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Hallemann—Streiff综合征8例报告
引用本文:方宝康,康前雁,崔守信.Hallemann—Streiff综合征8例报告[J].西安交通大学学报(医学版),1990(4).
作者姓名:方宝康  康前雁  崔守信
作者单位:西安医科大学第一附属医院眼科学教研室 (方宝康,康前雁),西安医科大学第一附属医院眼科学教研室(崔守信)
摘    要: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。

关 键 词:鸟面畸形  先天性白内障  Hallermann-Streiff综合征

HALLERMANN-STREIFF SYNDROME
Fang Baokang,Kang Qiany,Cui Shouxin.HALLERMANN-STREIFF SYNDROME[J].Journal of Xi‘an Jiaotong University:Medical Sciences,1990(4).
Authors:Fang Baokang  Kang Qiany  Cui Shouxin
Abstract:8 cases of Hallermann-streiff syndrome were found trom 1980 to 1988. Two of them had been followed up for 7 years. The patients consisted of 6 males and 2 females at 8-32 years of age. The clinical manifestations were as follows : ① dsycephaly with bird face and ; ② congenital cataract or aphakic in all cases; ③ dental anomaly in 7 cases; ④nanism in 4 cases and the fontanel frontalis (maior) of 1 casc (case 7, males 32 years of age, height 1. 23 m, weight 29. 5 kg) was not closed, ⑤ atrophy of skin in 7 cases whosc skin appeared shine and thin, particularly on the nose top, and one or two areas of alopecia appeared in 3 of 6 cases, ⑥ hypotrichosis in 8 cases and ⑦ bilateral microphthalmia in 6 cases. ① and ② were significant for the diagnosis of Hallemann-Streiff syndrome.
Keywords:dyscephaly with bird face  congenital cataract  Hallermann-Streiff syndrome
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