title: [Genetic predisposition and children infectious disease].
abstract: The classic primary immunodeficiencies confer predisposition to multiple infectious diseases. However since ten years severe pediatric infections which were idiopathic have now molecular explanation. Indeed, defects in several genes confer a predisposition to infection with specific pathogenes in otherwise healthy individuals. These children present a new kind of hereditary immunodeficiency with severe and/or recurrent infections caused by only one microorganisms family, in opposition of others patients with "classic" primary immunodeficiency.
authors.affiliation: Centre d'Etude des Déficits Immunitaires, Hôpital Necker-Enfants-Malades, Assistance Publique-Hôpitaux de Paris, 149, rue de Sèvres, 75015 Paris, France. picardc@necker.fr <picardc@necker.fr>
authors.forename: C
authors.initials: C
authors.lastname: Picard
authors.forename: O
authors.initials: O
authors.lastname: Filipe-Santos
authors.forename: A
authors.initials: A
authors.lastname: Chapgier
authors.forename: H
authors.initials: H
authors.lastname: von Bernuth
authors.forename: G
authors.initials: G
authors.lastname: Vogt
authors.forename: J-L
authors.initials: JL
authors.lastname: Casanova
mesh_terms: D002648:Child; D003141:Communicable Diseases / Q000235:genetics*; D020022:Genetic Predisposition to Disease; D006801:Humans; D051550:I-kappa B Kinase / Q000235:genetics; D020672:I-kappa B Proteins / Q000235:genetics; D007371:Interferon-gamma / Q000235:genetics; D053592:Interleukin-1 Receptor-Associated Kinases / Q000235:genetics; D018664:Interleukin-12 / Q000235:genetics; D050794:STAT1 Transcription Factor / Q000235:genetics
chemical_list: C083487:I kappa B beta protein; D020672:I-kappa B Proteins; C491731:IKBKG protein, human; D050794:STAT1 Transcription Factor; C494079:STAT1 protein, human; D018664:Interleukin-12; D007371:Interferon-gamma; C507773:IRAK4 protein, human; D053592:Interleukin-1 Receptor-Associated Kinases; D051550:I-kappa B Kinase