result of involvement of the smooth muscles of these organs. Histochemical differentiation of skeletal muscle fibres (main fibre types I and II, after Engel) indicated a predominant size reduction in type I fibres on demonstrating myofibrillary ATPase. In addition, demonstration of this enzyme also revealed a marked shift of the fibre type proportions in favour of type I. Isolated reduction in gammaG-globulins was also demonstrated. A defect of cellular and humoral response was excluded.
在两名患有强直性肌营养不良的兄弟(分别为26岁和29岁)中出现肾盂、输尿管和膀胱扩张,这可能是这些器官平滑肌受累的结果。骨骼肌纤维的组织化学分化(主要为恩格尔分类的I型和II型纤维)显示,在显示肌原纤维ATP酶时,I型纤维的大小明显减小。此外,该酶的显示还揭示了纤维类型比例明显向I型偏移。还证实了γG球蛋白单独减少。排除了细胞和体液反应缺陷。