Service d'endocrinologie et des maladies de la reproduction, Assistance publique-Hôpitaux de Paris, hôpitaux universitaires Paris-Sud, hôpital de Bicêtre, 94275 Le Kremlin-Bicêtre, France; Inserm 1185, faculté de médecine Paris Sud, université Paris-Sud, université Paris-Saclay, 94276 Le Kremlin-Bicêtre, France.
Service des maladies endocriniennes et métaboliques, CHU Paris Centre, hôpital Cochin, 75014 Paris, France.
Ann Endocrinol (Paris). 2017 Dec;78(6):495-511. doi: 10.1016/j.ando.2017.10.005. Epub 2017 Nov 23.
A diagnosis of adrenal insufficiency should be suspected in the presence of a number of non-specific symptoms (fatigue, anorexia, weight loss, hypotension, hyponatremia and hyperkalemia amongst adrenal causes of insufficiency). The diagnosis should be considered in case of pituitary disease or a state of shock. Treatment should be commenced immediately without waiting for confirmation from biochemical tests, which rely on cortisol level at 8am (expected to be low) and on ACTH level (expected to be high in the case of primary adrenal insufficiency). If these tests are inconclusive, a Synacthen test should be carried out. The threshold limits are provided as a guide. Low plasma cortisol and normal to low plasma ACTH indicates a pituitary origin for the deficiency. In this situation, the Synacthen test can give a false normal result, and if this adrenal insufficiency is strongly suspected, an insulin hypoglycemia test or metyrapone (Metopirone) test should be carried out. In children younger than 2yr, hypoglycemia, dehydration and convulsions are frequently observed and in young girls, virilization is suspect of congenital adrenal hyperplasia . The circadian rhythm of cortisol is not present until after 4months of age and the Synacthen test is the only one that is feasible. In children older than 2yrs, the signs and diagnostic methods are the same as in the adult. Cessation of corticosteroid treatment is a frequent circumstance however there is little published data and no evidence for definitive guidelines. After ceasing a short period of corticosteroid treatment, patient education is all that is required. After longer treatment, consensus leaves the choice up to the physician, between educating the patient and prescribing hydrocortisone in case of stress, or prescribing low daily dose hydrocortisone and evaluating the ACTH axis over time until normal function is recovered.
当存在许多非特异性症状（疲劳、厌食、体重减轻、低血压、低钠血症和高钾血症等肾上腺功能不全的原因）时，应怀疑存在肾上腺功能不全。如果存在垂体疾病或休克状态，则应考虑诊断。应立即开始治疗，无需等待生化测试结果确认，这些测试依赖于 8 点的皮质醇水平（预计较低）和 ACTH 水平（原发性肾上腺功能不全时预计较高）。如果这些测试没有结论，可以进行 Synacthen 测试。阈值限制仅供参考。低血浆皮质醇和正常至低血浆 ACTH 表明缺陷的垂体来源。在这种情况下，Synacthen 测试可能会得出假正常结果，如果强烈怀疑存在这种肾上腺功能不全，则应进行胰岛素低血糖测试或米托坦（Metopirone）测试。在 2 岁以下的儿童中，经常观察到低血糖、脱水和惊厥，而在年轻女孩中，先天性肾上腺增生可能怀疑存在性早熟。皮质醇的昼夜节律直到 4 个月后才出现，并且 Synacthen 测试是唯一可行的测试。在 2 岁以上的儿童中，症状和诊断方法与成人相同。然而，皮质类固醇治疗的停止是一种常见情况，但是发表的数据很少，没有明确的指南证据。停止短期皮质类固醇治疗后，仅需要对患者进行教育。在长期治疗后，共识让医生在教育患者和在应激时开 hydrocortisone 之间做出选择，或者开低剂量的 daily dose hydrocortisone 并随时间评估 ACTH 轴，直到恢复正常功能。
