促肾上腺皮质激素非依赖性肾上腺大结节样增生的研究进展

促肾上腺皮质激素非依赖性肾上腺大结节样增生(AIMAH)是肾上腺病变病理分类的一种,较少见.AIMAH在临床上多表现为库欣综合征或亚临床库欣综合征,同时可伴有其他系统疾病,有典型的影像学表现,更少见的为家族性AIMAH.AIMAH的发病机制可能为病变肾上腺组织中异常表达的多种激素和细胞因子受体导致皮质激素过度分泌,涉及相关基因的突变或缺陷.目前,AIMAH的治疗以手术为主.随着病理机制研究的不断深入,药物治疗越来越多地应用于临床.文章就AIMAH的临床特点、发病机制及治疗措施作一综述.

Research progress of adrenocorticotropic hormone-independent macronodular adrenal hyperplasia

Adrenocorticotropic hormone-independent macronodular adrenal hyperplasia(AIMAH) is a rare adrenal pathological lesion.AIMAH presents clinically as overt or subclinical Cushing’s syndrome with typical radiological findings,occasionally with other diseases.What is rarer is the familial type.The mechanism of AIMAH may be the aberrant expression of several hormones and cytokines receptors in adrenal,which leads to the oversecretion of glucocorticoid,and the molecular mechanism involves genic mutation or deficiency of the receptors.Although presently adrenalectomy is the main treatment,with further research on pathophysiology,more and more effective medication will be used.The research progress of clinical features,mechanism,and treatment of AIMAH is reviewed in this paper.