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Autosomal dominant retinitis pigmentosa: a log quotient analysis of the photopic and scotopic b-wave amplitude.

机译:常染色体显性遗传性视网膜色素变性:对视和暗视b波振幅的对数商分析。

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摘要

The relationship of the photopic and the scotopic b-wave amplitudes of the electroretinogram was studied in 85 normal subjects and 25 patients with autosomal dominant retinitis pigmentosa, in which one amplitude was at least 20 microvolts. The log quotient of their b-wave amplitudes--that is log of the photopic b-wave amplitude divided by the scotopic b-wave amplitude--was considered to represent the activity of cones relative to rods. The log quotient values had a normal gaussian distribution in the normal control eyes, while they formed two groups in the patients with autosomal dominant retinitis pigmentosa. In the first group (type 1), the scotopic b-wave was non-recordable while the photopic b-wave amplitude was larger than 20 microvolts in all cases, indicating that the log quotient is larger than 0.5 and that the rod system is much more severely affected than the cone system. The second group (type 2) had a log quotient smaller than 0.5 and its distribution almost overlapped the normal one, indicating more symmetrical damage in the cone and rod systems. The mean final rod threshold at 45 minutes for type 1 was significantly higher than that for type 2. The log quotient proved to be a useful index for analysing the cone and rod involvement and consequently provides a better understanding of autosomal dominant retinitis pigmentosa.
机译:研究了在85名正常受试者和25名常染色体显性遗传性视网膜炎色素沉着病患者中,视网膜电图的明视与暗视b波振幅之间的关系,其中一个振幅至少为20微伏。他们的b波振幅的对数商-即明视b波振幅的对数除以暗视b波振幅-被认为代表了锥体相对于棒的活动。对数商值在正常对照眼中具有正常的高斯分布,而在常染色体显性遗传性色素性视网膜炎患者中则分为两组。在第一组(1型)中,在所有情况下暗视b波均不可记录,而明视b波振幅均大于20微伏,这表明对数商大于0.5,并且棒系统非常多。比锥体系统受影响更严重。第二组(类型2)的对数商数小于0.5,并且其分布几乎与正态对数重叠,表明圆锥和杆系统中的对称损坏更为明显。 1型在45分钟时的平均最终杆阈值显着高于2型。对数商被证明是分析视锥和视杆受累的有用指标,因此可更好地了解常染色体显性遗传性视网膜炎。

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