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婴儿西综合征患者在 ACTH 治疗期间发生医源性糖尿病。

Iatrogenic diabetes mellitus during ACTH therapy in an infant with West syndrome.

机构信息

Department of Pediatrics, University of Pavia and IRCCS Policlinico San Matteo Foundation, P.le Golgi n.2, 27100, Pavia, Italy.

Department of Emergency Medicine, Intensive Care Unit-Section 2, IRCCS Policlinico San Matteo Foundation, Pavia, Italy.

出版信息

Acta Diabetol. 2011 Dec;48(4):345-347. doi: 10.1007/s00592-011-0253-5. Epub 2011 Jan 21.

Abstract

West syndrome is a rare epileptic disease of infancy, typified by an association of characteristic spasms, hypsarrhythmia on electroencephalography and severe psychomotor retardation or deterioration. Adrenocorticotropic hormone (ACTH) is the current first-line therapy for West syndrome despite the fact that ACTH therapy is associated with various adverse effects. We describe a rare case of iatrogenic diabetes mellitus during ACTH therapy in a patient with symptomatic West syndrome. The infant had cushingoid facies, hirsutism and biochemical evidence of diabetes due to excessive glucocorticoid production with hyperplasia of both adrenal glands at ultrasound examination, without mineralocorticoid excess; in addition, he presented also short-term weight gain, marked electrolyte disturbances, hypokalemic alkalosis and infections. When ACTH is used to treat patients with West syndrome, it is necessary to follow glycemic levels until to the end of therapy.

摘要

婴儿痉挛症是一种罕见的癫痫疾病,其特征是存在特定的痉挛、脑电图出现高波幅失律和严重的精神运动发育迟缓或恶化。尽管促肾上腺皮质激素(ACTH)治疗会引起各种不良反应,但它仍是目前婴儿痉挛症的一线治疗药物。我们描述了一例症状性婴儿痉挛症患者在 ACTH 治疗期间发生医源性糖尿病的罕见病例。该婴儿出现库欣样面容、多毛症,且超声检查显示双侧肾上腺增生,导致糖皮质激素过度产生,存在生化证据提示糖尿病,而无盐皮质激素过多;此外,他还出现短期体重增加、明显电解质紊乱、低钾性碱中毒和感染。当使用 ACTH 治疗婴儿痉挛症患者时,有必要在整个治疗过程中监测血糖水平。

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