Show simple item record

dc.contributor.authorStefansson Thors, V
dc.contributor.authorKolka, R
dc.contributor.authorSigurdardottir, S L
dc.contributor.authorEdvardsson, V O
dc.contributor.authorArason, G
dc.contributor.authorHaraldsson, A
dc.date.accessioned2006-05-18T14:13:59Z
dc.date.available2006-05-18T14:13:59Z
dc.date.issued2005-03-01
dc.identifier.citationScand. J. Immunol. 2005, 61(3):274-8en
dc.identifier.issn0300-9475
dc.identifier.pmid15787745
dc.identifier.doi10.1111/j.1365-3083.2005.01533.x
dc.identifier.urihttp://hdl.handle.net/2336/2845
dc.descriptionTo access publisher full text version of this article. Please click on the hyperlink in Additional Links fielden
dc.description.abstractHenoch-Schonlein purpura (HSP) is a vasculitis of unknown aetiology, possibly involving immune complexes. The complement system is essential for the clearance of immune complexes. Our aim was to explore the hypothesis that patients with HSP have abnormal complements, contributing to the development of the disease. The study included 56 patients diagnosed with HSP at the Children's Hospital, Iceland between 1984 and 2000, and 98 blood donors as controls. Serum levels of immunoglobulin A, C4A, C4B and mannan-binding lectin were measured and compared between the two groups. C4 null alleles were significantly more common in HSP patients than in controls (P = 0.018) and were carried by 66.1% of the patients compared with 41.2% of the controls. This difference was due to an increased frequency of C4B*Q0 allele in the HSP group (0.25 versus 0.11 in the control group; P = 0.002). The fact that the majority of our patients carried a C4 null allele indicates that children with C4 deficiencies may have an increased risk of developing HSP. This may reflect inadequate complement activity and possibly present an opportunity to identify patients at risk of developing serious morbidity associated with HSP.
dc.language.isoenen
dc.publisherBlackwell Scientific Publicationsen
dc.relation.urlhttp://www.blackwell-synergy.com/doi/full/10.1111/j.1365-3083.2005.01533.xen
dc.subjectAllelesen
dc.subjectCase-Control Studiesen
dc.subjectChilden
dc.subjectComplement C4ben
dc.subjectGene Frequencyen
dc.subjectImmunoglobulin Aen
dc.subjectMannose-Binding Lectinen
dc.subjectMutationen
dc.subjectPurpura, Schoenlein-Henochen
dc.titleIncreased frequency of C4B*Q0 alleles in patients with Henoch-Schönlein purpuraen
dc.typeArticleen
dc.format.digYES
html.description.abstractHenoch-Schonlein purpura (HSP) is a vasculitis of unknown aetiology, possibly involving immune complexes. The complement system is essential for the clearance of immune complexes. Our aim was to explore the hypothesis that patients with HSP have abnormal complements, contributing to the development of the disease. The study included 56 patients diagnosed with HSP at the Children's Hospital, Iceland between 1984 and 2000, and 98 blood donors as controls. Serum levels of immunoglobulin A, C4A, C4B and mannan-binding lectin were measured and compared between the two groups. C4 null alleles were significantly more common in HSP patients than in controls (P = 0.018) and were carried by 66.1% of the patients compared with 41.2% of the controls. This difference was due to an increased frequency of C4B*Q0 allele in the HSP group (0.25 versus 0.11 in the control group; P = 0.002). The fact that the majority of our patients carried a C4 null allele indicates that children with C4 deficiencies may have an increased risk of developing HSP. This may reflect inadequate complement activity and possibly present an opportunity to identify patients at risk of developing serious morbidity associated with HSP.


This item appears in the following Collection(s)

Show simple item record