NATURAL COURSE OF VISUAL FUNCTIONS IN THE BARDET-BIEDL SYNDROME

NATURAL COURSE OF VISUAL FUNCTIONS IN THE BARDET-BIEDL SYNDROME
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DOI:
10.1001/archopht.1993.01090110066026
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发表时间:
1993-11-01
影响因子:
--
通讯作者:
GLYNN, RJ
GLYNN, RJ
中科院分区:
其他
文献类型:
--
作者:
FULTON, AB;HANSEN, RM;GLYNN, RJ

文献摘要

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目的:为了确定Bardet-Biedl syndrome.Patients和方法的患者的视觉功能的过程中:21例Bardet-Biedl综合征在儿童医院,波士顿,马萨诸塞州,有视标和光栅敏锐度和暗适应阈值随时间的推移进行测量。首次访视时的年龄范围为2周至23岁(中位年龄为6岁)。结果:患者视功能明显下降,光栅和视标视力平均每年下降0.09个对数单位(约1行),阈值平均每年增加0.19个对数单位。这些视觉功能的损失率和预测水平的视觉功能在11至12岁(平均年龄的测量)之间的individual.Conclusions:视力预后儿童Bardet-Biedl综合征是穷人。中央和周边视觉功能的过程是可变的。
Objective: To determine the course of visual functions in patients with Bardet-Biedl syndrome.Patients and Methods: The 21 patients with Bardet-Biedl syndrome seen at Children's Hospital, Boston, Mass, had optotype and grating acuities and dark-adapted thresholds measured over time. Their ages at first visit ranged from 2 weeks to 23 years (median age, 6 years). The courses of the visual functions were analyzed with a random-effects model.Results: Substantial declines in visual functions were found. On average, grating and optotype acuities declined 0.09 log units (roughly 1 line) per year, and thresholds increased about 0.19 log units per year. The rates at which these visual functions were lost and the predicted level of the visual functions at ages 11 to 12 years (the mean ages of measurement) varied among individuals.Conclusions: The visual prognosis for children with Bardet-Biedl syndrome is poor. The course of both central and peripheral visual functions is variable.