Hyper-IgE syndrome and autoimmunity in Mexican children
Identifieur interne : 001D22 ( Main/Exploration ); précédent : 001D21; suivant : 001D23Hyper-IgE syndrome and autoimmunity in Mexican children
Auteurs : Marco Yamazaki-Nakashimada [Mexique] ; Samuel Zaltzman-Girshevich [Mexique] ; Silvestre Garcia De La Puente [Mexique] ; Beatriz De Leon-Bojorge [Mexique] ; Sara Espinosa-Padilla [Mexique] ; Marimar Saez-De-Ocariz [Mexique] ; Daniel Carrasco-Daza [Mexique] ; Victor Hernandez-Bautista [Mexique] ; Lorenzo Pérez-Fernandez [Mexique] ; Francisco Espinosa-Rosales [Mexique]Source :
- Pediatric Nephrology [ 0931-041X ] ; 2006-08-01.
English descriptors
- KwdEn :
Abstract
Abstract: Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by recurrent skin abscesses, recurrent pneumonia with pneumatocele formation, eczema, eosinophilia, and elevated levels of serum IgE. Patients with the autosomal recessive (AR) form of HIES appear to be prone to developing autoimmune diseases. We present two cases of HIES with autoimmune complications; one case was a product of a consanguineous marriage, the other one was a sporadic case. The first patient presented with recurrent episodes of erythema nodosum, warts, bronchiolitis obliterans and thrombocytopenia. The second patient developed glomerulonephritis resulting in endstage renal failure. She later developed malar rash, oral ulcers, cerebral infarcts with vasculitis and positive ANA, anti-dsDNA, and antiphospholipid antibodies. We discuss the dilemma in treating patients who present with both primary immunodeficiency and autoimmunity.
Url:
DOI: 10.1007/s00467-006-0178-3
Affiliations:
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Le document en format XML
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<author><name sortKey="Hernandez Bautista, Victor" sort="Hernandez Bautista, Victor" uniqKey="Hernandez Bautista V" first="Victor" last="Hernandez-Bautista">Victor Hernandez-Bautista</name>
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<front><div type="abstract" xml:lang="en">Abstract: Hyper-IgE syndrome (HIES) is a primary immunodeficiency characterized by recurrent skin abscesses, recurrent pneumonia with pneumatocele formation, eczema, eosinophilia, and elevated levels of serum IgE. Patients with the autosomal recessive (AR) form of HIES appear to be prone to developing autoimmune diseases. We present two cases of HIES with autoimmune complications; one case was a product of a consanguineous marriage, the other one was a sporadic case. The first patient presented with recurrent episodes of erythema nodosum, warts, bronchiolitis obliterans and thrombocytopenia. The second patient developed glomerulonephritis resulting in endstage renal failure. She later developed malar rash, oral ulcers, cerebral infarcts with vasculitis and positive ANA, anti-dsDNA, and antiphospholipid antibodies. We discuss the dilemma in treating patients who present with both primary immunodeficiency and autoimmunity.</div>
</front>
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<name sortKey="Garcia De La Puente, Silvestre" sort="Garcia De La Puente, Silvestre" uniqKey="Garcia De La Puente S" first="Silvestre" last="Garcia De La Puente">Silvestre Garcia De La Puente</name>
<name sortKey="Hernandez Bautista, Victor" sort="Hernandez Bautista, Victor" uniqKey="Hernandez Bautista V" first="Victor" last="Hernandez-Bautista">Victor Hernandez-Bautista</name>
<name sortKey="Perez Fernandez, Lorenzo" sort="Perez Fernandez, Lorenzo" uniqKey="Perez Fernandez L" first="Lorenzo" last="Pérez-Fernandez">Lorenzo Pérez-Fernandez</name>
<name sortKey="Saez De Ocariz, Marimar" sort="Saez De Ocariz, Marimar" uniqKey="Saez De Ocariz M" first="Marimar" last="Saez-De-Ocariz">Marimar Saez-De-Ocariz</name>
<name sortKey="Yamazaki Nakashimada, Marco" sort="Yamazaki Nakashimada, Marco" uniqKey="Yamazaki Nakashimada M" first="Marco" last="Yamazaki-Nakashimada">Marco Yamazaki-Nakashimada</name>
<name sortKey="Zaltzman Girshevich, Samuel" sort="Zaltzman Girshevich, Samuel" uniqKey="Zaltzman Girshevich S" first="Samuel" last="Zaltzman-Girshevich">Samuel Zaltzman-Girshevich</name>
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