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1944年Kallman等报告3个家族(62名亲属)中有9名男性发生性腺发育不良体征并伴有嗅觉障碍。这种病例在国外报道不多,国内更少见。我国最近两年内发现三例并调查其家属。今报道如下病例1 张××,男性,22岁。因身材矮、外阴部发育不良求治。患者自懂事起自觉身材比同年龄矮小。右眼视力0.1,左眼1.5,嗅觉差,只有气味强烈如氨水能辨出。身高167厘米,体重59公斤,智力正常,无腋毛和阴毛,睾丸明显萎缩,阴茎短小,能勃起,有射精,精液量4毫升,计数为1.9 ×10~7,活动率20%。家属中父亲和大姐嗅觉障碍,父母非近亲婚配。尿F.S.H.6.6单位,17酮类固醇10.6毫
In 1944, Kallman et al. Reported that 9 of the 3 families (62 relatives) had hypogonadal signs accompanied by olfactory dysfunction. This case is not reported in foreign countries, more rare in China. Three cases were found in our country in the last two years and their relatives were investigated. Now reported as follows a case × ×, male, 22 years old. Because of short stature, vulva dysplasia seeking treatment. Patients self-consciously conscious body than the same age short. Right eye 0.1, 1.5 left eye, poor sense of smell, only strong smell such as ammonia can be identified. Height 167 cm, weight 59 kg, normal intelligence, no armpit hair and pubic hair, testicular atrophy, short penis, erection, ejaculation, sperm volume 4 ml, a count of 1.9 × 10 ~ 7, activity rate of 20%. Family members of the eldest sister and sister smell disorders, parents non-relatives marriage. Urine F.S.H.6.6 units, 17 ketosteroids 10.6 milliliters