Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy.
Department of Obstetrics and Gynecology, Azienda Ospedaliera Nazionale SS. Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
Front Endocrinol (Lausanne). 2024 Aug 29;15:1423898. doi: 10.3389/fendo.2024.1423898. eCollection 2024.
Functional hypothalamic amenorrhea (FHA) is a common cause of amenorrhea and chronic anovulation in adolescent girls and young women, diagnosed after excluding other organic causes. It is commonly associated with calorie restriction, excessive physical exercise, and psychosocial stress. These stressors alter the pulsatile secretion of gonadotropin-releasing hormone, leading to a chronic condition of hypoestrogenism and significant health consequences. Recent evidence has highlighted a genetic predisposition to FHA that could explain interindividual variability in stress response. Indeed, not all women experience FHA in response to stress. Rare variants in genes associated with idiopathic hypogonadotropic hypogonadism have been identified in women with FHA, suggesting that these mutations may contribute to an increased susceptibility of women to the trigger of stress exposure. FHA appears today as a complex disease resulting from the combination of genetic predisposition, environmental factors, and epigenetic changes. Furthermore, the genetic background of FHA allows for the hypothesis of a male counterpart. Despite the paucity of data, preliminary findings indicate that an equivalent condition of FHA exists in men, warranting further investigation. This narrative review aims to summarize the recent genetic evidence contributing to the pathophysiology of FHA and to raise awareness on a possible male counterpart.
功能性下丘脑性闭经(FHA)是青春期女孩和年轻女性闭经和慢性无排卵的常见原因,在排除其他器质性原因后诊断。它通常与热量限制、过度体育锻炼和心理社会压力有关。这些应激源改变了促性腺激素释放激素的脉冲分泌,导致雌激素持续低下,并产生重大健康后果。最近的证据强调了 FHA 的遗传易感性,这可以解释应激反应中的个体间差异。事实上,并非所有女性在应激反应中都会出现 FHA。在 FHA 女性中发现了与特发性低促性腺激素性性腺功能减退症相关的基因中的罕见变异,这表明这些突变可能导致女性对应激暴露的易感性增加。FHA 如今表现为一种复杂的疾病,是遗传易感性、环境因素和表观遗传变化共同作用的结果。此外,FHA 的遗传背景允许存在男性对应物的假说。尽管数据有限,但初步研究结果表明,男性中存在类似 FHA 的情况,值得进一步研究。本综述旨在总结 FHA 病理生理学的最新遗传证据,并提高对可能的男性对应物的认识。