Leys M J, Schreiner L A, Hansen R M, Mayer D L, Fulton A B
Department of Ophthalmology, Children's Hospital, Boston, MA 02115.
Am J Ophthalmol. 1988 Nov 15;106(5):561-9. doi: 10.1016/0002-9394(88)90586-7.
We studied the visual acuities and dark-adapted sensitivities of 12 children with Bardet-Biedl syndrome. All except one child, who was seen only once, were tested serially. In the first decade of life, all visual acuities were within 2 octaves of normal. All but two final visual acuities obtained from patients in their second and third decades were more than 2 octaves poorer than normal. Dark-adapted sensitivities of all patients were, or became, significantly less than normal even in those patients whose period of follow-up was limited to the first decade of life. Of the 11 patients measured serially, seven showed decreases in dark-adapted sensitivities of at least 0.5 log unit during the follow-up period, and the last measured sensitivities of all patients were at least 2 log units less than the normal mean.
我们研究了12名患有巴德-比德尔综合征儿童的视力和暗适应敏感度。除了一名仅接受过一次检查的儿童外，其他所有儿童均接受了系列检查。在生命的第一个十年中，所有儿童的视力均在正常范围的2个倍频程之内。从处于第二个和第三个十年的患者获得的最终视力中，除两名患者外，其他所有患者的视力均比正常情况差2个倍频程以上。即使是那些随访期仅限于生命第一个十年的患者，所有患者的暗适应敏感度也均显著低于正常水平，或变得显著低于正常水平。在接受系列测量的11名患者中，有7名患者在随访期间暗适应敏感度下降了至少0.5对数单位，并且所有患者最后一次测量的敏感度比正常平均值至少低2对数单位。
