The French endocrinology society (SFE) and the French pediatric endocrinology society (DFSDP) have drawn up recommendations for the management of primary and secondary adrenal insufficiency in the adult and child, based on an analysis of the literature by 19 experts in 6 work-groups. A diagnosis of adrenal insufficiency should be suspected in the presence of a number of non-specific symptoms except hyperpigmentation which is observed in primary adrenal insufficiency. Diagnosis rely on plasma cortisol and ACTH measurement at 8am and/or the cortisol increase after synacthen administration. When there is a persistant doubt of secondary adrenal insufficiency, insulin hypoglycemia test should be carried out in adults, adolescents and children older than 2 years. For determining the cause of primary adrenal insufficiency, measurement of anti-21-hydroxylase antibodies is the initial testing. An adrenal CT scan should be performed if auto-antibody tests are negative, then assay for very long chain fatty acids is recommended in young males. In children, a genetic anomaly is generally found, most often congenital adrenal hyperplasia. In the case of isolated corticotropin (ACTH) insufficiency, it is recommended to first eliminate corticosteroid-induced adrenal insufficiency, then perform an hypothalamic-pituitary MRI. Acute adrenal insufficiency is a serious condition, a gastrointestinal infection being the most frequently reported initiating factor. After blood sampling for cortisol and ACTH assay, treatment should be commenced by parenteral hydrocortisone hemisuccinate together with the correction of hypoglycemia and hypovolemia. Prevention of acute adrenal crisis requires an education of the patient and/or parent in the case of pediatric patients and the development of educational programs. Treatment of adrenal insufficiency is based on the use of hydrocortisone given at the lowest possible dose, administered several times per day. Mineralocorticoid replacement is often necessary for primary adrenal insufficiency but not for corticotroph deficiency. Androgen replacement by DHEA may be offered in certain conditions. Monitoring is based on the detection of signs of under- and over-dosage and on the diagnosis of associated auto-immune disorders.
Annales d'endocrinologie. 2018 Jan 12 [Epub]
Yves Reznik, Pascal Barat, Jérôme Bertherat, Claire Bouvattier, Frédéric Castinetti, Olivier Chabre, Philippe Chanson, Christine Cortet, Brigitte Delemer, Bernard Goichot, Damien Gruson, Laurence Guignat, Emmanuelle Proust-Lemoine, Marie-Laure Raffin Sanson, Rachel Reynaud, Dinane Samara Boustani, Dominique Simon, Antoine Tabarin, Delphine Zenaty
Service d'endocrinologie diabète et maladies métaboliques, CHU de Caen, 14033 Caen cedex, France; Université de Caen, 14032 Caen cedex, France. Electronic address: ., Service d'endocrinologie et diabétologie pédiatriques, hôpital des Enfants, CHU de Bordeaux, place Amélie-Raba-Léon, Bordeaux cedex, France., Service des maladies endocriniennes et métaboliques, hôpital Cochin, CHU Paris Centre, 75014 Paris, France., Service d'endocrinologie de l'enfant, hôpital de Bicêtre, GHU Paris Sud, Le Kremlin-Bicêtre, France., UMR7286, CNRS, CRN2M, service d'endocrinologie, hôpital La-Conception, Aix Marseille université, AP-HM, Marseille, France., Service d'endocrinologie diabétologie nutrition, CHU Grenoble-Alpes, 38043 Grenoble cedex, France., Service d'endocrinologie et des maladies de la reproduction, hôpital de Bicêtre, hôpitaux universitaires Paris-Sud, Assistance publique-Hôpitaux de Paris, 94275 Le Kremlin Bicêtre, France; Inserm 1185, faculté de médecine Paris Sud, université Paris-Sud, université Paris-Saclay, 94276 Le Kremlin-Bicêtre, France., Service d'endocrinologie, diabétologie et maladies métaboliques, CHRU de Lille, rue Polonowski, Lille cedex, France., Service d'endocrinologie - maladies métaboliques, hôpital Robert-Debré, CHU de Reims, avenue du Général-Koenig, 51092 Reims cedex, France., Service de médecine interne, endocrinologie et nutrition, hôpitaux universitaires de Strasbourg, 67098 Strasbourg cedex, France., Pôle de recherche en endocrinologie, diabète et nutrition, department of laboratory medicine, institut de recherche expérimentale et clinique, cliniques universitaires Saint-Luc, université catholique de Louvain, Brussels, Belgium., Polyclinique d'Aguilera, 21, rue de l'Estagnas, 64200 Biarritz, France., Service d'endocrinologie et nutrition, hôpital Ambroise-Paré, AP-HP, Boulogne-Billancourt, France; Inserm U1173, université Versailles-Saint-Quentin, Montigny-Le-Bretonneux, France., Service de pédiatrie multidisciplinaire, CHU Timone-Enfants, AP-HM, 13005 Marseille, France., Service d'endocrinologie, gynécologie et diabétologie pédiatrique, hôpital Necker, CHU de Paris, Paris, France., Service d'endocrinologie diabétologie pédiatrique, hôpital Robert-Debré, centre de référence des maladies endocriniennes rares de la croissance et du développement, université Paris Diderot, Assistance publique-Hôpitaux de Paris, 48, boulevard Sérurier, 75019 Paris, France., Service d'endocrinologie, diabète et nutrition, université de Bordeaux, CHU de Bordeaux, 33604 Pessac, France.