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一名早产儿的结节性硬化症(作者译)

[Tuberous sclerosis in a premature infant (author's transl)].

作者信息

Probst A, Ohnacker H

出版信息

Acta Neuropathol. 1977 Oct 10;40(2):157-61. doi: 10.1007/BF00688705.

Abstract

A sporadic case of tuberous sclerosis in a stillborn infant is reported. The death at the 31st week of gestation was presumably due to the development of enormous rhabdomyomas of the heart. The typical cerebral lesions were fully developed as in patients decreased later in life. The atypical cells found in the cortical tubers demonstrated ultractructural features of reactive astrocytes. Moreover, they showed innumerable microvillilike projections on their surface and junctional complexes, mostly of the zonula adharens type, reminescent of ependymocytes. The significance of such glio-epithelial cellular features is discussed.

摘要

本文报道了一例死产婴儿患结节性硬化症的散发病例。妊娠31周时死亡可能是由于心脏出现巨大横纹肌瘤。典型的脑损伤已完全形成,与年龄稍大的患者情况相同。在皮质结节中发现的非典型细胞表现出反应性星形胶质细胞的超微结构特征。此外,它们在表面有无数微绒毛样突起和连接复合体,大多为黏着小带类型,类似室管膜细胞。文中讨论了这种神经胶质上皮细胞特征的意义。

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