8例原发性色素性结节状肾上腺皮质病的临床分析

目的总结原发性色素性结节状肾上腺皮质病(PPNAD)临床特点。方法回顾分析北京协和医院2001年~2009年所诊治的PPNAD患者8例,采集患者临床资料。结果PPNAD好发于青少年,62.5%伴有Carney综合征(CNC)。在临床表现中,闭经及身高增长停滞或减慢较为明显,分别占100%和62.5%。血ACTH检测不到,血F节律消失,50%的患者地塞米松抑制实验为反常性升高。75%的患者双侧肾上腺未见明显异常或仅有可疑小结节样改变。肾上腺病理可见色素沉积的结节样改变。单侧肾上腺全切术可缓解症状,但血ACTH和血F节律难以恢复,可能复发。结论对于影像学检查未见肾上腺明显病变的ACTH非依赖性库欣综合征的患者,要警惕PPNAD,同时要注意筛查及随诊有无合并CNC。

Clinical analysis of 8 cases of primary pigmented nodular adrenocortical disease

Objective To summarize the clinical characteristics of primary pigmented nodular adrenocortical disease(PPNAD).Methods Eight patients with PPNAD from 2001 to 2009 in PUMC Hospital were reviewed,their clinical data were collected.Results PPNAD often occurred in adolescents.62.5% of patients with PPNAD were complicated with Carney complex(CNC).In addition to general features of Cushing's syndrome,amenorrhea and growth retardation in stature were very frequent in clinical manifestations of PPNAD.Plasma ACTH wa...