School of Medicine, Sydney, The University of Notre Dame, 160 Oxford St, Darlinghurst, NSW, 2010, Australia.
Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, SA, Australia.
Endocrine. 2022 Jun;77(1):1-10. doi: 10.1007/s12020-022-03070-3. Epub 2022 May 18.
Review the literature concerning adrenal insufficiency (AI) and adrenal crisis (AC) in adolescents and young adults.
Searches of PubMed identifying relevant reports up to March 2022.
AI is rare disorder that requires lifelong glucocorticoid replacement therapy and is associated with substantial morbidity and occasional mortality among adolescents and young adults. Aetiologies in this age group are more commonly congenital, with acquired causes, resulting from tumours in the hypothalamic-pituitary area and autoimmune adrenalitis among others, increasing with age. All patients with AI are at risk of AC, which have an estimated incidence of 6 to 8 ACs/100 patient years. Prevention of ACs includes use of educational interventions to achieve competency in dose escalation and parenteral glucocorticoid administration during times of physiological stress, such as an intercurrent infection. While the incidence of AI/AC in young children and adults has been documented, there are few studies focussed on the AC occurrence in adolescents and young adults with AI. This is despite the range of developmental, psychosocial, and structural changes that can interfere with chronic disease management during this important period of growth and development.
In this review, we examine the current state of knowledge of AC epidemiology in emerging adults; examine the causes of ACs in this age group; and suggest areas for further investigation that are aimed at reducing the incidence and health impact of ACs in these patients.
回顾有关青少年和年轻成年人肾上腺功能不全（AI）和肾上腺危象（AC）的文献。
检索 PubMed 以确定截至 2022 年 3 月的相关报告。
AI 是一种罕见的疾病，需要终身糖皮质激素替代治疗，在青少年和年轻成年人中与相当大的发病率和偶尔的死亡率相关。该年龄段的病因更常见于先天性，后天性病因包括下丘脑-垂体区域的肿瘤和自身免疫性肾上腺炎等，随着年龄的增长而增加。所有 AI 患者都有发生 AC 的风险，估计每 100 名患者年有 6 至 8 例 AC。AC 的预防包括使用教育干预措施，以提高在生理应激期间（如并发感染）增加剂量和进行静脉内糖皮质激素给药的能力。虽然已经记录了幼儿和成年人 AI/AC 的发病率，但很少有研究关注 AI 青少年和年轻成年人的 AC 发生情况。尽管在此重要的生长和发育期间，存在多种发育、心理社会和结构变化会干扰慢性疾病管理，但仍存在这种情况。
在本次综述中，我们检查了青少年和年轻成年人 AC 流行病学的现有知识状态；检查了该年龄段 AC 发生的原因；并提出了进一步调查的领域，旨在降低这些患者 AC 的发病率和健康影响。
