ACTH resistance syndrome: An experience of three cases

Abstract

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