(753) Aggressive Management as a Tool to Prevent Fatality: Autoimmune Polyglandular Syndrome Case Report

Introduction: Polyglandular Autoimmune Syndrome (PAS) is an intricate condition marked by autoimmune dysfunction affecting two or more endocrine glands . It can result from mutations in the regulator gene (AIRE) or polygenic factors. Diagnosis usually emerges later, when patients manifest symptoms like hypocalcemia from hypoparathyroidism or evident Addison's disease. PAS is classified into two main types: Type 1 and Type 2. Type 1, often initiating in early infancy, presents a triad of manifestations: candidiasis, hypoparathyroidism, and Addison’s disease. In contrast, Type 2, prevalent in infancy and adulthood, involves Addison’s disease, type 1 diabetes, and chronic thyroiditis.

Description: We present a case of a 13-year-old Hispanic girl admitted to the Pediatric Intensive Care Unit (PICU) after an adrenal crisis. Her medical history comprises PAS, encompassing Addison’s disease, hypoparathyroidism, and Growth Hormone deficiency, currently managed with hormonal replacements: calcitriol, hydrocortisone, insulin, cholecalciferol, calcium citrate. Throughout her stay, laboratory results indicated hypocalcemia, hyperglycemia, hypercapnia, and hypomagnesemia, all of which were promptly managed. Extensive consultation with an endocrinologist led to concurrence on adjusted medication and future follow-up. Prior PICU admissions disclosed symptoms like hypercalcemia, hyponatremia, pallor, jaundiced skin, low glucose, vomiting, and vertigo. Additional history involves first-degree AV block, palpitations, constipation, and nausea.

Discussion: This case underscores early recognition of endocrine dysfunction in diverse symptoms, emphasizing PAS consideration. The patient was educated on the severity of her condition and has strict instructions that, when she starts noticing the symptoms, she should come straight to our PICU for adequate management. Managing electrolyte imbalances and body homeostasis remains crucial for positive outcomes. Hormonal treatment, with strong compliance, is pivotal for sustained assessment and progress. In conclusion, this case underscores PAS's intricacy and the vital role of tailored management, optimizing patient well-being through vigilant evaluation.