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대한생식의학회지 제25권 제3호 2010년
KALIG _ 1유전자 음성을 보인 격리성 성선자극호르몬 부족증 1례
포천중문의과대학 산부인과학교실
남윤성, 이숙환, 곽인평, 윤태기, 차광열,
A Case of Isolated Gonadotropin Deficiency with Negative KALIG _ 1 Gene
Nam ys, Lee sh, Kwak ip, Yoon tk, Cha ky
Hypogonadotropic hypogonadism, or the lack of function of the testis or ovary secondary to the lack of pituitary and or hypothalamic trophic hormones, is also sometimes generally termed Kallmann’s syndrome. Whether such deficiencies arise from an inborn error of hypothalamic organization and pituitary connection or damage to the hypothalamic pituitary system in prepubertal life, the manifestations of a eunuchoid or apubertal individual with potentially competent pituitary and gonadal function will result. Beyond the achievement of puberty, a similar situation can be recreated by the administration of a long - acting GnRH analog or by conditions of secondary hypothalamic dysfunction such as anorexia nervosa where shutdown of GnRH and its resultant effects cause cessation of gonadal function and even a regression of secondary sexual characteristics. Technically, these conditions are not Kallmann’s syndrome but one must recognize the similarities. We have experienced a case of isolated gonadotropin deficiency which showed a negative KALIG – 1 gene in infertile patient with primary amenorrhea. So we report this case with a brief review of literatures.
키워드 : Isolated gonadotropin deficiency, KALIG – 1
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