Mytinger John R, Bowden Sasigarn A
Department of Pediatrics, Division of Pediatric Neurology, Nationwide Children's Hospital, The Ohio State University , Columbus, OH , USA.
Department of Pediatrics, Division of Pediatric Endocrinology, Nationwide Children's Hospital, The Ohio State University , Columbus, OH , USA.
Front Neurol. 2015 Dec 8;6:259. doi: 10.3389/fneur.2015.00259. eCollection 2015.
Prednisolone and adrenocorticotropic hormone (ACTH) are "hormone" therapies for infantile spasms. There is limited data on the occurrence of decreased adrenal reserve or signs of clinical adrenal insufficiency after hormone therapy. This is a retrospective medical record review of patients referred to our Infantile Spasms Program. Our standardized infantile spasms management guideline began in September 2012 and initially included a post-hormone laboratory assessment of adrenal function. Medical records were assessed for hormone treatments, adrenal function testing, and signs of adrenal insufficiency. Forty-two patients who received one or both hormone therapies met inclusion criteria. A post-hormone laboratory assessment of adrenal function was done in 14 patients. Of these 14 patients, 2 had an abnormal laboratory assessment of adrenal function, both by adrenal stimulation testing - one after ACTH and one after prednisolone. One patient received hydrocortisone replacement and the other received stress dose hydrocortisone as needed; neither patient developed signs of adrenal insufficiency. Another patient treated with both types of hormone therapy in tandem, who did not have a post-hormone laboratory assessment, developed signs of mild adrenal insufficiency and required replacement hydrocortisone. Our study suggests that adrenal suppression can occur after modern hormone therapy regimens. We found two patients with abnormal adrenal function testing after hormone therapy and another patient with signs adrenal insufficiency. Given the seriousness of adrenal crisis, caregiver education on the signs of adrenal insufficiency is critical. Greater vigilance may be indicated in patients receiving both types of hormone therapy in tandem. Although a routine post-hormone laboratory assessment of adrenal function may not be feasible in all patients, replacement or stress dose hydrocortisone is necessary for all patients with suspected adrenal insufficiency.
泼尼松龙和促肾上腺皮质激素（ACTH）是用于婴儿痉挛症的“激素”疗法。关于激素治疗后肾上腺储备减少或临床肾上腺功能不全体征发生情况的数据有限。这是一项对转诊至我们婴儿痉挛症项目的患者进行的回顾性病历审查。我们标准化的婴儿痉挛症管理指南于2012年9月开始实施，最初包括激素治疗后的肾上腺功能实验室评估。对病历进行了激素治疗、肾上腺功能测试和肾上腺功能不全体征的评估。42例接受一种或两种激素治疗的患者符合纳入标准。14例患者进行了激素治疗后的肾上腺功能实验室评估。在这14例患者中，2例肾上腺功能实验室评估异常，均通过肾上腺刺激试验得出——1例在ACTH治疗后，1例在泼尼松龙治疗后。1例患者接受了氢化可的松替代治疗，另1例根据需要接受了应激剂量的氢化可的松治疗；这两名患者均未出现肾上腺功能不全的体征。另一名同时接受两种激素治疗但未进行激素治疗后实验室评估的患者出现了轻度肾上腺功能不全的体征，需要氢化可的松替代治疗。我们的研究表明，现代激素治疗方案后可能会发生肾上腺抑制。我们发现2例患者在激素治疗后肾上腺功能测试异常，另1例患者有肾上腺功能不全的体征。鉴于肾上腺危象的严重性，对护理人员进行肾上腺功能不全体征的教育至关重要。对于同时接受两种激素治疗的患者，可能需要更高的警惕性。尽管对所有患者进行常规的激素治疗后肾上腺功能实验室评估可能不可行，但对于所有疑似肾上腺功能不全的患者，氢化可的松替代治疗或应激剂量治疗是必要的。
