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[先天性肾上腺皮质增生症的临床研究]

[A clinical study of congenital adrenal hyperplasia].

作者信息

Nanbu A, Kumamoto Y, Tachiki H, Itoh N, Mikuma N, Tsunekawa T, Takagi Y, Maruta H

机构信息

Department of Urology, Sapporo Medical College.

出版信息

Hinyokika Kiyo. 1989 Nov;35(11):1831-7.

PMID:2559616
Abstract

We have compared three treatments of congenital adrenal hyperplasia (CAH) for their effect on physical development. Thirteen girls and two boys with CAH due to 21-hydroxylase deficiency were treated with three different treatments, hydrocortisone (HC), dexamethasone (DXM) and cyproterone acetate (CA). The results showed that height growth was better with HC and CA than DXM, and bone excessive maturation was more suppressed with DXM and CA than HC. A dose-dependent relationship was revealed between body weight and dose of HC. Iatrogenic obesity was found in 42.9% and 38.1% of the patients treated with DXM and HC, but none of the patients treated with CA did became obese. Physical growth was better with CA treatment than HC or DXM treatment, but CA may have a suppressive effect on the pituitary-adrenal axis observed carefully, especially on prepubertal and pubertal cases.

摘要

我们比较了三种先天性肾上腺皮质增生症(CAH)治疗方法对身体发育的影响。13名因21-羟化酶缺乏导致CAH的女孩和2名男孩接受了三种不同治疗,即氢化可的松(HC)、地塞米松(DXM)和醋酸环丙孕酮(CA)。结果显示,HC和CA治疗组的身高增长优于DXM治疗组,而DXM和CA治疗组对骨骼过度成熟的抑制作用强于HC治疗组。体重与HC剂量之间呈现剂量依赖性关系。接受DXM和HC治疗的患者中,分别有42.9%和38.1%出现医源性肥胖,但接受CA治疗的患者均未出现肥胖。CA治疗组的身体生长情况优于HC或DXM治疗组,但仔细观察发现,CA可能对垂体-肾上腺轴有抑制作用,尤其是对青春期前和青春期病例。

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