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目的 探讨Kallmann综合征的临床诊治特点。方法 回顾分析 12例Kallmann综合征病人的临床资料 ,均为男性 ,年龄 19～ 2 8岁 ,青春期后外生殖器男性第二性征不发育 ,无阴茎勃起及遗精 ,全部病例伴嗅觉缺失或不同程度的减退 ;性染核型均为 4 6XY ,血清黄体生成素、卵泡刺激素、睾酮水平明显降低。结果 12例均用HCG治疗 ,治疗后外生殖器和第二性征有不同程度的发育 ,7例已婚者恢复性生活。 6例病人的精液量较治疗前增多。结论 促性腺功能低下型性腺功能低下和嗅觉缺失或低下是本病的特征性临床表现 ,目前对本征尚无根治措施 ,仅限于替代治疗 ,早期诊断是治疗Kallmann综合征的关键。
Objective To investigate the clinical diagnosis and treatment of Kallmann’s syndrome. Methods The clinical data of 12 patients with Kallmann’s syndrome were retrospectively analyzed. All of them were male, ranging in age from 19 to 28 years. After adolescence, the second sexual characteristics of male genitals were not developed, no penile erection and nocturnal emission were found. All cases were associated with loss of sense of smell or different degrees Of the descending; sex-stained karyotype are 4 6XY, serum luteinizing hormone, follicle stimulating hormone, testosterone levels were significantly lower. Results All 12 cases were treated with HCG. After treatment, the external genitalia and secondary sexual characteristics had different degrees of development, and 7 married people recovered from life. 6 patients semen volume increased compared with before treatment. Conclusions Gonadal hypoplasia hypogonadism and olfactory loss or hypofunction are the characteristic clinical manifestations of the disease. At present, there is no radical treatment for this symptom, so it is limited to replacement therapy. Early diagnosis is the key to the treatment of Kallmann syndrome.