良性肾上腺源性库欣综合征临床特点比较分析
Comparison and analysis of clinical features of benign adrenal Cushing syndrome
苗新宇 1林璐 2李乐乐 3窦京涛 4陈康 4吕朝晖 4巴建明 4裴育 4谷伟军 4杜锦 4郭清华 4王先令 4臧丽 4李一君 4闫文华 4李春霖 5母义明4
作者信息
- 1. 中国人民解放军总医院第一医学中心内分泌科,北京 100853;中国人民解放军总医院第二医学中心内分泌科国家老年疾病临床医学研究中心,北京100853
- 2. 中国人民解放军总医院第一医学中心内分泌科,北京 100853;海南省人民医院内分泌科,海南 海口 570311
- 3. 首都医科大学附属北京儿童医院内分泌遗传代谢科国家儿童医学中心,北京 100045
- 4. 中国人民解放军总医院第一医学中心内分泌科,北京 100853
- 5. 中国人民解放军总医院第二医学中心内分泌科国家老年疾病临床医学研究中心,北京100853
- 折叠
摘要
目的 比较分析具有显性库欣综合征临床表现的肾上腺皮质腺瘤(ADA)、双侧肾上腺大结节增生(BMAH)和原发性色素性结节样肾上腺皮质病(PPNAD)患者临床特点,为临床鉴别诊断提供依据.方法 回顾性分析2008-2020年于解放军总医院住院明确诊断为ADA患者86例、BMAH患者23例、PPNAD患者7例的临床资料.结果 (1)三组患者BMAH组年龄最大,平均(51.7±9.5)岁,ADA次之,PPNAD组就诊年龄最小,平均(19.9±5.7)岁.ADA、PPNAD组患者女性居多,BMAH患者男女比例相当.(2)临床表现方面,PPNAD组紫纹最多(71.4％),多血质、瘀斑、满月脸、向心性肥胖及皮肤变薄等方面差异无统计学意义.(3)合并症方面,BMAH组糖代谢异常和血脂紊乱患者最多(分别为87.0％、68.2％),三组患者均合并较高比例的骨质疏松或低骨量(＞75％).BMAH组收缩压、糖化血红蛋白、血甘油三酯及血钠水平更高(P＜0.05);三组患者均存在明显胰岛素抵抗.(4)促肾上腺皮质激素(ACTH)、皮质醇(F)水平,三组患者基线血ACTH均明显受抑制,血F及24 h尿游离皮质醇(UFC)显著升高,组间差异无统计学意义(P＞0.05).PPNAD组小、大剂量地塞米松抑制试验后24 h UFC水平较基线显著升高(P＜0.05).结论 良性非ACTH依赖性显性库欣综合征中,BMAH就诊年龄最大,合并代谢紊乱最多,PPNAD就诊年龄最小,具有小、大剂量地塞米松抑制试验后24 h UFC显著升高的特点.
Abstract
Objective To provide a clinical basis for differential diagnosis,clinical features of Adrenocortical adenoma(ADA),bilateral macronodular adrenal hyperplasia(BMAH)and primary pigmented nodular adrenocortical disease(PPNAD)with overt Cushing syndrome were compared.Methods The clinical data of inpatients with confirmed ADA(n=86),BMAH(n=23)and PPNAD(n=7)were analyzed retrospectively from 2008 to 2020 in Chinese PLA General Hospital.Results 1.Patients in BMAH were the oldest with the average age(51.7±9.5)years,and patients in PPNAD group were the youngest with the average age of(19.9±5.7)years.There were more female patients in ADA and PPNAD groups,whereas the proportion of males to females in BMAH group was approximately equal.2.Clinical presentation:Purple striae(71.4％)were more common in PPNAD group,but there were no statistically differences in the presence of facial plethora,easy bruising,moon face,central obesity,and skin thinning among the three groups.3.Metabolic complications:Hyperglycemia and lipid disorders in the BMAH group were more common(87.0％and 68.2％,respectively),and the percentage of low bone mass or osteoporosis were all high in the three groups(＞75％).Systolic pressure,Glycated hemoglobin A1c,triglyceride,and serum sodium were higher in BMAH group(P＜0.05),compared with other two groups.In addition,all three groups presented marked insulin resistance according to HOMA-IR.4.ACTH and cortisol(F)levels:The baseline ACTH levels of the three groups were inhibited,and serum F and 24 h UFC levels increased in all three groups(P＞0.05).In patients with PPNAD,the 24 h UFC levels significantly elevated after low-dose dexamethasone suppression test(LDDST)and high-dose dexamethasone suppression test(HDDST)(all P＜0.05),compared with baseline 24 h UFC levels.Conclusion In benign overt adrenal Cushing syndrome,patients with BMAH are older and have more metabolic disorders,whereas patients with PPNAD are younger.The elevated 24 h UFC levels after LDDST and HDDST are useful for identifying PPNAD.
关键词
库欣综合征/非ACTH依赖/皮质腺瘤/双侧肾上腺大结节增生/原发性色素性结节样肾上腺皮质疾病Key words
Cushing syndrome/ACTH-independent/adrenocortical adenoma/bilateral macronodular adrenal hyperplasia/primary pigmented nodular adrenocortical disease引用本文复制引用
基金项目
国家重点研发计划(2020YFC2004902)
北京市海淀区卫生健康发展科研培育计划(HP2021-03-80303)
出版年
2024
国家科技期刊平台
NSTL
万方数据