Fundus albipunctatus: A clinical study of the fundus lesions, the physiologic deficit, and the vitamin a metabolism
Fundus albipunctatus: A clinical study of the fundus lesions, the physiologic deficit, and the vitamin a metabolism
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眼底白斑：眼底病变、生理缺陷和维生素 A 代谢的临床研究
DOI：
10.1007/bf01569200
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发表时间：
1977
影响因子：
1.4
通讯作者：
M. Marmor
中科院分区：
文献类型：
--
作者：
M. Marmor
两个不相关的眼底白斑患者，每一个产品的近亲婚姻，进行了研究，参考他们的眼底病变，他们的生理缺陷和维生素A代谢。这两个病人都表现出白斑病变，出现深血管和阻塞荧光素或不可见的血管造影。一些病变在1-2年的观察期内发生了变化。1例眼底呈放射状排列。这两个病人都表现出极大的延迟暗适应，测量主观或与视网膜电图。视锥细胞和视杆细胞功能障碍，视野和色觉的细微异常被发现。视网膜电图的a波和b波都受到疾病的影响，这表明在a波产生之前存在适应缺陷。这些发现与卡尔、里普斯和西格尔（1974）的观察结果一致，即在这种疾病中，色素再生迟缓。两名患者的维生素A、胡萝卜素、视黄醇结合蛋白、氨基酸、蛋白质和脂蛋白的血液水平均正常。胃肠外和口服治疗水平的维生素A的管理没有影响的速度暗适应或fundamentallesions.This研究支持的观点，眼底albipunctatus是一个固定的隐性疾病，可以定义为生理学上的暗适应和视觉色素再生的速度缓慢，这是临床上分离进行性营养不良，如视网膜炎斑点albescens。眼底白斑的意义尚不清楚。如果维生素A代谢参与眼底白斑，参与必须在当地的水平。
Two unrelated patients with fundus albipunctatus, each the product of a consanguinous marriage, were studied with reference to their fundus lesions, their physiologic deficit and their vitamin A metabolism.Both patients showed albipunctate lesions that appeared deep to vessels and either blocked fluorescein or were invisible on angiography. Some of the lesions changed during a 1–2 year observation period. In one patient the lesions showeds a radial arrangement in the fundus. Both patients showed greatly retarded dark adaptation, measured subjectively or with the electroretinogram. Cone as well as rod dysfunction was involved, and subtle abnormalities of the visual fields and of color vision were found. The a- and b-waves of the electroretinogram were both affected by the disease, suggesting an adaptation defect prior to generation of the a-wave. These findings are consistent with the observation of Carr, Ripps & Siegel (1974) that photopigment regeneration is retarded in this disease. Both patients showed normal blood levels of vitamin A, carotene, retinol binding protein, amino acids, proteins and lipoproteins. The administration of therapeutic levels of vitamin A parenterally and orally had no effect on the rate of dark adaptation or the fundus lesions.This study supports the view that fundus albipunctatus is a stationary recessive disorder which can be defined physiologically by a slow rate of dark adaptation and visual pigment regeneration, and which is clinically separable from progressive dystrophies such as retinitis punctata albescens. The significance of the albipunctate fundus lesions remains unclear. If vitamin A metabolism is involved in fundus albipunctatus, the involvement must be at a local level.