Addison’s Disease in Paediatric Age

Introduction: Primary adrenal insufficiency not associated to enzymatic defects is rare in pediatric patients but may cause significant morbidity.Main etiologic diagnosis are Addison's disease (autoimmune) in both sex and adrenoleukodystrophy in males.Our objective was description of the original clinical presentation of patients with primary adrenal insufficiency, etiologic diagnosis, therapy and their evolution.Methods: Retrospective analysis of patients under 18 years of age with a confirmed diagnosis of primary adrenal insufficiency between 2010 and 2018.Results: Four patients were diagnosed with Addison's disease, all male, age ranging between 12 and 16 years.Clinical presentation was lethargy and salt craving and 50% showed skin hyperpigmentation and hypotension.Laboratory analysis established the diagnosis based on non-stimulated adrenocorticotropic hormone, serum cortisol and active renin; all patients had adrenal cortex antibodies.