先天性肾上腺皮质增生症并肾上腺危象患儿九例临床分析

目的分析先天性肾上腺皮质增生症（CAH）并肾上腺危象的临床特征。方法回顾性分析9例CAH并肾上腺危象的临床资料,并总结其临床特征。结果 9例中体重增长缓慢或不增6例、呕吐或腹泻8例、精神萎靡或抽搐5例,有家族遗传史3例,均伴有低血压、中度或重度脱水,血生化示低钠、低氯、高钾及代谢性酸中毒,促肾上腺皮质激素（ACTH）、睾酮（T）、孕酮（P）水平均升高,经补充电解质、糖皮质激素,纠正酸中毒,8例病情明显好转,1例抢救无效死亡。结论儿科医生应提高对CAH并肾上腺危象的诊治水平,避免漏诊、误诊。

Clinical analysis on 9 cases of congenital adrenal hyperplasia complicated with adrenal crisis

Objective：To analyze the clinical features of congenital adrenal hyperplasia（CAH） complicated with adrenal crisis.Methods：Analyze retrospectively 9 cases of CAH complicted with adrenal crisis,and summarise the clinical features.Results：6 cases of slow growth or no increase in weight in 9 cases,8 cases of vomiting or diarrhea,5 cases of apathetic or convulsant,3 cases have family history,all associated with low blood pressure,moderate or severe dehydration,blood biochemistry showed hyponatremia,low chlorine,hyperkalemia and metabolic acidosis,the level of adrenocorticotropic hormone（ACTH）,testosterone（T） and progesterone were high.By adding to electrolyte and glucocorticoid,correcting acidosis,the conditions of 8 cases improved significantly,1 died after rescue.Conclusion：The pediatrician should raise the level of diagnosis and treatment of CAH complicted with adrenal crisis,so as to avoid missed-diagnosis and misdiagnosis.