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1980年～1988年共遇到8例HalIermann-Streiff综合征,2例进行了7年后随访。病人包括男性6例,女性2例,年龄在8岁～32岁之间。临床表现为:①鸟面畸形;②先天性白内障或无晶体在全部病例中均具备;③牙齿异常7例;④体儒4例,其中1例(例7,男,32岁,身高1.23m,体重29.5 kg)前囟门未闭;⑤皮肤萎缩7例,萎缩灶发亮菲薄,特别在鼻尖部,3例有1～2块斑秃;⑥毛发稀少8例;⑦双侧小眼球6例。①和②对本征的诊断具有重要意义。
From 1980 to 1988, a total of 8 cases of HalIermann-Streiff syndrome were encountered, and 2 cases were followed up for 7 years. The patients included 6 males and 2 females, aged from 8 to 32 years old. The clinical manifestations were: ① bird face deformity; ② congenital cataracts or no lens in all cases are available; ③ 7 cases of abnormal teeth; ④ Confucianism in 4 cases, of which 1 case (Example 7, male, 32 years old, height 1.23m , Body weight 29.5 kg) before the fontanelle closed; ⑤ skin atrophy in 7 cases, atrophy focus thin, especially in the nasal tip, 3 cases of 1 to 2 alopecia areata; ⑥ hair scarce in 8 cases; ① and ② on the intrinsic diagnosis of great significance.