Division of Pediatric Endocrinology, Department of Pediatrics, Medical School of Patras, University Hospital, Rio, Patras, Greece.
J Pediatr Endocrinol Metab. 2023 May 25;36(7):700-703. doi: 10.1515/jpem-2023-0080. Print 2023 Jul 26.
Isolated ACTH deficiency (IAD) is defined as an impaired secretion of ACTH from the pituitary gland without any other anterior pituitary hormonal deficits. The idiopathic form of IAD has been described mainly in adults and is thought to be caused by an autoimmune mechanism.
Herein, we present an 11-year-old _prepubertal previously healthy boy, who suffered a severe hypoglycemic episode short after the initiation of thyroxine for autoimmune thyroiditis and was finally diagnosed with secondary adrenal failure due to idiopathic IAD, after all other etiologies were excluded, thought an extensive diagnostic work-up.
Idiopathic IAD is a rare entity of adrenal insufficiency in pediatrics that should be considered as an etiology of secondary adrenal failure in children, when clinical signs of glucocorticoid deficiency are present and other causes are excluded.
孤立性 ACTH 缺乏症（IAD）定义为垂体分泌 ACTH 受损，而无其他前垂体激素缺乏。特发性 IAD 主要在成人中描述，被认为是由自身免疫机制引起的。
本文介绍了 11 岁的 _ 青春期前健康男孩，在开始甲状腺素治疗自身免疫性甲状腺炎后不久，发生严重低血糖发作，最终在排除所有其他病因后，通过广泛的诊断检查，被诊断为特发性 IAD 导致的继发性肾上腺功能衰竭。
特发性 IAD 是儿科肾上腺功能不全的罕见疾病，当存在糖皮质激素缺乏的临床症状且排除其他病因时，应将其视为儿童继发性肾上腺功能衰竭的病因。
