Gonadal Function and Reproductive Health Challenges in Triple a Syndrome: A Narrative Review

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Background and Objectives: Triple A syndrome (TAS) is a rare autosomal recessive disorder characterized by the triad of adrenocorticotropic hormone (ACTH)-resistant adrenal insufficiency, alacrimia (absence of tear production), and achalasia. This article aims to provide a better understanding of gonadal function and reproductive health in TAS by summarizing existing data from the literature regarding this topic. Materials and Methods: A comprehensive literature review was carried out until September 2025, using four electronic databases (PubMed, Embase, Web of Science and Scopus). Results: The review included a total of 25 articles. The most frequent findings in the articles included in the review were erectile dysfunction, ejaculatory dysfunction, delayed puberty, hypogonadism and fertility issues. Furthermore, several studies revealed reduced adrenal androgen levels in male patients, while other case reports documented the presence of osteoporosis in individuals with TAS. Conclusions: Gonadal dysfunction and reproductive health challenges in TAS remain underexplored aspects. The multisystemic nature of TAS requires a comprehensive approach to patient care for optimizing quality of life, and this review underscores the importance of evaluating reproductive function in individuals with this rare syndrome.

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