Primary bilateral macronodular adrenal hyperplasia(PBMAH)is a rare etiology of endogenous Cushing's syndrome.Atypical bilateral adrenal hyperplasia and/or atypical hypercortisolism are infrequent in patients with PBMAH,which is clinically characterized by benign bilateral macronodular adrenal hyperplasia with varying degrees of hypercortisolism.The majority of PBMAH is incidentally found through imaging examination,with mild cortisol secretion disorders being more common.In recent years,significant progress has been made in the understanding of the pathophysiology and genetics underlying PBMAH.The mechanism involves various gene abnor-malities,predominantly exhibiting autosomal dominant inheritance and familial aggregation.However,the pathogenesis of PBMAH is not clear,and there is no unified understanding of the clinical characteristics and diagnosis and treatment methods.Misdiagnosis and missed diagnosis occur frequently in clinical practice.This review summarizes research on PBMAH both domestically and internation-ally while providing an overview of the definition,clinical manifestations,imaging features,molecular diagnostic techniques,and treat-ment methods of PBMAH,aiming to deepen clinicians'understanding and attention to this disease,improve diagnostic accuracy,and offer new insights for molecular diagnostics research as well as treatment strategies of PBMAH.
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