Peculiarities of autoimmune polyglandular syndromes in children and adolescents

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Giuseppina Zirilli
Simona Santucci
Chiara Cuzzupè
Domenico Corica
Elda Pitrolo
Giuseppina Salzano


Associations of autoimmune disorders, epidemiology, immunodeficiency, phenotypical expression


Background: no reviews have specifically addressed , to now, whether autoimmune polyglandular syndromes (APSs) may have a peculiar epidemiology and phenotypical expression in pediatric age

Objectives: to review the most recent literature data about the specific epidemiological and clinical peculiarities of APSs in childhood and adolescence

Design: the main features of the different APSs in pediatric age were compared among them.

Conclusions: 1) Among the different APSs, the one that is most typical of pediatric age is APS-1; 2) APS-1 is not characterized only by the classical triad (chronic moniliasis-hyposurrenalism-hypoparathyroidism) and its clinical spectrum is enlarging over time; 3)APS-2 may have a different epidemiological and clinical expression according to two different nosological classifications.



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