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La granulomatose septique chronique chez l'enfant

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Book Details
Language
French
Publishers
Éditions universitaires européennes (4 April 2024)
Weight
0.12 KG
Publication Date
04/04/2024
ISBN-10
6206707954
Pages
72 pages
ISBN-13
9786206707950
Dimensions
15 x 0.43 x 22 cm
SKU
9786206707950
Author Name
Roeya Kolsi (Author)
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La granulomatose septique chronique est un déficit immunitaire rare, d’origine génétique, consécutif à un dysfonctionnement du complexe nicotinamide adénine dinucléotide phosphate oxydase (NADPH oxydase) des cellules phagocytaires. Les manifestations cliniques sont classées en deux grandes catégories, infectieuses et inflammatoires.

Les infections constituent les manifestations les plus courantes de la maladie à tout âge et les atteintes inflammatoires sont, habituellement, l’apanage des adultes. Il s'agit d'une maladie lourde.

La prise en charge repose principalement sur les mesures hygiéno-diététiques et la chimio-prophylaxie afin de prévenir les infections fongiques et bactériennes. L’espérance de vie s’est nettement améliorée mais le pronostic peut être mis en jeu par les complications infectieuses et inflammatoires.

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