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Przewlekła septyczna granulomatoza u dzieci

Przewlekła septyczna granulomatoza u dzieci

Abir Kallel / Imene Moalla / Manel Weli

72,27 €
IVA incluido
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Editorial:
KS OmniScriptum Publishing
Año de edición:
2026
Materia
Medicina clínica e interna
ISBN:
9786630188394
72,27 €
IVA incluido
Disponible

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Sobre IBD

Przewlekła granulomatoza septyczna to rzadki pierwotny niedobór odpornościowy spowodowany nieprawidłowym funkcjonowaniem kompleksu oksydazy NADPH w fagocytach.Choroba ma podłoże genetyczne i jest najczęściej dziedziczona w sposób recesywny związany z chromosomem X, rzadziej w sposób autosomalny recesywny. Postacie związane z chromosomem X są zazwyczaj cięższe i pojawiają się wcześniej.Diagnozę często stawia się już w pierwszych latach życia w związku z ciężkimi i nawracającymi infekcjami. Pacjenci są szczególnie narażeni na infekcje bakteryjne i grzybicze, zwłaszcza na inwazyjną aspergilozę.Przewlekłe ziarniniaki zapalne mogą również atakować wiele narządów i pogarszać jakość życia dotkniętych chorobą dzieci.

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