The term adrenocorticotropin (ACTH) resistance syndrome is used for a group of rare inherited disorders, which present with primary adrenal insufficiency during childhood. The syndrome includes two disorders inherited in an autosomal recessive fashion - familial glucocorticoid deficiency and triple A syndrome. Herein, we report our experience of three cases with ACTH resistance syndrome, highlighting the approach to diagnosis and management in such patients.
Keywords: ACTH resistance syndrome; adrenal insufficiency; familial glucocorticoid deficiency; isolated glucocorticoid deficiency; triple A syndrome.