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Granulomatosi settica cronica nei bambini

Granulomatosi settica cronica nei bambini

Abir Kallel / Imene Moalla / Manel Weli

72,21 €
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Editorial:
KS OmniScriptum Publishing
Año de edición:
2026
Materia
Medicina clínica e interna
ISBN:
9786630185836
72,21 €
IVA incluido
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Sobre IBD

La granulomatosi settica cronica è una rara immunodeficienza primaria dovuta a una disfunzione del complesso NADPH ossidasi dei fagociti.La malattia è di origine genetica, trasmessa più spesso con modalità recessiva legata al cromosoma X, più raramente con modalità autosomica recessiva. Le forme legate al cromosoma X sono generalmente più gravi e si manifestano più precocemente.La diagnosi viene spesso formulata già nei primi anni di vita, in presenza di infezioni gravi e ricorrenti. I pazienti sono particolarmente esposti alle infezioni batteriche e fungine, in particolare all’aspergillosi invasiva.Granulomi infiammatori cronici possono inoltre interessare diversi organi e compromettere la qualità della vita dei bambini affetti.

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