Septicemia without Sepsis: Inherited Disorders of Nuclear Factor-kB-Mediated Inflammation
Identifieur interne : 000532 ( France/Analysis ); précédent : 000531; suivant : 000533Septicemia without Sepsis: Inherited Disorders of Nuclear Factor-kB-Mediated Inflammation
Auteurs : Horst Von Bernuth [France, Allemagne] ; Anne Puel [France] ; Cheng-Lung Ku [France] ; Kun Yang [France, République populaire de Chine] ; Jacinta Bustamante [France] ; Huey-Hsuan Chang [France] ; Capucine Picard [France] ; Jean-Laurent Casanova [France, République populaire de Chine]Source :
- Clinical Infectious Diseases [ 1058-4838 ] ; 2005-11-15.
Abstract
Septicemia is a life-threatening condition that may lead to sepsis and even septic shock. This cascade is usually accompanied by a pronounced inflammatory response, leading to high body temperature and elevated levels of laboratory markers of inflammation. However, this response can be significantly diminished in children with inherited disorders of nuclear factor (NF)–κB–mediated immunity. Three disease-causing genes involved in NF-κB activation have been identified:NEMO, IKBA, and IRAK4. Patients with anhidrotic ectodermal dysplasia and immunodeficiency, which is caused by mutations in NEMO and IKBA, have sparse hair, dry skin, and conical teeth an dare at increased risk of severe infections caused by pyogenic bacteria and atypical mycobacteria. Patients with interleukin-1 receptor—associated kinase–4 deficiency are at increased risk of invasive disease due to pyogenic bacteria. An underlying defect in NF-κB activation should be suspected in children with bacterial septicemia accompanied by mild signs of inflammation.
Url:
DOI: 10.1086/431994
Affiliations:
- Allemagne, France, République populaire de Chine
- District de Dresde, Saxe (Land), Île-de-France
- Dresde, Paris
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ISTEX:5929D418F4ED291378BE47B7B8A23244B2B43C6CLe document en format XML
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Chicago Press</publisher><date type="published" when="2005-11-15">2005-11-15</date><biblScope unit="volume">41</biblScope><biblScope unit="issue">Supplement_7</biblScope><biblScope unit="page" from="S436">S436</biblScope><biblScope unit="page" to="S439">S439</biblScope></imprint><idno type="ISSN">1058-4838</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">1058-4838</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract">Septicemia is a life-threatening condition that may lead to sepsis and even septic shock. This cascade is usually accompanied by a pronounced inflammatory response, leading to high body temperature and elevated levels of laboratory markers of inflammation. However, this response can be significantly diminished in children with inherited disorders of nuclear factor (NF)–κB–mediated immunity. Three disease-causing genes involved in NF-κB activation have been identified:NEMO, IKBA, and IRAK4. Patients with anhidrotic ectodermal dysplasia and immunodeficiency, which is caused by mutations in NEMO and IKBA, have sparse hair, dry skin, and conical teeth an dare at increased risk of severe infections caused by pyogenic bacteria and atypical mycobacteria. Patients with interleukin-1 receptor—associated kinase–4 deficiency are at increased risk of invasive disease due to pyogenic bacteria. An underlying defect in NF-κB activation should be suspected in children with bacterial septicemia accompanied by mild signs of inflammation.</div></front></TEI><affiliations><list><country><li>Allemagne</li><li>France</li><li>République populaire de Chine</li></country><region><li>District de Dresde</li><li>Saxe (Land)</li><li>Île-de-France</li></region><settlement><li>Dresde</li><li>Paris</li></settlement></list><tree><country name="France"><region name="Île-de-France"><name sortKey="Bernuth, Horst Von" sort="Bernuth, Horst Von" uniqKey="Bernuth H" first="Horst Von" last="Bernuth">Horst Von Bernuth</name></region><name sortKey="Bustamante, Jacinta" sort="Bustamante, Jacinta" uniqKey="Bustamante J" first="Jacinta" last="Bustamante">Jacinta Bustamante</name><name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name><name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name><name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name><name sortKey="Chang, Huey Hsuan" sort="Chang, Huey Hsuan" uniqKey="Chang H" first="Huey-Hsuan" last="Chang">Huey-Hsuan Chang</name><name sortKey="Ku, Cheng Lung" sort="Ku, Cheng Lung" uniqKey="Ku C" first="Cheng-Lung" last="Ku">Cheng-Lung Ku</name><name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name><name sortKey="Picard, Capucine" sort="Picard, Capucine" uniqKey="Picard C" first="Capucine" last="Picard">Capucine Picard</name><name sortKey="Puel, Anne" sort="Puel, Anne" uniqKey="Puel A" first="Anne" last="Puel">Anne Puel</name><name sortKey="Yang, Kun" sort="Yang, Kun" uniqKey="Yang K" first="Kun" last="Yang">Kun Yang</name></country><country name="Allemagne"><region name="Saxe (Land)"><name sortKey="Bernuth, Horst Von" sort="Bernuth, Horst Von" uniqKey="Bernuth H" first="Horst Von" last="Bernuth">Horst Von Bernuth</name></region></country><country name="République populaire de Chine"><noRegion><name sortKey="Yang, Kun" sort="Yang, Kun" uniqKey="Yang K" first="Kun" last="Yang">Kun Yang</name></noRegion><name sortKey="Casanova, Jean Laurent" sort="Casanova, Jean Laurent" uniqKey="Casanova J" first="Jean-Laurent" last="Casanova">Jean-Laurent Casanova</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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