[Innate immunity primary immunodeficiencies and infections].
The diagnosis of primary immunodeficiency diseases (PIDs) is important for the early and adaptive care of patients and their families. Among the various known PIDs, a number of them concern the innate immune system, which involve a set of cells and mechanisms involved in the host defense by a nonspecific and fast response. The majority of patients with innate immunity defects have a predisposition to one isolated type of infection (bacterial, viral, or fungal), dependent on the genetic defect involved. This article describes the different PIDs involving innate immunity and the immunological investigations allowing for their diagnosis.
Centre d'étude des déficits immunitaires, hôpital Necker-Enfants-Malades AP-HP, 149, rue de Sèvres, 75015 Paris, France; Faculté de médecine Necker, université Paris Descartes, Paris Sorbonne Cité, 12, rue de l'École-de-Médecine, 75006 Paris, France.
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Duchamp
Centre d'étude des déficits immunitaires, hôpital Necker-Enfants-Malades AP-HP, 149, rue de Sèvres, 75015 Paris, France.
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Miot
Centre d'étude des déficits immunitaires, hôpital Necker-Enfants-Malades AP-HP, 149, rue de Sèvres, 75015 Paris, France; Faculté de médecine Necker, université Paris Descartes, Paris Sorbonne Cité, 12, rue de l'École-de-Médecine, 75006 Paris, France; Inserm UMR 1163, génétique humaine des maladies infectieuses, institut imagine, 24, boulevard du Montparnasse, 75015 Paris, France.
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Bustamante
Centre d'étude des déficits immunitaires, hôpital Necker-Enfants-Malades AP-HP, 149, rue de Sèvres, 75015 Paris, France; Faculté de médecine Necker, université Paris Descartes, Paris Sorbonne Cité, 12, rue de l'École-de-Médecine, 75006 Paris, France; Inserm UMR 1163, génétique humaine des maladies infectieuses, institut imagine, 24, boulevard du Montparnasse, 75015 Paris, France. Electronic address: capucine.picard@inserm.fr.
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Picard
D003165:Complement System Proteins / Q000172:deficiency; D006105:Granulomatous Disease, Chronic / Q000209:etiology; D006801:Humans; D007113:Immunity, Innate; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D009165:Mycobacterium Infections, Nontuberculous / Q000276:immunology; D009894:Opportunistic Infections / Q000209:etiology*
D003165:Complement System Proteins