Clouston syndrome: an ultrastructural study
Clouston syndrome: an ultrastructural study
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克劳斯顿综合征：超微结构研究
作者：
V. Escobar;L. I. Goldblatt;D. Bixler;D. Weaver
描述了一个以前未描述的受Clouston综合征（少汗性外胚层发育不良）影响的法裔加拿大家庭。毛发的超微结构研究显示毛原纤维紊乱，表皮皮质丢失。扫描电镜的结果是一致的模型，表明在角质的皮肤系统的生化缺陷。
A previously undescribed French‐Canadian family affected with Clouston Syndrome (Hypo‐hidrotic Ectodermal Dysplasia) is described. Ultrastructural study of the hair shows disorganization of the hair fibrils with loss of the cuticular cortex. The SEM findings are consistent with the model, suggesting a biochemical defect in the keratin of the integumentary system.