造精機能導入に成功したKlallmann症候群の1例

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A 26-year-old single man visited our hospital with the chief complaint of having neither an ejaculation nor an orgasm. He was 180 cm tall and weighed 65 kg. He had very sparse pubic hair and no palpable prostate. Both testes were fairly small: The left testis was 28×19×17 mm. The basal levels of LH, FSH and testosterone in the blood were 4.5mIU/ml, 2.0mIU/ml and 68ng/dl, respectively, which showed hypo gonadotropic hypogonadism. He was found to have anosmia in the venous olfactory test with Alinamine(R) (Thiamin propyl disulfide). The diagnosis of Kallmann's syndrome was made by the above findings. To induce secondary sexual characteristics, hCG of 1000 I.U. was administered twice a week. After 30 weeks of hCG administration, he had pubic hair which was of female type, and he was bearded. Then he was able to have an ejaculation and the results of the semen analysis were: volume 1.5ml, sperm count 82×10/ml and motility 30%.
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