ESPE Abstracts (2022) 95 P2-20

ESPE2022 Poster Category 2 Adrenals and HPA Axis (27 abstracts)

Autoimmune Polyglandular Syndrome Type 2: Two Different Applications

Ayse Derya Bulus 1 , Yuksel Yasartekin 1 & Mihriban İnözü 2

1Ankara Atatürk Sanatoryum Atatürk Sanatorium Education and Research Hospital, Ankara, Turkey; 2Ankara City Hospital, Ankara, Turkey

Autoimmune polyglandular syndrome (APS), on the other hand, is a cluster of endocrine disorders resulting from immune dysregulation, often accompanied by damage to non-endocrine organs. There are 2 types of APS: type 1 and type 2 (APS-1 and APS-2). Type 2 APS is characterized by the development of organ- and tissue-specific antibodies. In those with Addison's disease, antibodies to the 21-hydroxylase enzyme found in the adrenal cortex are present in more than 90% of patients. It should be known that autoantibodies will be found positive before clinical disease development. Our first case, a 16-year-old male patient, applied to the emergency service with the complaints of drinking a lot of water for 3 weeks, urinating a lot, rapid weight loss, weakness, dizziness and nausea in the last week. The fact that the patient's autoimmune adrenalitis findings appeared at a relatively late age and was associated with type 1 diabetes suggest APS type 2, and 21 hydroxylase antibodies in our case were found to be 42.20 U/ml. Our patient is being followed up in our outpatient clinic. Our second case was admitted to our emergency department at the age of 7 years and 10 months with the complaint of abdominal pain.. As the laboratory tests revealed glucose: 84 mg/dl, Na: 108 mmol/l, F: 4.03 mmol/l, HGB: 8.8 g/dl, Na-increasing fluid therapy was started and ACTH and cortisol were studied with a preliminary diagnosis of adrenal insufficiency. ACTH: We started hydrocortisone and fludricortisone treatments for our patient with cortisol renin aldosterone. Since our patient had congenital hypothyroidism with follow-up and the addition of primary adrenal insufficiency, we ran 21 hydroxylase antibodies and it was found to be 46.70 U/ml positive. Our patient is currently 16 years old and is still being followed up in our outpatient clinic. It is noteworthy that our first case presented as diabetic ketosis, but there were signs of adrenal insufficiency after laboratory and physical examination. It is noteworthy that autoimmune adrenalitis was present at the time of initial diagnosis, newly diagnosed Type 1 diabetes, and there was no such case described in the literature. In our second case, both the presence of autoimmune adrenalitis and the presence of thyroiditis support APS type 2, and the development of thyroiditis first and then adrenalitis is both important and important to keep in mind.

Volume 95

60th Annual ESPE (ESPE 2022)

Rome, Italy
15 Sep 2022 - 17 Sep 2022

European Society for Paediatric Endocrinology 

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