Cortisol and Aldosterone Responses to Hypoglycemia and Na Depletion in Women With Non-Classic 21-Hydroxylase Deficiency
| Autor: | Laurence Pietri, Jean-François Gautier, H. Bry, Michel Azizi, Anne Blanchard, Julien Thomas, Antonin Lamaziere, Yves Le Bouc, Sylvie Salenave, Sylvie Brailly-Tabard, Sophie Christin-Maitre, Lise Duranteau, Bruno Donadille, Céline Piedvache, Aurélien Lorthioir, Peter Kamenický, Jacques Young, Philippe Chanson, Seray Genc, Marie-Laure Raffin-Sanson |
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| Přispěvatelé: | CIC - HEGP (CIC 1418), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPC) |
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
Hydrocortisone
Endocrinology Diabetes and Metabolism [SDV]Life Sciences [q-bio] Clinical Biochemistry Biochemistry chemistry.chemical_compound 0302 clinical medicine Endocrinology Prospective Studies Aldosterone 030219 obstetrics & reproductive medicine biology Incidence 21-Hydroxylase Middle Aged Prognosis Female France Pseudogenes Cortisol secretion Adult medicine.medical_specialty Adolescent food.diet 030209 endocrinology & metabolism Equivalence Trials as Topic Low sodium diet Hypoglycemia 03 medical and health sciences Young Adult food Internal medicine Renin–angiotensin system medicine Adrenal insufficiency Humans Adrenal Hyperplasia Congenital business.industry Biochemistry (medical) Insulin tolerance test Sodium medicine.disease chemistry Case-Control Studies biology.protein business Biomarkers Follow-Up Studies |
| Zdroj: | Journal of Clinical Endocrinology and Metabolism Journal of Clinical Endocrinology and Metabolism, Endocrine Society, 2020, 105 (1), pp.55-64. ⟨10.1210/clinem/dgz005⟩ |
| ISSN: | 0021-972X 1945-7197 |
| DOI: | 10.1210/clinem/dgz005⟩ |
| Popis: | Background Non-classic 21-hydroxylase deficiency is usually diagnosed in post-pubertal women because of androgen excess. Indication of systematic steroid replacement therapy is controversial because the risk of acute adrenal insufficiency is unknown. In order to specify this risk we evaluated the cortisol and aldosterone secretions in response to appropriate pharmacologic challenges. Methods In this prospective case–control non-inferiority study we investigated 20 women with non-classic 21-hydroxylase deficiency carrying biallelic CYP21A2 mutations and with serum 17-hydroxyprogesterone (17OHP) >10 ng/mL after stimulation with Synacthen® (tetracosactrin) and 20 age- and body mass index-matched healthy women with 17OHP after Synacthen® Findings The peak serum cortisol concentration after insulin hypoglycemia was lower in patients than in controls (mean difference –47 ng/mL, 90% CI, –66, P = 0.0026). A peak serum cortisol above a cutoff value of 170 ng/mL was obtained in all controls but only in 55% of patients (P = 0.0039). Twenty-four hours after sodium depletion, blood pressure, plasma sodium, potassium, and serum aldosterone concentrations were comparable between the two groups, but patients had higher stimulated renin concentrations than controls (P = 0.0044). Interpretation Patients with non-classic 21-hydroxylase deficiency frequently display partial cortisol insufficiency and compensated defect in aldosterone secretion. Their clinical management should systematically include assessment of adrenal functions. |
| Databáze: | OpenAIRE |
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