Insights Into Pediatric Non-congenital Adrenal Hyperplasia: A Review Based on 5 Different Rare Cases with Primary Adrenal Insufficiency

Adrenal insufficiency presents a significant clinical challenge due to its diverse etiologies and
potentially life-threatening consequences. This review highlights the spectrum of adrenal insuf-
ficiency, focusing on primary adrenal insufficiency (PAI). Childhood PAI, predominantly con-
genital, presents unique diagnostic and management considerations. 

An aspect of this review is the discussion of PAI related to non-congenital adrenal hyperpla-
sia, particularly adrenocorticotropic hormone (ACTH) resistance syndromes and autoimmune
adrenal insufficiency. The clinical presentation, diagnosis, and treatment management of these
rare childhood PAI types are assessed through 5 case studies.

Despite advancements in genetic understanding, some cases are unsolved and remain diag-
nostic mysteries. There is a need for further research and elucidation of molecular etiopatho-
genesis in adrenal insufficiency. Clinicians are pivotal in identifying these rare diseases and
providing lifesaving outcomes.

Cite this article as: Cetin SK, Siklar Z, Ozsu E, Aycan Z, Berberoglu M. Insights into pediatric non-congenital adrenal hyperplasia: A review based on 5 different rare cases with primary adrenal insufficiency. Turk ArchPediatr. 2025;60(3):258-267.