Abbasi A A, Prasad A S, Ortega J, Congco E, Oberleas D
Ann Intern Med. 1976 Nov;85(5):601-5. doi: 10.7326/0003-4819-85-5-601.
Thirty-two adult patients with sickle cell anemia were evaluated endocrinologically. Secondary sex characteristics were abnormal in 29, and eunuchoidal skeletal proportions were present in all except one. The age at which different stages of pubic hair growth were attained in these patients was delayed in comparison to normals (P less than 0.005). Hormonal assays were carried out in 14 patients. Basal serum testosterone, dihydrotestosterone, and androstenedione values were lower (P less than 0.02) in patients than controls. Serum LH and FSH levels before and after stimulation with gonadotropin-releasing hormone were consistent with primary testicular failure. Erythrocyte and hair zinc concentrations were significantly decreased, and there was positive correlation between erythrocyte zinc and serum testosterone (r = 0.61, P less than 0.01) in sickle cell anemia. Our study shows that androgen deficiency in this disease is a result of primary rather than secondary hypogondadism. Further studies are required to establish the role of zinc in the pathogenesis of testicular failure in sickle cell anemia.
对32例成年镰状细胞贫血患者进行了内分泌评估。29例患者的第二性征异常，除1例患者外，其余患者均有类无睾体型骨骼比例。与正常人相比，这些患者达到不同阴毛生长阶段的年龄延迟（P<0.005）。对14例患者进行了激素检测。患者的基础血清睾酮、双氢睾酮和雄烯二酮值低于对照组（P<0.02）。用促性腺激素释放激素刺激前后的血清促黄体生成素（LH）和促卵泡生成素（FSH）水平与原发性睾丸功能衰竭一致。镰状细胞贫血患者的红细胞和毛发锌浓度显著降低，且红细胞锌与血清睾酮之间存在正相关（r=0.61，P<0.01）。我们的研究表明，该疾病中的雄激素缺乏是原发性而非继发性性腺功能减退的结果。需要进一步研究以确定锌在镰状细胞贫血睾丸功能衰竭发病机制中的作用。
