儿童肾上腺皮质功能减退症

Adrenal Insufficiency in Children

作者信息

Kilberg Marissa J., Vogiatzi Maria G.

机构信息

Assistant Professor of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, PA 19104

Professor of Pediatrics, Children’s Hospital of Philadelphia, Philadelphia, PA 19104

Abstract

Adrenal insufficiency (AI) is an uncommon but potentially life-threatening condition related to impaired secretion of cortisol by the adrenal gland. In general, this condition can be divided into primary (adrenal failure) and central (hypothalamic/pituitary) causes. In this chapter, we categorize the causes of adrenal insufficiency and systematically review the etiologies and associations to guide the laboratory evaluation and treatment, specifically as it pertains to the pediatric patient. Early diagnosis and treatment can prevent the development of adrenal crisis – prompt recognition can be lifesaving. Understanding the manifestation of unique types of adrenal insufficiency can guide management with glucocorticoid +/- mineralocorticoids and guide further investigation for associated disorders. We discuss the treatment of adrenal insufficiency, reviewing both the acute (crisis) and chronic management. For complete coverage of all related areas of Endocrinology, please visit our on-line FREE web-text, WWW.ENDOTEXT.ORG.

摘要

肾上腺功能不全（AI）是一种罕见但可能危及生命的疾病，与肾上腺皮质醇分泌受损有关。一般来说，这种疾病可分为原发性（肾上腺衰竭）和中枢性（下丘脑/垂体）病因。在本章中，我们对肾上腺功能不全的病因进行分类，并系统回顾其病因及关联，以指导实验室评估和治疗，特别是针对儿科患者。早期诊断和治疗可预防肾上腺危象的发生——及时识别可挽救生命。了解肾上腺功能不全独特类型的表现，可指导糖皮质激素±盐皮质激素的管理，并指导对相关疾病的进一步检查。我们讨论肾上腺功能不全的治疗，回顾急性（危象）和慢性管理。如需全面涵盖内分泌学的所有相关领域，请访问我们的在线免费网络文本，网址为WWW.ENDOTEXT.ORG。