Lykkesfeldt G, Bennett P, Lykkesfeldt A E, Micic S, Møller S, Svenstrup B
Clin Endocrinol (Oxf). 1985 Oct;23(4):385-93. doi: 10.1111/j.1365-2265.1985.tb01096.x.
Serum levels of sex hormone binding globulin (SHBG), testosterone, free testosterone, dihydrotestosterone, androstenedione, dehydroepiandrosterone sulphate, oestradiol, oestrone, oestrone sulphate, FSH, and LH were measured in 20 steroid sulphatase-deficient men with recessive X-linked ichthyosis and in normal men. The serum oestrone sulphate level was significantly higher than normal in the patients (P less than 0.0001). In affected men, there was a tendency towards higher dehydroepiandrosterone sulphate levels and no decline with age was seen in the patients as opposed to normal men (interaction: P less than 0.025). Serum androstenedione, and oestradiol levels were lower than normal in the patients (P less than 0.0005 and P = 0.055, respectively), while their LH level was higher than normal (P less than 0.0005). The serum levels of SHBG, total and free testosterone, dihydrotestosterone, oestrone, and FSH were not significantly different from normal in the icthyotic patients. We suggest that the observed abnormalities in these patients are a consequence of the enzyme deficiency which severely impairs the ability of tissues to hydrolyse steroid sulphates.
对20名患有隐性X连锁鱼鳞病的类固醇硫酸酯酶缺乏症男性患者和正常男性的血清性激素结合球蛋白（SHBG）、睾酮、游离睾酮、二氢睾酮、雄烯二酮、硫酸脱氢表雄酮、雌二醇、雌酮、硫酸雌酮、促卵泡激素（FSH）和促黄体生成素（LH）水平进行了测定。患者的血清硫酸雌酮水平显著高于正常水平（P＜0.0001）。与正常男性相比，患病男性的硫酸脱氢表雄酮水平有升高趋势，且患者未出现随年龄增长而下降的情况（交互作用：P＜0.025）。患者的血清雄烯二酮和雌二醇水平低于正常水平（分别为P＜0.0005和P = 0.055），而其LH水平高于正常水平（P＜0.0005）。鱼鳞病患者的血清SHBG、总睾酮和游离睾酮、二氢睾酮、雌酮以及FSH水平与正常水平无显著差异。我们认为，这些患者中观察到的异常是酶缺乏的结果，该酶缺乏严重损害了组织水解类固醇硫酸盐的能力。
