LAUSR

Background 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. Case presentation 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. Discussion 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. Conclusion Early identification of IHH can help in treatment efficiency.