Service d'endocrinologie diabète et maladies métaboliques, CHU de Caen, 14033 Caen cedex, France; Université de Caen, 14032 Caen cedex, France.
Service d'endocrinologie et diabétologie pédiatriques, hôpital des Enfants, CHU de Bordeaux, place Amélie-Raba-Léon, Bordeaux cedex, France.
Ann Endocrinol (Paris). 2018 Feb;79(1):1-22. doi: 10.1016/j.ando.2017.12.001. Epub 2018 Jan 12.
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.
法国内分泌学会（SFE）和法国儿科内分泌学会（DFSDP）根据 6 个工作组的 19 名专家对文献的分析，制定了成人和儿童原发性和继发性肾上腺功能不全的管理建议。除了原发性肾上腺功能不全观察到的色素沉着外，只有在存在许多非特异性症状的情况下才应怀疑存在肾上腺功能不全。诊断依赖于上午 8 点时血浆皮质醇和 ACTH 的测量值和/或促肾上腺皮质激素释放激素（synacthen）给药后的皮质醇增加。如果对继发性肾上腺功能不全仍有持续怀疑，应在成人、青少年和 2 岁以上的儿童中进行胰岛素低血糖试验。为了确定原发性肾上腺功能不全的原因，测量抗 21-羟化酶抗体是初始检测。如果自身抗体检测结果为阴性，应进行肾上腺 CT 扫描，然后建议在年轻男性中进行非常长链脂肪酸检测。在儿童中，通常会发现遗传异常，最常见的是先天性肾上腺皮质增生症。在孤立性促肾上腺皮质激素（ACTH）功能不全的情况下，建议首先消除皮质类固醇诱导的肾上腺功能不全，然后进行下丘脑-垂体 MRI。急性肾上腺功能不全是一种严重的疾病，胃肠道感染是最常见的起始因素。在进行皮质醇和 ACTH 测定的血液采样后，应通过给予氢化可的松琥珀酸钠进行治疗，并同时纠正低血糖和低血容量。需要对患者（在儿科患者的情况下为患者和/或其父母）进行教育并制定教育计划，以预防急性肾上腺危象。肾上腺功能不全的治疗基于使用尽可能低剂量的氢化可的松，每天多次给予。原发性肾上腺功能不全通常需要补充盐皮质激素，但对于促皮质素缺乏症则不需要。在某些情况下，可以提供脱氢表雄酮（DHEA）来替代雄激素。监测基于检测剂量不足和过量的迹象以及诊断相关的自身免疫性疾病。
