Program on Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892-1109, USA.
Curr Opin Endocrinol Diabetes Obes. 2010 Jun;17(3):217-23. doi: 10.1097/MED.0b013e328338f608.
Adrenal insufficiency, first codified in 1855 by Thomas Addison, remains relevant in 2010 because of its lethal nature.
Reports illuminate features of adrenal insufficiency cause, diagnosis and treatment, and the role of glucocorticoids in critical illness.
Progress has been made in identifying human leukocyte antigen and major histocompatability complex alleles that predispose to the development of adrenal insufficiency in patients with antibodies to 21-hydroxylase, but their role in clinical care is not established. Reports of HIV-associated infections and medication-induced hypocortisolism are reminders that autoimmune adrenal destruction does not underlie all cases. The diagnosis is adequately established by the 250 microg adrenocortocotropin hormone stimulation test in most patients; the 1 microg test carries the risk of misdiagnosis of healthy individuals as adrenally insufficient. Glucocorticoids provide life-saving treatment, but long-term quality of life is impaired, perhaps because therapy is not given in a physiologic way. The current recommended total daily dose is lower than that often prescribed. Dehydroepiandrosterone replacement may be useful in pubertal girls with hypopituitarism, but not in adults. Supraphysiologic hydrocortisone doses may aid in the reversal of septic shock independent of underlying adrenal function.
1855 年由托马斯·爱迪生首次确定的肾上腺功能不全,由于其致命性,在 2010 年仍具有重要意义。
报告阐明了肾上腺功能不全的病因、诊断和治疗的特点,以及糖皮质激素在危重病中的作用。
在确定人类白细胞抗原和主要组织相容性复合体等位基因方面取得了进展,这些等位基因易使抗 21-羟化酶抗体的患者发生肾上腺功能不全,但它们在临床护理中的作用尚未确定。HIV 相关感染和药物引起的皮质醇减少症的报告提醒人们,自身免疫性肾上腺破坏并非所有病例的基础。在大多数患者中,250μg 促肾上腺皮质激素刺激试验可充分确立诊断;1μg 试验有将健康个体误诊为肾上腺功能不全的风险。糖皮质激素提供救命治疗,但长期生活质量受损,这可能是因为治疗方式不具有生理性。目前推荐的总日剂量低于通常的处方剂量。脱氢表雄酮替代治疗可能对青春期女孩的垂体功能减退有用,但对成年人无效。超生理剂量的氢化可的松可能有助于逆转脓毒性休克,而与潜在的肾上腺功能无关。