Husebye E S, Aanderud S
Medisinsk avdeling Haukeland Sykehus, Bergen.
Tidsskr Nor Laegeforen. 1998 Feb 10;118(4):542-4.
Primary adrenocortical insufficiency (Addison's disease) is characterised by weakness, tiredness, fatigue, weight loss, hypotension, hyperpigmentation and a craving for salt. Without treatment lethality is 100%; correctly treated, life expectancy is normal. Addison's disease may appear isolated or as part of a polyendocrine syndrome. Because several of the symptoms are unspecific and develop over the course of several years, many patients are not diagnosed before a life-threatening adrenal crisis develops. Autoimmune destruction of the adrenal cortex is the main cause of adrenocortical failure in the industrialised world. This condition is characterised by circulating autoantibodies against the steroidogenic enzyme 21-hydroxylase. These autoantibodies can now easily be quantified. More unusual causes of adrenocortical failure are tuberculosis, bleeding, metastasis and adrenoleukodystrophy. Using three cases we highlight the clinical, diagnostic and therapeutic aspects of adrenocortical failure.
原发性肾上腺皮质功能减退症（艾迪生病）的特征为虚弱、疲倦、乏力、体重减轻、低血压、色素沉着以及嗜盐。若不治疗，致死率为100%；经正确治疗，预期寿命正常。艾迪生病可能单独出现，也可能是多内分泌综合征的一部分。由于其中一些症状不具特异性且会在数年内逐渐出现，许多患者在危及生命的肾上腺危象发生之前未被诊断出来。在工业化国家，肾上腺皮质自身免疫性破坏是肾上腺皮质功能衰竭的主要原因。这种情况的特征是存在针对类固醇生成酶21 - 羟化酶的循环自身抗体。现在这些自身抗体能够轻易地进行定量检测。肾上腺皮质功能衰竭更不常见的病因包括结核病、出血、转移瘤和肾上腺脑白质营养不良。我们通过三个病例突出了肾上腺皮质功能衰竭的临床、诊断和治疗方面。
