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Bothou, Christina; Anand, Gurpreet; Li, Dingfeng; Kienitz, Tina; Seejore, Khyatisha; Simeoli, Chiara; Ebbehoj, Andreas; Ward, Emma G.; Paragliola, Rosa Maria; Ferrigno, Rosario; Badenhoop, Klaus; Bensing, Sophie; Oksnes, Marianne; Esposito, Daniela; Bergthorsdottir, Ragnhildur; Drake, William; Wahlberg, Jeanette; Reisch, Nicole; Hahner, Stefanie; Pearce, Simon; Trainer, Peter; Etzrodt-Walter, Gwendolin; Thalmann, Sebastien P.; Saevik, Ase B.; Husebye, Eystein; Isidori, Andrea M.; Falhammar, Henrik; Meyer, Gesine; Corsello, Salvatore M.; Pivonello, Rosario; Murray, Robert; Bancos, Irina; Quinkler, Marcus und Beuschlein, Felix (2020): Current Management and Outcome of Pregnancies in Women With Adrenal Insufficiency: Experience from a Multicenter Survey. In: Journal of Clinical Endocrinology & Metabolism, Bd. 105, Nr. 8, E2853-E2863

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Abstract

Context: Appropriate management of adrenal insufficiency (Al) in pregnancy can be challenging due to the rarity of the disease and lack of evidence-based recommendations to guide glucocorticoid and mineralocorticoid dosage adjustment. Objective: Multicenter survey on current clinical approaches in managing Al during pregnancy. Design: Retrospective anonymized data collection from 19 international centers from 2013 to 2019. Setting and Patients: 128 pregnancies in 113 women with different causes of Al: Addison disease (44%), secondary Al (25%), congenital adrenal hyperplasia (25%), and acquired Al due to bilateral adrenalectomy (6%). Results: Hydrocortisone (HC) was the most commonly used glucocorticoid in 83% (97/117) of pregnancies. Glucocorticoid dosage was increased at any time during pregnancy in 73/128 (57%) of cases. In these cases, the difference in the daily dose of HC equivalent between baseline and the third trimester was 8.6 +/- 5.4 (range 1-30) mg. Fludrocortisone dosage was increased in fewer cases (7/54 during the first trimester, 9/64 during the second trimester, and 9/62 cases during the third trimester). Overall, an adrenal crisis was reported in 9/128 (7%) pregnancies. Cesarean section was the most frequent mode of delivery at 58% (69/118). Fetal complications were reported in 3/120 (3%) and minor maternal complications in 15/120 (13%) pregnancies without fatal outcomes. Conclusions: This survey confirms good maternal and fetal outcome in women with Al managed in specialized endocrine centers. An emphasis on careful endocrine follow-up and repeated patient education is likely to have reduced the risk of adrenal crisis and resulted in positive outcomes.

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