Comparison and analysis of clinical features of benign adrenal Cushing syndrome
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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.
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.
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