Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular (LIM42), Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brasil.
Arch Endocrinol Metab. 2021 Nov 3;65(4):488-494. doi: 10.20945/2359-3997000000392. Epub 2021 Jul 16.
Primary adrenal insufficiency (PAI) is characterized by the inability of the adrenal cortex to produce sufficient amounts of glucocorticoids and/or mineralocorticoids. Addison's disease (AD) and congenital adrenal hyperplasia (CAH) are the most frequent disorders in adults and children, respectively. Despite the diagnostic advances and the availability of glucocorticoid and mineralocorticoid replacements, adrenal crisis (AC) is still a potentially lethal condition contributing to the increased mortality, not only during the first year of life, but also throughout life. Failure in increasing glucocorticoid doses during acute stress, when greater amounts of glucocorticoids are required, can lead to AC and an increase morbimortality rate of PAI. Considering a mortality rate of 0.5 per 100 patient years, up to 1,500 deaths from AC are expected in Brazil in the coming decade, which represents an alarming situation. The major clinical features are hypotension and volume depletion. Nonspecific symptoms such as fatigue, lack of energy, anorexia, nausea, vomiting, and abdominal pain are common. The main precipitating factors are gastrointestinal diseases, other infectious disease, stressful events (e.g., major pain, surgery, strenuous physical activity, heat, and pregnancy), and withdrawal of glucocorticoid therapy. Suspected AC requires immediate therapeutic action with intravenous (iv) hydrocortisone, fluid infusion, monitoring support, and antibiotics if necessary. AC is best prevented through patient education, precocious identification and by adjusting the glucocorticoid dosage in stressor situations. The emergency card, warning about acute glucocorticoid replacement, has high value in reducing the morbidity and mortality of AC.
原发性肾上腺功能不全（PAI）的特征是肾上腺皮质无法产生足够量的糖皮质激素和/或盐皮质激素。艾迪生病（AD）和先天性肾上腺增生症（CAH）分别是成人和儿童中最常见的疾病。尽管诊断技术有所进步，糖皮质激素和盐皮质激素替代治疗也已普及，但肾上腺危象（AC）仍然是一种潜在的致命病症，会导致死亡率增加，不仅在生命的头一年，而且在整个生命过程中都是如此。在急性应激期间，当需要更多的糖皮质激素时，如果不能增加糖皮质激素剂量，可能会导致 AC 并增加 PAI 的发病率和死亡率。考虑到每 100 名患者年的死亡率为 0.5，预计未来十年巴西将有多达 1500 人死于 AC，这是一个令人震惊的情况。主要的临床特征是低血压和容量不足。疲劳、乏力、食欲不振、恶心、呕吐和腹痛等非特异性症状很常见。主要的诱发因素是胃肠道疾病、其他传染病、应激事件（如剧烈疼痛、手术、剧烈体力活动、高温和妊娠）以及糖皮质激素治疗的停药。疑似 AC 需要立即采取静脉（iv）氢化可的松、补液、监测支持和必要时使用抗生素等治疗措施。通过患者教育、早期识别和在应激情况下调整糖皮质激素剂量，可以最好地预防 AC。紧急情况下的卡片，即关于急性糖皮质激素替代的警告，对于降低 AC 的发病率和死亡率具有很高的价值。
