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白化病:其对屈光发育的影响。

Albinism: its implications for refractive development.

作者信息

Wildsoet C F, Oswald P J, Clark S

机构信息

Department of Bioscience, New England College of Optometry, Boston, Massachusetts 02115, USA.

出版信息

Invest Ophthalmol Vis Sci. 2000 Jan;41(1):1-7.

Abstract

PURPOSE

Albinism involves the mutation of one or more of the genes associated with melanin synthesis and has many ramifications for vision. This study focuses on the refractive implications of albinism in the context of emmetropization.

METHODS

Refractive, biometric, and visual acuity data were collected for a group of 25 albino individuals that included the following: 18 oculocutaneous (13 tyrosine positive, 5 tyrosine negative); 7 ocular (2 autosomal recessive, 5 sex-linked recessive). Their age range was 3 to 51 years. All exhibited horizontal pendular nystagmus.

RESULTS

There were no statistically significant differences relating to albino subtype for any of the measured parameters. All the subjects had reduced visual acuity (mean: 0.90, logMAR) and overall, there was a bias toward hyperopia in their refractive errors (mean: + 1.07 D). However the refractive errors of the group covered a broad range (SD: 4.67 D) and included both high myopia and high hyperopia. An axial origin to the refractive errors is implied by the high correlation between refractive errors and axial lengths. Refractive astigmatism averaged 2.37 D and was consistently with-the-rule and highly correlated with corneal astigmatism, which was also with-the-rule. Meridional analysis of the refractive data indicated that the vertical meridian for hyperopic subjects was consistently nearer emmetropia compared to their horizontal meridian. Myopic subjects showed the opposite trend.

CONCLUSIONS

The overall refractive profile of the subjects is consistent with emmetropization being impaired in albinism. However, the refractive errors of hyperopic subjects also can be explained in terms of "meridional emmetropization." The contrasting refractive profiles of myopic subjects may reflect operational constraints of the emmetropization process.

摘要

目的

白化病涉及一个或多个与黑色素合成相关基因的突变,对视功能有诸多影响。本研究聚焦于白化病在正视化过程中的屈光相关影响。

方法

收集了一组25名白化病患者的屈光、生物测量和视力数据,其中包括:18例眼皮肤白化病患者(13例酪氨酸阳性,5例酪氨酸阴性);7例眼部白化病患者(2例常染色体隐性遗传,5例性连锁隐性遗传)。他们的年龄范围为3至51岁。所有患者均表现为水平摆动性眼球震颤。

结果

对于任何测量参数,白化病亚型之间均无统计学上的显著差异。所有受试者视力均下降(平均值:0.90,logMAR),总体而言,其屈光不正倾向于远视(平均值:+1.07 D)。然而,该组患者的屈光不正范围较广(标准差:4.67 D),包括高度近视和高度远视。屈光不正与眼轴长度之间的高度相关性提示屈光不正源于眼轴。屈光性散光平均为2.37 D,始终为顺规性,且与角膜散光高度相关,角膜散光也为顺规性。对屈光数据的子午线分析表明,远视患者的垂直子午线相比水平子午线始终更接近正视。近视患者则呈现相反趋势。

结论

受试者的总体屈光特征与白化病中正视化受损一致。然而,远视患者的屈光不正也可用“子午线正视化”来解释。近视患者的屈光特征差异可能反映了正视化过程的操作限制。

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