少年型家族性黄斑变性(Stargardt氏病)在遗传性黄斑变性中,是最常见的类型之一。但在我国仅有个案报告,尚未见到大组家系与病例分析。为此,将我们近年见到确诊的13个家系(15例)进行分析,旨在了解本病的临床表现、遗传规律与防治要点。对象与结果本组少年型家族性黄斑变性的诊断标准为:1.症状为视力减退。2.眼底有局限于黄斑的萎缩变性区(可有或无眼底黄点)。3.起病年龄为儿童或青少年。一、性别与年龄:本组患者起病年龄可见表1。从表可见起病年龄分布于儿童与青少年期,平均起病年龄为12.4岁。大多数患者起病于少年期(10～14岁)。本组中男性8例,女性7例,两性无明显差异。二、症状与视功能:本病主要症状是进行视力减退。本组患者每例都作了检影验光,矫正视力均在0.3以下(见表2)。从 Juvenile familial macular degeneration (Stargardt’s disease) is one of the most common types in atopic macular degeneration. However, there are only case reports in our country, but we have not seen a large group of family members and case analysis. To this end, we have seen the diagnosis of 13 families in recent years (15 cases) were analyzed in order to understand the clinical manifestations of the disease, genetic rules and prevention and treatment points. Objects and Results The diagnosis of juvenile familial macular degeneration: 1. Symptoms of vision loss. 2. The fundus is confined to the atrophy of the macular degeneration (with or without the yellow point of the fundus). 3. Age of onset for children or adolescents. First, gender and age: onset age of this group of patients can be seen in Table 1. From the table shows the onset age distribution in children and adolescents, the average age of onset was 12.4 years old. Most patients with onset in adolescence (10 to 14 years old). The group of 8 males and 7 females, no significant gender differences. Second, the symptoms and visual function: The main symptoms of this disease is vision loss. The patients in each case had a retinoscopy, corrected visual acuity were below 0.3 (see Table 2). From