Sugiarto Ariella Maisie, Soelistijo Soebagijo Adi
Department of Internal Medicine, Faculty of Medicine, Universitas Airlangga - Dr. Soetomo General Academic Hospital, Surabaya, Indonesia.
Ann Med Surg (Lond). 2022 Jan 26;74:103289. doi: 10.1016/j.amsu.2022.103289. eCollection 2022 Feb.
Isolated Hypogonadotropic Hypogonadism (IHH) is a clinical syndrome that results from gonadal failure due to abnormal pituitary gonadotropin levels, in the presence of normal baseline and reserve testing of the remaining pituitary hormones.
An 18 years old female came with primary amenorrhea, accompanied by poor breast and pubic development, with low levels of estradiol and gonadotropins but normal levels of other anterior pituitary hormones. Imaging of the hypothalamic-pituitary region revealed hypophyseal hypoplasia due to ischemia. Sex steroids therapy was given to induce pubertal development. IHH represents a rare condition but with a good prognosis.
Early diagnosis and treatment can prevent negative physical and psychological sequelae, and restore fertility in affected patients. Constant surveillance is required due to the possibility of gonadal axis reversal and/or relapse of gonadal axis failure and to identify any adverse effects related to therapy.
Early identification of IHH can help in treatment efficiency.
孤立性低促性腺激素性性腺功能减退症（IHH）是一种临床综合征，由于垂体促性腺激素水平异常导致性腺功能衰竭，而其余垂体激素的基线和储备测试正常。
一名18岁女性因原发性闭经前来就诊，伴有乳房和阴毛发育不良，雌二醇和促性腺激素水平低，但其他垂体前叶激素水平正常。下丘脑-垂体区域成像显示因缺血导致垂体发育不全。给予性类固醇疗法以诱导青春期发育。IHH是一种罕见疾病，但预后良好。
早期诊断和治疗可预防负面的身体和心理后遗症，并恢复受影响患者的生育能力。由于性腺轴逆转和/或性腺轴功能衰竭复发的可能性以及识别与治疗相关的任何不良反应，需要持续监测。
早期识别IHH有助于提高治疗效率。
