A study on clinical features of ACTH-independent bilateral macronodular adrenal hyperplasia

Abstract

Objective To study the clinical features of patients with ACTH-independent bilateral macronodular adrenal hyperplasia(AIMAH).Methods Eighteen cases with AIMAH diagnosed in Chinese PLA General Hospital from 1998 to 2010 were analyzed retrospectively.Results ( 1 ) AIMAH was equally distributed between genders,mean age at diagnosis was ( 51.4 ± 10.7 ) years,and average course was ( 9.9:t:2.7 ) years.( 2 )Most patients visited hospital because of adrenal incidentaloma; hypertension and impaired glucose tolerance/diabetes mellitius were the most common clinical presentations; typical signs of Cushing′s syndrome (CS) such as moon face and central obesity were less frequent.( 3 ) All cases′ laboratory findings met the diagnostic criteria of ACTH-independent CS,some cases with the features of subclinical CS.24 h dynamic plasma ACTH and cortisol monitoring had the advantage of revealing the features of subclinical CS for some AIMAH cases; there was remarkable elevation of plasma cortisol after exogenous ACTH stimulation in AIMAH patients.(4) Bilateral adrenal macronodular changes could be visualized on CT scan.( 5 ) Pathology identified bilateral macronodular adrenal hyperplasia.( 6 ) Bilateral adrenalectomy was the most useful treatment.Conclusions AIMAH is an infrequent cause of CS,and some patients present subclinical CS.Its marked clinical feature is ACTH-independent bilateral macronodular adrenal hyperplasia. Key words: Adrenocorticotrophichormone; Adrenocortical,hyperplasia; Cushing′s syndrome