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良性肾上腺源性库欣综合征临床特点比较分析

苗新宇 杜锦 郭清华 王先令 臧丽 李一君 闫文华 李春霖 母义明 林璐 李乐乐 窦京涛 陈康 吕朝晖 巴建明 裴育 谷伟军

中国实用内科杂志2024,Vol.44Issue(2):134-139,6.
中国实用内科杂志2024,Vol.44Issue(2):134-139,6.DOI:10.19538/j.nk2024020109

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杜锦 2郭清华 2王先令 2臧丽 2李一君 2闫文华 2李春霖 3母义明 2林璐 4李乐乐 5窦京涛 2陈康 2吕朝晖 2巴建明 2裴育 2谷伟军2

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
  • 3. 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
  • 4. 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
  • 5. 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
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摘要

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

分类

医药卫生

引用本文复制引用

苗新宇,杜锦,郭清华,王先令,臧丽,李一君,闫文华,李春霖,母义明,林璐,李乐乐,窦京涛,陈康,吕朝晖,巴建明,裴育,谷伟军..良性肾上腺源性库欣综合征临床特点比较分析[J].中国实用内科杂志,2024,44(2):134-139,6.

基金项目

国家重点研发计划(2020YFC2004902) (2020YFC2004902)

北京市海淀区卫生健康发展科研培育计划(HP2021-03-80303) (HP2021-03-80303)

中国实用内科杂志

OA北大核心CSTPCD

1005-2194

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