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目的 探讨非ACTH依赖性双侧肾上腺皮质大结节增生症 (AIMAH)的临床特点。方法分析 4例AIMAH临床和实验室资料 ,结合有关文献进行讨论。结果 4例AIMAH均有库欣综合征的临床表现及生化异常 ,实验检查显示血浆ACTH水平低下 ,皮质醇分泌节律紊乱 ,小剂量、大剂量地塞米松抑制试验均不被抑制 ,CT检查示双侧肾上腺明显增大呈结节样改变。结论 AIMAH是库欣综合征中病因独特的临床亚型 ,有其特有的临床特点及病理改变
Objective To investigate the clinical features of non-ACTH-dependent bilateral adrenal cortex nodular hyperplasia (AIMAH). Methods Four cases of clinical and laboratory data of AIMAH were analyzed and discussed with relevant literature. Results The clinical manifestations and biochemical abnormalities of Cushing’s syndrome were found in 4 cases of AIMAH. The results of laboratory tests showed that plasma ACTH level was low, cortisol secretion rhythm disorder, low-dose and high-dose dexamethasone suppression test were not inhibited. CT examination showed double Side of the adrenal gland was significantly increased nodular-like changes. Conclusion AIMAH is a unique clinical subtype of Cushing’s syndrome with its unique clinical features and pathological changes