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eISSN: 2300 - 8660
ISSN: 0031-3939
Pediatria Polska - Polish Journal of Paediatrics
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2/2022
vol. 97
 
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abstract:
Case report

Autoimmune polyglandular syndrome type 2 in an 15-year-old boy

Andrzej Badeński
1
,
Marta Badeńska
2
,
Magdalena Mierzwa
3
,
Małgorzata Stojewska
1
,
Grażyna Deja
4
,
Maria Szczepańska
1
,
Katarzyna Ziora
1

1.
Department of Pediatrics, Faculty of Medical Sciences in Zabrze, Medical University of Silesia, Katowice, Poland
2.
Academic Centre of Dentistry and Specialized Medicine Limited Liability Company, Bytom, Poland
3.
Department of Pediatric Endocrinology, Public Clinical Hospital No. 1 Zabrze, Zabrze, Poland
4.
Department of Children’s Diabetology, Faculty of Medical Sciences in Katowice, Medical University of Silesia, Katowice, Poland
Pediatr Pol 2022; 97 (2): 151-155
Online publish date: 2022/06/10
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Autoimmune polyendocrine syndromes (APS) include a diverse group of clinical conditions, characterized by functional impairment of many endocrine glands. Type 2 APS is characterized by the mandatory presence of Addison’s disease, which may be associated with either autoimmune thyroid diseases as a Schmidt’s syndrome or type 1 diabetes mellitus as a Carpenter syndrome.

The described patient with well-controlled diabetes mellitus type 1, presented with sudden, frequent episodes of hypoglycemia, daytime somnolence and weariness, as well as remarkable hyperpigmentation of the skin. Laboratory tests revealed adrenal insufficiency with a positive titer of anti-adrenal antibodies, which led to APS-2 diagnosis in the presented case.

As the clinical outcome at the early onset of the APS-2 is not distinctive, the authors would like to emphasize the significance of screening methods, especially in patients with other hormone abnormalities, to protect them from the consequences of life-threatening disturbances.
keywords:

Addison’s disease, diabetes mellitus type 1, autoimmune polyglandular syndrome, rare diseases, carpenter syndrome