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正角κ:先天性眼球震颤患者白化病的一个体征。

Positive angle kappa: a sign of albinism in patients with congenital nystagmus.

作者信息

Brodsky Michael C, Fray Katherine J

机构信息

University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

出版信息

Am J Ophthalmol. 2004 Apr;137(4):625-9. doi: 10.1016/j.ajo.2003.11.066.

Abstract

PURPOSE

To determine whether the association of positive angle kappa and congenital nystagmus is a distinguishing feature of albinism.

DESIGN

Observational case series.

METHODS

Prospective examination of the location of the corneal light reflex in patients with albinism and idiopathic congenital nystagmus.

RESULTS

A positive angle kappa in at least one eye was noted in 20/21 (95%) patients with albinism versus 4/12 (33%) patients with congenital nystagmus (P =.0003, Fisher exact test). A positive angle kappa in both eyes was noted in 15/21 (71.4%) patients with albinism versus 2/12 (16.6%) patients with idiopathic congenital nystagmus (P =.0039, Fisher exact test).

CONCLUSION

A positive angle kappa in patients with congenital nystagmus is associated with albinism. The pathophysiology of the positive angle kappa may relate to the anomalous decussation of optic axons that characterizes the albinotic visual system.

摘要

目的

确定正角κ与先天性眼球震颤的关联是否为白化病的一个显著特征。

设计

观察性病例系列。

方法

对白化病和特发性先天性眼球震颤患者角膜反光位置进行前瞻性检查。

结果

21例白化病患者中有20例(95%)至少一只眼存在正角κ,而12例先天性眼球震颤患者中有4例(33%)存在正角κ(P = 0.0003,Fisher精确检验)。21例白化病患者中有15例(71.4%)双眼存在正角κ,而12例特发性先天性眼球震颤患者中有2例(16.6%)双眼存在正角κ(P = 0.0039,Fisher精确检验)。

结论

先天性眼球震颤患者的正角κ与白化病有关。正角κ的病理生理学可能与白化病视觉系统特有的视神经轴突异常交叉有关。

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