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先天性肾上腺皮质增生症失盐型的围手术期替代治疗

[Perioperative substitution therapy in congenital adrenogenital syndrome with salt loss].

作者信息

Abel M, von Petrykowski W

出版信息

Anaesthesist. 1984 Aug;33(8):374-6.

PMID:6486393
Abstract

Patients with an inborn error of adrenal steroid synthesis suffer from primary adrenocortical insufficiency requiring corticosteroid replacement therapy and appropriate stress coverage. The case report of a two year old infant with congenital adrenal hyperplasia of the salt-losing type demonstrates the consequences of an accidental steroid underdosage. The necessity of increased cortisol replacement therapy in case of anesthetic procedures--even of short duration and without surgical measures--is underlined. The details of adequate perioperative steroid and i.v. fluid therapy in pediatric patients with congenital adrenal hyperplasia of the salt-losing type are discussed.

摘要

肾上腺类固醇合成先天性缺陷的患者患有原发性肾上腺皮质功能不全,需要进行皮质类固醇替代治疗并给予适当的应激处理。一名两岁失盐型先天性肾上腺皮质增生症婴儿的病例报告显示了意外类固醇剂量不足的后果。强调了在进行麻醉操作时(即使是短时间且无手术措施)增加皮质醇替代治疗的必要性。本文还讨论了失盐型先天性肾上腺皮质增生症儿科患者围手术期适当的类固醇和静脉输液治疗细节。

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