4.0 Article

Autoimmune polyglandular syndrome type 2: A case report

Journal

ANNALS OF MEDICINE AND SURGERY
Volume 78, Issue -, Pages -

Publisher

ELSEVIER SCI LTD
DOI: 10.1016/j.amsu.2022.103742

Keywords

Autoimmune polyendocrine syndrome; Autoimmune Addison?s disease; Schmidt syndrome

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This case report presents a rare case of a 9-year-old male with APS 2 and multiple gland insufficiencies. The report emphasizes the importance of early recognition and treatment of acute endocrine diseases, as well as the necessity to investigate pediatric patients with autoimmune diseases for coexisting conditions.
Introduction: Autoimmune polyglandular syndrome 2(APS 2) is immune-mediated destruction that affects two or more endocrine glands and causes a constellation of multiple glands insufficiencies. Case presentation: we reported a rare case 9 years old male diagnosed with APS 2; he had adrenal insufficiency three years ago due to leak adherence to hydrocortisone. He was admitted to the hospital for adrenal crises after hemodynamic stability; laboratory evaluation showed that he had Hashimoto's thyroiditis, celiac disease, and the glutamic acid decarboxylase antibody (GAD) Anti-islet cell antibodies were positive, so he was also predisposed to DM 1 later. Discussion: APS 2, also known as Schmidt's syndrome, is usually defined by the occurrence of the same fludrocortisone or more of the followings: primary adrenal insufficiency (Addison's disease), Grave's disease, primary hypothyroidism, type 1 diabetes mellitus, celiac disease, and pernicious anemia. Conclusion: This case report underlines the importance of early recognition and treatment of acute endocrine diseases and the necessity to investigate pediatric patients with autoimmune diseases for coexisting conditions.

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