Peculiarities of autoimmune polyglandular syndromes in children and adolescents

Main Article Content

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

Keywords

Associations of autoimmune disorders, epidemiology, immunodeficiency, phenotypical expression

Abstract

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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