内科

目的对先天性肾上腺皮质增生症（CAH）合并中枢性性早熟进行临床分析，提高对CAH合并性早熟的认识。方法回顾分析我院2003年1月至2010年9月住院的所有CAH患者，挑选合并中枢性性早熟的患者，结合文献，对其临床表现、实验室检查和影像学检查等特点加以讨论。结果50例CAH患者中，4例合并中枢性性早熟，年龄4.1～7.7岁，骨龄9.5～12.0岁，骨龄提前3.9～5.4岁，均为21羟化酶缺乏，女性单纯男性化型。其中，2例为初诊，另2例分别发生在糖皮质激素抑制替代治疗后的9个月和12个月。结论对CAH患儿，尤其是骨龄明显提前者，应注意排查中枢性性早熟；在随后的糖皮质激素抑制替代治疗过程中，有部分患儿会出现性早熟，应定期密切随访。以便早诊、早治，及时调整治疗策略，改善终身高。

ObjectiveAimed to improve the understanding of congenital adrenal hyperplasia (CAH) complicated with central precocious puberty(CPP),we analysed the features of patients with CAH complicated with CPP.MethodsAll the patients with CAH complicated with CPP of our hospital,from 2003 January to 2010 September,were retrospectively analysed.Clinical features,laboratory examinations and imaging examination of the patients were discussed.Results4 out of 50 patients with CAH were diagnosed with CPP,both of them were 21-Hydroxylase Deficiency,and smiple virilizing form.Two patients were preliminary diagnosis,the other 2 patients were diagnosed after 9 months and 12 months of hydrocortisone therapy respectively.ConclusionsWe concluded that it is highly necessary to investigate CPP in CAH children,especially in those who have obvious skeletal advancement;some features will occur in the following hydrocortisone therapy,we should observe closely,so as to diagnosis,treatment early,adjust the treatment strategy,and promote final height of the patients.

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