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肾上腺危象的治疗与预防及家庭教育

Treatment and Prevention of Adrenal Crisis and Family Education.

作者信息

Çamtosun Emine, Sangün Özlem

机构信息

İnönü University Faculty of Medicine, Department of Pediatric Endocrinology, Malatya, Turkey

Başkent University Faculty of Medicine, Department of Pediatric Endocrinology, Adana, Turkey

出版信息

J Clin Res Pediatr Endocrinol. 2025 Jan 10;17(Suppl 1):80-92. doi: 10.4274/jcrpe.galenos.2024.2024-6-12-S. Epub 2024 Dec 23.

DOI:10.4274/jcrpe.galenos.2024.2024-6-12-S
PMID:39713905
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11730097/
Abstract

Adrenal crisis is a life threatening complication of adrenal insufficiency (AI). Its treatment is urgent and parenteral hydrocortisone (HC) should be given at 10-15 times physiological doses in this situation. If HC is not available, alternatively prednisolone or methyl prednisolone may be used. In cases where peripheral venous access cannot be achieved quickly, intramuscular (IM) administration should be performed without delay. Fluid deficit, hypoglycemia, hyponatremia and hyperkalemia should be evaluated and corrected. Stressful conditions, such as physical stress, accidents, injuries, surgical interventions and anesthesia increase the need for cortisol and may lead the development of adrenal crisis. In order to prevent adrenal crisis, glucocorticoid dose should be increased according to the magnitude and severity of the stress situation as described in this review. Patients’ and/or their families’ education may improve the management of AI and reduce the frequency of adrenal crisis and/or mortality. They should be trained about conditions leading to adrenal crisis, how to increase the glucocorticoid dose in stress situations, recognizing signs of adrenal crisis and using IM HC if it is needed. All patients should be encouraged to carry a card/information sheet/medical alert bracelet or necklace indicating the diagnosis of AI and need for HC administration. It is useful for patients and parents to have an emergency glucocorticoid injection kit and to receive self-injection training.

摘要

肾上腺危象是肾上腺皮质功能减退症(AI)的一种危及生命的并发症。其治疗刻不容缓,在此情况下应给予静脉注射氢化可的松(HC),剂量为生理剂量的10 - 15倍。若无法获得HC,也可使用泼尼松龙或甲泼尼龙。若无法迅速建立外周静脉通路,应立即进行肌肉注射(IM)。应评估并纠正液体缺失、低血糖、低钠血症和高钾血症。身体应激、事故、损伤、手术干预和麻醉等应激状态会增加对皮质醇的需求,并可能导致肾上腺危象的发生。为预防肾上腺危象,应如本综述所述,根据应激情况的严重程度增加糖皮质激素剂量。对患者和/或其家属进行教育可能会改善AI的管理,并降低肾上腺危象的发生率和/或死亡率。应培训他们了解导致肾上腺危象的情况、如何在应激状态下增加糖皮质激素剂量、识别肾上腺危象的体征以及在需要时使用肌肉注射HC。应鼓励所有患者携带卡片/信息表/医疗警示手环或项链,标明患有AI以及需要注射HC。为患者和家长提供应急糖皮质激素注射套件并进行自我注射培训是有用的。

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本文引用的文献

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Clinical, Biochemical and Molecular Characteristics of Congenital Adrenal Hyperplasia Due to 21-hydroxylase Deficiency.21-羟化酶缺乏所致先天性肾上腺皮质增生症的临床、生化及分子特征
J Clin Res Pediatr Endocrinol. 2025 Jan 10;17(Suppl 1):3-11. doi: 10.4274/jcrpe.galenos.2024.2024-6-6-S. Epub 2024 Dec 23.
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Perioperative glucocorticoid management based on current evidence.基于现有证据的围手术期糖皮质激素管理。
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8
Prevention of Adrenal Crisis: Cortisol Responses to Major Stress Compared to Stress Dose Hydrocortisone Delivery.预防肾上腺危象:大应激与应激剂量氢化可的松给药后皮质醇反应的比较。
J Clin Endocrinol Metab. 2020 Jul 1;105(7):2262-74. doi: 10.1210/clinem/dgaa133.
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Guidelines for the management of glucocorticoids during the peri-operative period for patients with adrenal insufficiency: Guidelines from the Association of Anaesthetists, the Royal College of Physicians and the Society for Endocrinology UK.肾上腺皮质功能减退症患者围手术期糖皮质激素管理指南:麻醉医师协会、皇家内科医师学会和英国内分泌学会指南。
Anaesthesia. 2020 May;75(5):654-663. doi: 10.1111/anae.14963. Epub 2020 Feb 3.
10
Stress-Dosed Glucocorticoids and Mineralocorticoids Before Intensive Endurance Exercise in Primary Adrenal Insufficiency.原发性肾上腺功能不全患者进行大强度耐力运动前应激剂量糖皮质激素和盐皮质激素的应用
Clin J Sport Med. 2019 Nov;29(6):e73-e75. doi: 10.1097/JSM.0000000000000540.