![]() ![]() ![]() Macronodular BAHs, ACTH-independent macronodular hyperplasia (AIMAH) or massive macronodular adrenocortical disease (MMAD) are less frequently genetic and almost never present in children (except in McCune-Albright syndrome) they present often with atypical CS in middle-aged or elderly adults. In both adults and children, adrenocortical lesions causing CS include the common, isolated and sporadic, solitary cortisol-producing adenoma, the rare adrenocortical cancer, and a spectrum of recently recognized, bilateral hyperplasias (bilateral adrenocortical hyperplasias - BAHs): micronodular adrenal disease (MAD) and its pigmented variant, primary pigmented nodular adrenocortical disease (PPNAD) are mostly genetic processes. In younger children, ACTH-independent CS may account for as many as half of CS cases. Endogenous Cushing syndrome (CS) is caused by excess adrenal glucocorticoid secretion that is adrenocorticotropin (ACTH)-dependent or independent ACTH-independent adrenocortical causes of CS account for up to 20% of CS in adults, and 15% in children over age 7 years. ![]()
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