PROSTATE VISUALIZATION STUDIES IN MALES HOMOZYGOUS AND HETEROZYGOUS FOR 5-ALPHA-REDUCTASE DEFICIENCY
PROSTATE VISUALIZATION STUDIES IN MALES HOMOZYGOUS AND HETEROZYGOUS FOR 5-ALPHA-REDUCTASE DEFICIENCY
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DOI：
10.1210/jc.75.4.1022
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发表时间：
1992-10-01
影响因子：
5.8
通讯作者：
KAZAM, E
中科院分区：
文献类型：
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作者：
IMPERATOMCGINLEY, J;GAUTIER, T;KAZAM, E
5 α-还原酶缺乏的男性假两性畸形患者在直肠检查时生殖器模糊，前列腺不可触及，提示这些结构依赖于双氢睾酮。为了清楚地描述前列腺的状态，对5 α-还原酶缺乏的男性假两性体进行了前列腺的经直肠超声检查，并将结果与年龄匹配的男性对照组进行了比较。在六个男性假两性，磁共振成像研究的前列腺也进行了。杂合子的父亲也进行了前列腺的经直肠超声检查，并将结果与年龄匹配的对照组进行比较。在前列腺超声和磁共振成像上，男性假两性体的前列腺都表现为尿道后的板状软组织结构。前列腺体积，通过两种不同的方法，前列腺超声确定，是显着小于（约十分之一）的体积比年龄匹配的controls.Transurethral超声引导活检的前列腺在两个受影响的主题显示间质组织。这些结果与受影响受试者中检测不到的前列腺特异性抗原相关，表明上皮萎缩或缺乏上皮分化。这项研究表明，前列腺正常分化和生长依赖于双氢睾酮。男性假两性体中存在一些前列腺组织可能是由于5 α-还原酶活性降低而不是缺乏，和/或患有这种疾病的受试者中睾酮水平的升高部分补偿了双氢睾酮水平的降低。杂合子父亲的双氢睾酮生产足够的正常前列腺的生长和发育。
Male pseudohermaphrodites with 5alpha-reductase deficiency have ambiguous genitalia and nonpalpable prostates on rectal examination, suggesting the dihydrotestosterone dependency of these structures. To clearly delineate the status of the prostate, male pseudohermaphrodites with 5alpha-reductase deficiency had transrectal sonography of the prostate performed, and the results were compared to that of age-matched male controls. In six male pseudohermaphrodites, magnetic resonance imaging studies of the prostate were also performed. Heterozygote fathers also had transrectal sonography of the prostate performed and the results compared to age-matched controls.The prostates of the male pseudohermaphrodites appeared as plate-like soft tissue structures posterior to the urethra on both prostatic ultrasound and magnetic resonance imaging. Prostatic volume, as determined on prostatic ultrasound by two different methods, was significantly smaller (approximately one-tenth) than the volume of age-matched controls.Transurethral ultrasound guided biopsy of the prostate in two affected subjects revealed stromal tissue. These results correlate with undetectable prostate-specific antigen in affected subjects, suggesting atrophic epithelium or lack of epithelial differentiation.This study demonstrates the dihydrotestosterone dependence of the prostate for normal differentiation and growth. The presence of some prostatic tissue in the male pseudohermaphrodites may be due to the fact that there is a decrease and not an absence of 5alpha-reductase activity, and/or that the increased level of testosterone in subjects with this condition partially compensates for the decreased level of dihydrotestosterone.There was no difference, however, in prostate size between heterozygous fathers and age-matched control males. The heterozygote fathers had dihydrotestosterone production sufficient for normal prostate growth and development.