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先天性肌强直性营养不良早产儿的骨骼肌。形态学和组织化学研究。

Skeletal muscle in preterm infants with congenital myotonic dystrophy. Morphologic and histochemical study.

作者信息

Sahgal V, Bernes S, Sahgal S, Lischwey C, Subramani V

出版信息

J Neurol Sci. 1983 Apr;59(1):47-55. doi: 10.1016/0022-510x(83)90080-1.

Abstract

The skeletal muscle in 3 preterm infants (27, 34, 37 weeks gestation age) born to mothers with myotonic dystrophy showed a syncytial pattern at 27 weeks and a decreasing percentage of satellite cells and central nuclei at 34 and 37 weeks gestation. The fiber type differentiation was observed only at 37 weeks of gestational age. In all 3 cases muscle fibers with multiple acid phosphatase positive were seen. The muscle spindles also had thick capsules and showed lack of morphologic and histochemical differentiation into fiber types. These findings suggest immaturity of skeletal muscle in comparison to the normal. The immaturity of the skeletal muscle correlated well with the prognosis of the patients.

摘要

3例患有强直性肌营养不良母亲所生的早产婴儿(孕龄分别为27、34、37周)的骨骼肌,在孕27周时呈现多核细胞模式,在孕34周和37周时卫星细胞和中央核的百分比逐渐降低。仅在孕37周时观察到纤维类型分化。在所有3例病例中均可见到多个酸性磷酸酶阳性的肌纤维。肌梭也有厚的被膜,且未表现出向纤维类型的形态学和组织化学分化。这些发现提示与正常情况相比骨骼肌不成熟。骨骼肌的不成熟与患者的预后密切相关。

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