SFE/SFEDP adrenal insufficiency French consensus: Introduction and handbook

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
Annales d'Endocrinologie. 2018-02-01; 79(1): 1-22
DOI: 10.1016/j.ando.2017.12.001

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1. Ann Endocrinol (Paris). 2018 Feb;79(1):1-22. doi: 10.1016/j.ando.2017.12.001.
Epub 2018 Jan 12.

SFE/SFEDP adrenal insufficiency French consensus: Introduction and handbook.

Reznik Y(1), Barat P(2), Bertherat J(3), Bouvattier C(4), Castinetti F(5), Chabre
O(6), Chanson P(7), Cortet C(8), Delemer B(9), Goichot B(10), Gruson D(11),
Guignat L(3), Proust-Lemoine E(12), Sanson MR(13), Reynaud R(14), Boustani
DS(15), Simon D(16), Tabarin A(17), Zenaty D(16).

Author information:
(1)Service d’endocrinologie diabète et maladies métaboliques, CHU de Caen, 14033
Caen cedex, France; Université de Caen, 14032 Caen cedex, France. Electronic
address: .
(2)Service d’endocrinologie et diabétologie pédiatriques, hôpital des Enfants,
CHU de Bordeaux, place Amélie-Raba-Léon, Bordeaux cedex, France.
(3)Service des maladies endocriniennes et métaboliques, hôpital Cochin, CHU Paris
Centre, 75014 Paris, France.
(4)Service d’endocrinologie de l’enfant, hôpital de Bicêtre, GHU Paris Sud, Le
Kremlin-Bicêtre, France.
(5)UMR7286, CNRS, CRN2M, service d’endocrinologie, hôpital La-Conception, Aix
Marseille université, AP-HM, Marseille, France.
(6)Service d’endocrinologie diabétologie nutrition, CHU Grenoble-Alpes, 38043
Grenoble cedex, France.
(7)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.
(8)Service d’endocrinologie, diabétologie et maladies métaboliques, CHRU de
Lille, rue Polonowski, Lille cedex, France.
(9)Service d’endocrinologie – maladies métaboliques, hôpital Robert-Debré, CHU de
Reims, avenue du Général-Koenig, 51092 Reims cedex, France.
(10)Service de médecine interne, endocrinologie et nutrition, hôpitaux
universitaires de Strasbourg, 67098 Strasbourg cedex, France.
(11)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.
(12)Polyclinique d’Aguilera, 21, rue de l’Estagnas, 64200 Biarritz, France.
(13)Service d’endocrinologie et nutrition, hôpital Ambroise-Paré, AP-HP,
Boulogne-Billancourt, France; Inserm U1173, université Versailles-Saint-Quentin,
Montigny-Le-Bretonneux, France.
(14)Service de pédiatrie multidisciplinaire, CHU Timone-Enfants, AP-HM, 13005
Marseille, France.
(15)Service d’endocrinologie, gynécologie et diabétologie pédiatrique, hôpital
Necker, CHU de Paris, Paris, France.
(16)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.
(17)Service d’endocrinologie, diabète et nutrition, université de Bordeaux, CHU
de Bordeaux, 33604 Pessac, France.

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.

Copyright © 2017 Elsevier Masson SAS. All rights reserved.

DOI: 10.1016/j.ando.2017.12.001
PMID: 29338844 [Indexed for MEDLINE]

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