Schweikert H U, Knauf W, Romalo G, Höller W, Bidlingmaier F, Knorr D
Medizinische Universitäts-Poliklinik, Universität Bonn, Germany.
Horm Metab Res. 1987 Oct;19(10):497-501. doi: 10.1055/s-2007-1011862.
Androgens stimulate development and growth of the external male genitalia. Since hypospadias represents the most common congenital abnormality in the male newborn and the mechanism of action in this disorder is still unclear, androgen binding was assessed in cultured fibroblasts from biopsies from genital skin of 10 patients with idiopathic hypospadias. For comparison, binding was determined in corresponding samples from 8 males with normal penile development and from 9 patients with known androgen resistance syndromes (testicular feminization, Reifenstein syndrome, pseudovaginal perineoscrotal hypospadias). Finally, binding was measured in 10 samples of nongenital skin. Maximum specific binding (Bmax) in idiopathic hypospadias varied from 3.2 to 15.5 (median 6.6) fmol.mg protein-1. Bmax in samples of persons with normal genital development was between 12.2 and 17.9 fmol.mg protein-1 (median 13.2). Bmax in samples of patients with known androgen resistance syndromes was exactly in the range reported previously in the literature. It is evident that Bmax in samples of patients with idiopathic hypospadias differs significantly (P less than 0.01), (Mann Whitney U-test) from those with normal genital development. Thus it seems reasonable to conclude that in some patients with idiopathic hypospadias the genital defect is caused by receptor deficiency.
雄激素刺激男性外生殖器的发育和生长。由于尿道下裂是男性新生儿中最常见的先天性异常,且该疾病的作用机制仍不清楚,因此对10例特发性尿道下裂患者生殖器皮肤活检的培养成纤维细胞中的雄激素结合情况进行了评估。作为对照,测定了8例阴茎发育正常男性以及9例已知雄激素抵抗综合征(睾丸女性化、赖芬斯坦综合征、会阴阴囊假阴道性尿道下裂)患者相应样本中的雄激素结合情况。最后,测定了10份非生殖器皮肤样本中的雄激素结合情况。特发性尿道下裂的最大特异性结合(Bmax)在3.2至15.5(中位数6.6)fmol·mg蛋白-1之间。生殖器发育正常者样本的Bmax在12.2至17.9 fmol·mg蛋白-1之间(中位数13.2)。已知雄激素抵抗综合征患者样本的Bmax正好在先前文献报道的范围内。显然,特发性尿道下裂患者样本的Bmax与生殖器发育正常者样本的Bmax有显著差异(P小于0.01)(曼-惠特尼U检验)。因此,似乎有理由得出结论,在一些特发性尿道下裂患者中,生殖器缺陷是由受体缺陷引起的。