Fukutani K
Department of Urology, Asoka Hospital.
Nihon Rinsho. 1997 Nov;55(11):2920-4.
Male pseudohermaphroditism is a condition of sex differentiation disorders in which the gonads are tests, but the genital ducts and/or external genitalia are incompletely masculinized. This syndrome is caused by a failure of the sequential process in embryonal development of the testis. In the presence of functioning testis the Müllerian ducts regress, while the mesonephric ducts and urogenital sinus differentiate into the internal and external male genitalia. Male pseudohermaphroditism is classified to subtypes according to etiological factors: (1) testicular unresponsiveness to hCG and LH; (2) defect in testosterone biosynthesis; (3) end-organ resistance to androgen; (4) defects in the intracellular metabolism of testosterone; (5) aberrations in testicular organogenesis; (6) defects in anti-Müllerian hormone.
男性假两性畸形是一种性分化障碍疾病，其性腺为睾丸，但生殖管道和/或外生殖器未完全男性化。该综合征是由睾丸胚胎发育过程中连续进程的失败引起的。在有功能的睾丸存在的情况下，苗勒管退化，而中肾管和泌尿生殖窦分化为男性内、外生殖器。男性假两性畸形根据病因分为以下亚型：(1)睾丸对人绒毛膜促性腺激素(hCG)和促黄体生成素(LH)无反应；(2)睾酮生物合成缺陷；(3)终末器官对雄激素抵抗；(4)睾酮细胞内代谢缺陷；(5)睾丸器官发生异常；(6)抗苗勒管激素缺陷。
