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目的 :探讨Stargardt′s病的临床表现。方法 :回顾性分析 69例 13 8眼Stargardt′s病的临床和眼底荧光素血管造影 (fundusfluoresceinangiographyFFA)资料 ,分析其视力损害程度、年龄分布状态及病灶表现形态。结果 :视力 0 0 2～ 0 1者 115眼 (83 3 3 % ) ,0 2～ 0 5者 2 3眼 (16 66% ) ;近视力最高Jr =2、最低Jr =0。其中 10岁前起病者3 3例、 11～ 2 0岁起病者 2 6例、 2 1～ 3 0岁起病 4例、 3 1～ 40岁起病 6例。病灶形态 :①单纯黄斑病灶型 2 4例 48眼 ;②黄斑病灶联合眼底黄色斑点型 2 9例 5 4眼 ;③黄斑病灶与后极部色素脱失相融合型 16例 3 2眼。第三型病灶形态与前两型病灶在起病年龄与病程长短方面差别无明显意义 (P >0 0 5 )。三种病灶形态的病变程度差别亦无意义 (P >0 0 5 )。结论 :①Stargardt′s病对视力的影响较大 ,但暂未见到视力全部丧失的病人 ;②Stargardt′s病主要出现于青少年时期 ,亦可在成年人后期发病 ;③黄斑病灶与后极部色素脱失融合为一体的特殊病灶可能是Stargardt′s病的另一种特殊的FFA变异形态。
Objective: To investigate the clinical manifestations of Stargardt’s disease. Methods: The clinical and fundus fluorescein angiography (FFA) data of 69 cases of Stargardt’s disease in 13 cases were retrospectively analyzed. The degree of visual impairment, age distribution and the morphology of lesions were analyzed. Results: There were 115 eyes (83.3%) with visual acuity of 0 0 2 ~ 0 1 and 23 eyes (16 66%) with 0 2 ~ 0 5 visual acuity. The highest visual acuity was Jr = 2 and the lowest was Jr = 0. Among them, there were 33 cases of onset before age 10, 26 cases of onset from 11 to 20 years old, 4 cases from 21 to 30 years old and 6 cases from 3 to 40 years old. Morphology of the lesion: ①Four cases of macular lesions were found in 24 cases and 48 eyes; ②Morphosis of macular foci was observed in 29 cases and 54 cases of yellow speckles; ③Different macular degeneration and macular degeneration were seen in 16 cases and 32 eyes. There was no significant difference between the third type of lesions and the first two types of lesions in the age of onset and duration of disease (P> 0.05). There was no significant difference in the degree of lesion between the three lesions (P> 0.05). Conclusion: (1) Stargardt’s disease has a great effect on visual acuity, but no patients with total loss of visual acuity have yet to be seen; (2) Stargardt’s disease mainly occurs in adolescence and later in adults; (3) macular degeneration and posterior pole pigment The special lesion of fusion and loss may be another special variant of FFA of Stargardt’s disease.