Classical and non-classical congenital adrenal hyperplasia: What is the difference in subsequent fertility? |
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| Institution: | 1. AP–HP, IE3M, hôpital Pitié-Salpêtrière, department of endocrinology and reproductive medicine and Centre de référence des maladies endocriniennes rares de la croissance, centre de référence des pathologies gynécologiques Rares, ICAN, Paris, France;2. Sorbonne Université, Paris, France;1. Assistance Publique–Hôpitaux de Paris, Hôpital Saint-Antoine, Centre National de Référence des Pathologies Rares de l’Insulino–Sécrétion et de l’Insulino–Sensibilité (PRISIS) et Centre National de Référence des Maladies Endocriniennes Rares de la Croissance et du Développement (CRMERCD), Service d’Endocrinologie, Diabétologie et Endocrinologie de la Reproduction, Paris, France;2. Sorbonne Université, Inserm UMR_S 938, Centre de Recherche Saint-Antoine, Institut Hospitalo–Universitaire de Cardio–métabolisme et Nutrition (ICAN), Paris, France;1. Sorbonne Université, service d’endocrinologie, diabétologie et médecine de la reproduction, hôpital St-Antoine, AP–HP, centre de référence des maladies endocriniennes rares de la croissance et du développement, 75012 Paris, France;2. Université Paris Saclay, service d’endocrinologie de le reproduction, hôpital Bicêtre, AP-HP, 94275 Bicêtre, France;1. Department of Gynecology-Andrology, Cliniques Universitaires Saint-Luc, Brussels 1200, Belgium;2. Andrology Lab, Institut de Recherche Expérimentale et Clinique (IREC), Université Catholique de Louvain, Brussels 1200, Belgium |
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| Abstract: | 21-Hydroxylase deficiency (21OHD) is the most common cause of congenital adrenal hyperplasia. Increased production of adrenal-derived androgens and progesterone in 21OHD women interfere with their reproductive function and their fertility in many different ways, depending on the severity of the disease. Sexuality and fertility in women with classic 21OHD is impaired, due to several issues such as disrupted gonadotropic axis due to androgen and progesterone overproduction, and mechanical, psychological factors related to genital surgery. Fertility and fecundity in these women get better over the years. Subfertility seems contrariwise to be relative in non-classic 21OHD women. Before pregnancy, genotyping the partner and genetic counselling is mandatory. |
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| Keywords: | Congenital adrenal hyperplasia 21 hydroxylase deficiency Fertility Pregnancy |
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