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  • Primary Immunodeficiency Diseases in two neighboring pediatric centers : registry data bring out a wide spectrum of diseases with complex clinical presentations
    Pirrone, Angela ...
    In the last few decades, the spectrum of primary immunoŽ-Ždeficiencies (PIDs) has greatly widened, including more than 150 different definite entities [1], and this is slowly being reflected in the ... distribution of PID in internationalregistries [2]. Besides classical PIDs, new disorders are being increasingly diagnosed, which are associated with complex defects of immune functions [3]. Nowadays, it is a well accepted concept that the clinical spectrum of PID includes inflammatory, autoimmune and lymphoproliferation symptoms as well as recurrent or severe infections. Indeed, even disorders classically characterized by infections may present at first with non-infectious symptoms [4]. As a consequence, warning signs developed many years ago to improve the recognition of PIDs - such as the 10 signs promoted by the Jeffrey Modell Foundation - may be insufficient today, in particular inpediatric practice [5]. The European Society for Immunodeficiencies (ESID) proposed a different set of warning criteria that should include inflammatory,autoimmune and lymphoproliferative features, as well as other long-established criteria, to allow a better identification of PIDs by non immunologists [6]. However, the consequences of these developments in the daily clinical practice of pediatric departments have not been analyzed so far. On the other hand, according to recent data, the specific training of physicians on PID seems to have a major role in improving recognition and diagnosis of these disorders.
    Vir: Clinical immunology. - ISSN 1521-6616 (Vol. 37, iss. 4, 2012, str. 365-370)
    Vrsta gradiva - članek, sestavni del
    Leto - 2012
    Jezik - angleški
    COBISS.SI-ID - 658348

    Povezava(-e):

    http://www.termedia.pl/Clinical-immunology-Primary-Immunodeficiency-Diseases-in-two-neighboring-pediatric-centers-registry-data-bring-out-a-wide-spectrum-of-diseases-with-complex-clinical-presentations,10,20089,1,1.html
    DOI

vir: Clinical immunology. - ISSN 1521-6616 (Vol. 37, iss. 4, 2012, str. 365-370)
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