Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)

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dc.contributor.authorBohm, Marek
dc.contributor.authorFernandez, Maria Isabel Gonzalez
dc.contributor.authorOzen, Seza
dc.contributor.authorPistorio, Angela
dc.contributor.authorDolezalova, Pavla
dc.contributor.authorBrogan, Paul
dc.contributor.authorBarbano, Giancarlo
dc.contributor.authorSengler, Claudia
dc.contributor.authorKlein-Gitelman, Marisa
dc.contributor.authorQuartier, Pierre
dc.contributor.authorFasth, Anders
dc.contributor.authorHerlin, Troels
dc.contributor.authorTerreri, Maria Teresa R. A. [UNIFESP]
dc.contributor.authorNielsen, Susan
dc.contributor.authorvan Rossum, Marion A. J.
dc.contributor.authorAvcin, Tadej
dc.contributor.authorRodolfo Castell, Esteban
dc.contributor.authorFoeldvari, Ivan
dc.contributor.authorFoell, Dirk
dc.contributor.authorKondi, Anuela
dc.contributor.authorKone-Paut, Isabelle
dc.contributor.authorKuester, Rolf-Michael
dc.contributor.authorMichels, Hartmut
dc.contributor.authorWulffraat, Nico
dc.contributor.authorBen Amer, Halima
dc.contributor.authorMalattia, Clara
dc.contributor.authorMartini, Alberto
dc.contributor.authorRuperto, Nicolino
dc.contributor.authorPaediat Rheumatology Int
dc.contributor.institutionPRINTO
dc.contributor.institutionCharles Univ Prague
dc.contributor.institutionGen Univ Hosp Prague
dc.contributor.institutionHacettepe Univ
dc.contributor.institutionIst Giannina Gaslini
dc.contributor.institutionNHS Fdn Trust
dc.contributor.institutionCharite
dc.contributor.institutionAnn & Robert H Lurie Childrens Hosp Chicago
dc.contributor.institutionUniv Paris 05
dc.contributor.institutionUniv Gothenburg
dc.contributor.institutionAarhus Univ Hosp
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.contributor.institutionRigshosp
dc.contributor.institutionEmma Children Hosp AMC
dc.contributor.institutionUniv Childrens Hosp
dc.contributor.institutionHosp Dr Felipe Glasman
dc.contributor.institutionKlinikum Eilbek Hs 6
dc.contributor.institutionUniv Hosp Ctr
dc.contributor.institutionUniv Paris 11
dc.contributor.institutionAsklepios Klin Altona
dc.contributor.institutionKinderklin Garmisch Partenkirchen gGmbH
dc.contributor.institutionWilhelmina Childrens Hosp
dc.contributor.institutionBenghazi Children Hosp Benghazi
dc.contributor.institutionUniv Genoa
dc.date.accessioned2016-01-24T14:37:18Z
dc.date.available2016-01-24T14:37:18Z
dc.date.issued2014-05-26
dc.description.abstractBackground: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA paediatric patients.Children with age at diagnosis <= 18 years, fulfilling the EULAR/PRINTO/PRES GPA/WG classification criteria were extracted from the PRINTO vasculitis database. the clinical signs/symptoms and laboratory features were analysed before or at the time of diagnosis and at least 3 months thereafter and compared with other paediatric and adult case series (>50 patients) derived from the literature.Findings: the 56 children with GPA/WG were predominantly females (68%) and Caucasians (82%) with a median age at disease onset of 11.7 years, and a median delay in diagnosis of 4.2 months. the most frequent organ systems involved before/at the time of diagnosis were ears, nose, throat (91%), constitutional (malaise, fever, weight loss) (89%), respiratory (79%), mucosa and skin (64%), musculoskeletal (59%), and eye (35%), 67% were ANCA-PR3 positive, while haematuria/proteinuria was present in > 50% of the children. in adult series, the frequency of female involvement ranged from 29% to 50% with lower frequencies of constitutional (fever, weight loss), ears, nose, throat (oral/nasal ulceration, otitis/aural discharge), respiratory (tracheal/endobronchial stenosis/obstruction), laboratory involvement and higher frequency of conductive hearing loss than in this paediatric series.Conclusions: Paediatric patients compared to adults with GPA/WG have similar pattern of clinical manifestations but different frequencies of organ involvement.en
dc.description.affiliationPRINTO, Ist Giannina Gaslini Pediat Reumatol 2, Genoa, Italy
dc.description.affiliationCharles Univ Prague, Fac Med 1, Prague, Czech Republic
dc.description.affiliationGen Univ Hosp Prague, Prague, Czech Republic
dc.description.affiliationHacettepe Univ, Childrens Hosp, Dept Pediat Rheumatol & Nephrol, Ankara, Turkey
dc.description.affiliationIst Giannina Gaslini, Serv Epidemiol & Biostat, I-16148 Genoa, Italy
dc.description.affiliationNHS Fdn Trust, Great Ormond St Hosp, Dept Rheumatol, London, England
dc.description.affiliationIst Giannina Gaslini, I-16148 Genoa, Italy
dc.description.affiliationCharite, Dept Pediat, Div Pneumol & Immunol, D-13353 Berlin, Germany
dc.description.affiliationAnn & Robert H Lurie Childrens Hosp Chicago, Chicago, IL USA
dc.description.affiliationUniv Paris 05, Unit Immunol Hematol & Rhumatol Pediat, Ctr Reference Natl Arthrit Juveniles, Hop Necker Enfants Malad,Inst IMAGINE, Paris, France
dc.description.affiliationUniv Gothenburg, Dept Pediat, Queen Silvia Childrens Hosp, Gothenburg, Sweden
dc.description.affiliationAarhus Univ Hosp, Skejby Sygehus, Dept Pediat, Pediat Rheumatol Clin, DK-8000 Aarhus, Denmark
dc.description.affiliationUniversidade Federal de São Paulo, São Paulo, Brazil
dc.description.affiliationRigshosp, Juliane Marie Centret, Pediat Klin 2, DK-2100 Copenhagen, Denmark
dc.description.affiliationEmma Children Hosp AMC, Dept Pediat, Amsterdam, Netherlands
dc.description.affiliationUniv Childrens Hosp, Univ Med Ctr Ljubljana, Dept Allergol Rheumatol & Clin Immunol, Ljubljana, Slovenia
dc.description.affiliationHosp Dr Felipe Glasman, Rheumatolgy Sect, Buenos Aires, DF, Argentina
dc.description.affiliationKlinikum Eilbek Hs 6, Hamburger Zentrum Kinder & Jugendrheumatol, Hamburg, Germany
dc.description.affiliationUniv Childrens Hosp, Dept Pediat Rheumatol & Immunol, Munster, Germany
dc.description.affiliationUniv Hosp Ctr, Dept Pediat, Tirana, Albania
dc.description.affiliationUniv Paris 11, APHP, CHU Le Kremlin Bicetre, CEREMAI Ctr Reference Natl Malad Autoinflammat Rh, Paris, France
dc.description.affiliationAsklepios Klin Altona, Hamburg, Germany
dc.description.affiliationKinderklin Garmisch Partenkirchen gGmbH, Deutsch Zentrum Kinder & Jugendrheumatol, Garmisch Partenkirchen, Germany
dc.description.affiliationWilhelmina Childrens Hosp, Dept Pediat Immunol & Rheumatol, Utrecht, Netherlands
dc.description.affiliationBenghazi Children Hosp Benghazi, MUB Rheumatol Clin, Benghazi, Libya
dc.description.affiliationUniv Genoa, Dipartimento Pediat, Genoa, Italy
dc.description.affiliationUnifespPediatrics, Universidade Federal de São Paulo, São Paulo, Brazil
dc.description.sourceWeb of Science
dc.description.sponsorshipIstituto G. Gaslini
dc.description.sponsorshipPaediatric Rheumatology International Trials Organisation (PRINTO), Genoa, Italy
dc.description.sponsorshipCharles University Grant Agency
dc.description.sponsorshipEuropean League Against Rheumatism (EULAR) Scientific Training Bursaries
dc.description.sponsorshipIDCharles University Grant Agency: GAUK 52608/2008
dc.format.extent5
dc.identifierhttp://dx.doi.org/10.1186/1546-0096-12-18
dc.identifier.citationPediatric Rheumatology. London: Biomed Central Ltd, v. 12, 5 p., 2014.
dc.identifier.doi10.1186/1546-0096-12-18
dc.identifier.fileWOS000336749400001.pdf
dc.identifier.issn1546-0096
dc.identifier.urihttp://repositorio.unifesp.br/handle/11600/37782
dc.identifier.wosWOS:000336749400001
dc.language.isoeng
dc.publisherBiomed Central Ltd
dc.relation.ispartofPediatric Rheumatology
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectWegener's granulomatosisen
dc.subjectGranulomatosis with polyangiitisen
dc.subjectClinical studyen
dc.subjectClinical picture of diseaseen
dc.subjectComparison with literatureen
dc.titleClinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)en
dc.typeinfo:eu-repo/semantics/article
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