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Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review

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Resumo:Abstract Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis that primarily affects children. Major manifestations include purpuric rash, proteinuria/hematuria, arthralgia, and abdominal pain. In this article, the authors review data on the epidemiology, diagnosis, and treatment of IgAV in children. IgAV affects 10 to 56 children per 100,000 per year. The mean age at presentation is six years. Both genetic and environmental factors contribute to its pathogenesis, but the deposition of immune complexes containing abnormal glycosylated immunoglobulin (Ig) A1 plays a predominant role. The course of the disease is usually benign and supportive care is sufficient. Short-term complications are mostly related to gastrointestinal involvement, including the risk of perforation and bleeding. Long-term morbidity is due to chronic kidney disease and hypertension. Corticosteroids are not recommended for prevention of renal involvement, but may be useful as a treatment strategy, as well as more aggressive immunosuppressive drugs.
Autores principais:Sousa,Patrícia
Outros Autores:Oliveira,Susana Correia de; Dias,Ângela; Tavares,Cláudia
Assunto:child diagnosis epidemiology IgA vasculitis physiopathology therapy
Ano:2023
País:Portugal
Tipo de documento:artigo
Tipo de acesso:acesso aberto
Instituição associada:Fundação para a Ciência e Tecnologia
Idioma:inglês
Origem:SciELO Portugal
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author Sousa,Patrícia
author2 Oliveira,Susana Correia de
Dias,Ângela
Tavares,Cláudia
author2_role author
author
author
author_facet Sousa,Patrícia
Oliveira,Susana Correia de
Dias,Ângela
Tavares,Cláudia
author_role author
country_str PT
creators_json_txt [{\"Person.name\":\"Sousa,Patrícia\"},{\"Person.name\":\"Oliveira,Susana Correia de\"},{\"Person.name\":\"Dias,Ângela\"},{\"Person.name\":\"Tavares,Cláudia\"}]
datacite.creators.creator.creatorName.fl_str_mv Sousa,Patrícia
Oliveira,Susana Correia de
Dias,Ângela
Tavares,Cláudia
datacite.rights.fl_str_mv http://purl.org/coar/access_right/c_abf2
datacite.subjects.subject.fl_str_mv child
diagnosis
epidemiology
IgA vasculitis
physiopathology
therapy
datacite.titles.title.fl_str_mv Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
dc.creator.none.fl_str_mv Sousa,Patrícia
Oliveira,Susana Correia de
Dias,Ângela
Tavares,Cláudia
dc.format.none.fl_str_mv text/html
dc.identifier.none.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542023000300205
dc.language.none.fl_str_mv eng
dc.publisher.none.fl_str_mv Centro Hospitalar do Porto
dc.rights.none.fl_str_mv http://purl.org/coar/access_right/c_abf2
dc.source.none.fl_str_mv Nascer e Crescer v.32 n.3 2023
dc.subject.none.fl_str_mv child
diagnosis
epidemiology
IgA vasculitis
physiopathology
therapy
dc.title.fl_str_mv Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
dc.type.none.fl_str_mv http://purl.org/coar/resource_type/c_6501
description Abstract Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis that primarily affects children. Major manifestations include purpuric rash, proteinuria/hematuria, arthralgia, and abdominal pain. In this article, the authors review data on the epidemiology, diagnosis, and treatment of IgAV in children. IgAV affects 10 to 56 children per 100,000 per year. The mean age at presentation is six years. Both genetic and environmental factors contribute to its pathogenesis, but the deposition of immune complexes containing abnormal glycosylated immunoglobulin (Ig) A1 plays a predominant role. The course of the disease is usually benign and supportive care is sufficient. Short-term complications are mostly related to gastrointestinal involvement, including the risk of perforation and bleeding. Long-term morbidity is due to chronic kidney disease and hypertension. Corticosteroids are not recommended for prevention of renal involvement, but may be useful as a treatment strategy, as well as more aggressive immunosuppressive drugs.
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person_str_mv Sousa,Patrícia
Oliveira,Susana Correia de
Dias,Ângela
Tavares,Cláudia
publishDate 2023
publisher.none.fl_str_mv Centro Hospitalar do Porto
reponame_str SciELO Portugal
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spelling Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature ReviewSousa,PatríciaOliveira,Susana Correia deDias,ÂngelaTavares,CláudiachilddiagnosisepidemiologyIgA vasculitisphysiopathologytherapyopen accesshttp://purl.org/coar/access_right/c_abf2http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542023000300205URLhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542023000300205URLHasVersion2023-09-01Abstract Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis that primarily affects children. Major manifestations include purpuric rash, proteinuria/hematuria, arthralgia, and abdominal pain. In this article, the authors review data on the epidemiology, diagnosis, and treatment of IgAV in children. IgAV affects 10 to 56 children per 100,000 per year. The mean age at presentation is six years. Both genetic and environmental factors contribute to its pathogenesis, but the deposition of immune complexes containing abnormal glycosylated immunoglobulin (Ig) A1 plays a predominant role. The course of the disease is usually benign and supportive care is sufficient. Short-term complications are mostly related to gastrointestinal involvement, including the risk of perforation and bleeding. Long-term morbidity is due to chronic kidney disease and hypertension. Corticosteroids are not recommended for prevention of renal involvement, but may be useful as a treatment strategy, as well as more aggressive immunosuppressive drugs.Centro Hospitalar do PortoNascer e Crescer v.32 n.3 2023text/htmlengjournal articlehttp://purl.org/coar/resource_type/c_6501literature
spellingShingle Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
Sousa,Patrícia
child
diagnosis
epidemiology
IgA vasculitis
physiopathology
therapy
status SINGLETON
subject.fl_str_mv child
diagnosis
epidemiology
IgA vasculitis
physiopathology
therapy
title Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
title_full Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
title_fullStr Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
title_full_unstemmed Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
title_short Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
title_sort Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
topic child
diagnosis
epidemiology
IgA vasculitis
physiopathology
therapy
topic_facet child
diagnosis
epidemiology
IgA vasculitis
physiopathology
therapy
url http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542023000300205
visible 1