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Article: Defective B-cell and regulatory T-cell function in Wiskott-Aldrich syndrome

TitleDefective B-cell and regulatory T-cell function in Wiskott-Aldrich syndrome
Authors
KeywordsChinese
lymphocytes
platelets
Wiskott-Aldrich syndrome
Issue Date1992
PublisherSpringer Verlag. The Journal's web site is located at http://link.springer.de/link/service/journals/00431/index.htm
Citation
European Journal Of Pediatrics, 1992, v. 151 n. 9, p. 680-683 How to Cite?
AbstractWe report two Chinese boys with Wiskott-Aldrich syndrome presenting with gastro-intestinal bleeding, eczema and recurrent infection. They had thrombocytopenia and the mean platelet volume was small. Serum IgG and IgA were elevated and lymphocyte proliferation in response to phytohaemagglutinin, concanavalin A and pokeweed mitogen was defective. Despite documented herpes simplex virus type 1 and cytomegalovirus infection in one patient, he did not mount any humoral response. The generation of antibody-secreting cells in response to pokeweed mitogen was markedly defective in aplaque-forming cell assay. Both patients' regulatory T cell and B-cell functions were defective in this assay. The genetic defect in Wiskott-Aldrich syndrome therefore affects T-cells, B-cells and platelets.
Persistent Identifierhttp://hdl.handle.net/10722/170259
ISSN
2015 Impact Factor: 1.791
2015 SCImago Journal Rankings: 0.772
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorLau, YLen_HK
dc.contributor.authorJones, BMen_HK
dc.contributor.authorLow, LCKen_HK
dc.contributor.authorWong, SNen_HK
dc.contributor.authorLeung, NKen_HK
dc.date.accessioned2012-10-30T06:07:02Z-
dc.date.available2012-10-30T06:07:02Z-
dc.date.issued1992en_HK
dc.identifier.citationEuropean Journal Of Pediatrics, 1992, v. 151 n. 9, p. 680-683en_HK
dc.identifier.issn0340-6199en_HK
dc.identifier.urihttp://hdl.handle.net/10722/170259-
dc.description.abstractWe report two Chinese boys with Wiskott-Aldrich syndrome presenting with gastro-intestinal bleeding, eczema and recurrent infection. They had thrombocytopenia and the mean platelet volume was small. Serum IgG and IgA were elevated and lymphocyte proliferation in response to phytohaemagglutinin, concanavalin A and pokeweed mitogen was defective. Despite documented herpes simplex virus type 1 and cytomegalovirus infection in one patient, he did not mount any humoral response. The generation of antibody-secreting cells in response to pokeweed mitogen was markedly defective in aplaque-forming cell assay. Both patients' regulatory T cell and B-cell functions were defective in this assay. The genetic defect in Wiskott-Aldrich syndrome therefore affects T-cells, B-cells and platelets.en_HK
dc.languageengen_US
dc.publisherSpringer Verlag. The Journal's web site is located at http://link.springer.de/link/service/journals/00431/index.htmen_HK
dc.relation.ispartofEuropean Journal of Pediatricsen_HK
dc.subjectChineseen_HK
dc.subjectlymphocytesen_HK
dc.subjectplateletsen_HK
dc.subjectWiskott-Aldrich syndromeen_HK
dc.subject.meshAntibody Formationen_US
dc.subject.meshB-Lymphocytes - Cytology - Physiologyen_US
dc.subject.meshCell Divisionen_US
dc.subject.meshHumansen_US
dc.subject.meshInfanten_US
dc.subject.meshMaleen_US
dc.subject.meshT-Lymphocytes - Cytology - Physiologyen_US
dc.subject.meshWiskott-Aldrich Syndrome - Genetics - Immunology - Pathologyen_US
dc.titleDefective B-cell and regulatory T-cell function in Wiskott-Aldrich syndromeen_HK
dc.typeArticleen_HK
dc.identifier.emailLau, YL: lauylung@hku.hken_HK
dc.identifier.emailLow, LCK: lcklow@hkucc.hku.hken_HK
dc.identifier.authorityLau, YL=rp00361en_HK
dc.identifier.authorityLow, LCK=rp00337en_HK
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1007/BF01957573-
dc.identifier.pmid1396930-
dc.identifier.scopuseid_2-s2.0-0026693064en_HK
dc.identifier.volume151en_HK
dc.identifier.issue9en_HK
dc.identifier.spage680en_HK
dc.identifier.epage683en_HK
dc.identifier.isiWOS:A1992JG58300011-
dc.publisher.placeGermanyen_HK
dc.identifier.scopusauthoridLau, YL=7201403380en_HK
dc.identifier.scopusauthoridJones, BM=7404958958en_HK
dc.identifier.scopusauthoridLow, LCK=7007049461en_HK
dc.identifier.scopusauthoridWong, SN=7404590305en_HK
dc.identifier.scopusauthoridLeung, NK=55107144800en_HK

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