Management of the Female With Non-Classical Congenital Adrenal Hyperplasia: A Patient-Oriented Approach

    June 2019 in “ Frontiers in Endocrinology
    Sarantis Livadas, Christina Bothou
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    TLDR The document concludes that managing non-classical congenital adrenal hyperplasia in females requires personalized treatment, genetic counseling, and a team of specialists.
    The document provides a comprehensive overview of the management of non-classical congenital adrenal hyperplasia (NCCAH) in females, highlighting the importance of a patient-oriented approach. It discusses the challenges in diagnosing NCCAH due to its symptoms often being mistaken for other hyperandrogenic disorders and recommends the Synacthen test and molecular testing for accurate diagnosis. Treatment goals include regular menstrual cycles, reduced hirsutism and acne, and improved fertility, with hydrocortisone treatment for severe hyperandrogenism, and oral contraceptives and/or antiandrogens for hirsutism and acne. A study within the document found that 4.2% of 950 hyperandrogenic women had NCCAH, with a high prevalence of hirsutism and increasing alopecia with age. Management involves glucocorticoid therapy, with concerns about long-term adverse effects and subfertility, which is significant in NCCAH patients. The document also covers fertility issues, genetic testing, and treatment during pregnancy, noting an 11% infertility incidence and recommending glucocorticoid therapy to reduce miscarriage rates. It stresses the need for individualized treatment, genetic counseling, and psychological support, and calls for physicians to stay updated with the latest developments and integrate new information into clinical practice. The ideal management includes a multidisciplinary approach with specialists such as dermatologists, gynecologists, and psychologists, and emphasizes lifestyle improvements and psychological support.
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