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

Autor: Bohm, Marek, Fernandez, Maria Isabel Gonzalez, Ozen, Seza, Pistorio, Angela, Dolezalova, Pavla, Brogan, Paul, Barbano, Giancarlo, Sengler, Claudia, Klein-Gitelman, Marisa, Quartier, Pierre, Fasth, Anders, Herlin, Troels, Terreri, Maria Teresa R. A. [UNIFESP], Nielsen, Susan, van Rossum, Marion A. J., Avcin, Tadej, Rodolfo Castell, Esteban, Foeldvari, Ivan, Foell, Dirk, Kondi, Anuela, Kone-Paut, Isabelle, Kuester, Rolf-Michael, Michels, Hartmut, Wulffraat, Nico, Ben Amer, Halima, Malattia, Clara, Martini, Alberto, Ruperto, Nicolino, Paediat Rheumatology Int
Přispěvatelé: PRINTO, Charles Univ Prague, Gen Univ Hosp Prague, Hacettepe Univ, Ist Giannina Gaslini, NHS Fdn Trust, Charite, Ann & Robert H Lurie Childrens Hosp Chicago, Univ Paris 05, Univ Gothenburg, Aarhus Univ Hosp, Universidade Federal de São Paulo (UNIFESP), Rigshosp, Emma Children Hosp AMC, Univ Childrens Hosp, Hosp Dr Felipe Glasman, Klinikum Eilbek Hs 6, Univ Hosp Ctr, Univ Paris 11, Asklepios Klin Altona, Kinderklin Garmisch Partenkirchen gGmbH, Wilhelmina Childrens Hosp, Benghazi Children Hosp Benghazi, Univ Genoa, AII - Amsterdam institute for Infection and Immunity, Paediatric Infectious Diseases / Rheumatology / Immunology, Çocuk Sağlığı ve Hastalıkları
Jazyk: angličtina
Rok vydání: 2014
Předmět:
Zdroj: Repositório Institucional da UNIFESP
Universidade Federal de São Paulo (UNIFESP)
instacron:UNIFESP
Pediatric Rheumatology Online Journal
Pediatric rheumatology online journal, 12. BioMed Central
Bohm, M, Gonzalez Fernandez, M I, Ozen, S, Pistorio, A, Dolezalova, P, Brogan, P, Barbano, G, Sengler, C, Klein-Gitelman, M, Quartier, P, Fasth, A, Herlin, T, Terreri, M T R A, Nielsen, S, van Rossum, M A J, Avcin, T, Castell, E R, Foeldvari, I, Foell, D, Kondi, A, Koné-Paut, I, Kuester, R-M, Michels, H, Wulffraat, N, Amer, H B, Malattia, C, Martini, A & Ruperto, N 2014, ' Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis) ', Pediatric Rheumatology, vol. 12, pp. 18 . https://doi.org/10.1186/1546-0096-12-18
ISSN: 1546-0096
Popis: Istituto G. Gaslini Paediatric Rheumatology International Trials Organisation (PRINTO), Genoa, Italy Charles University Grant Agency European League Against Rheumatism (EULAR) Scientific Training Bursaries Background: 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 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. PRINTO, Ist Giannina Gaslini Pediat Reumatol 2, Genoa, Italy Charles Univ Prague, Fac Med 1, Prague, Czech Republic Gen Univ Hosp Prague, Prague, Czech Republic Hacettepe Univ, Childrens Hosp, Dept Pediat Rheumatol & Nephrol, Ankara, Turkey Ist Giannina Gaslini, Serv Epidemiol & Biostat, I-16148 Genoa, Italy NHS Fdn Trust, Great Ormond St Hosp, Dept Rheumatol, London, England Ist Giannina Gaslini, I-16148 Genoa, Italy Charite, Dept Pediat, Div Pneumol & Immunol, D-13353 Berlin, Germany Ann & Robert H Lurie Childrens Hosp Chicago, Chicago, IL USA Univ Paris 05, Unit Immunol Hematol & Rhumatol Pediat, Ctr Reference Natl Arthrit Juveniles, Hop Necker Enfants Malad,Inst IMAGINE, Paris, France Univ Gothenburg, Dept Pediat, Queen Silvia Childrens Hosp, Gothenburg, Sweden Aarhus Univ Hosp, Skejby Sygehus, Dept Pediat, Pediat Rheumatol Clin, DK-8000 Aarhus, Denmark Universidade Federal de São Paulo, São Paulo, Brazil Rigshosp, Juliane Marie Centret, Pediat Klin 2, DK-2100 Copenhagen, Denmark Emma Children Hosp AMC, Dept Pediat, Amsterdam, Netherlands Univ Childrens Hosp, Univ Med Ctr Ljubljana, Dept Allergol Rheumatol & Clin Immunol, Ljubljana, Slovenia Hosp Dr Felipe Glasman, Rheumatolgy Sect, Buenos Aires, DF, Argentina Klinikum Eilbek Hs 6, Hamburger Zentrum Kinder & Jugendrheumatol, Hamburg, Germany Univ Childrens Hosp, Dept Pediat Rheumatol & Immunol, Munster, Germany Univ Hosp Ctr, Dept Pediat, Tirana, Albania Univ Paris 11, APHP, CHU Le Kremlin Bicetre, CEREMAI Ctr Reference Natl Malad Autoinflammat Rh, Paris, France Asklepios Klin Altona, Hamburg, Germany Kinderklin Garmisch Partenkirchen gGmbH, Deutsch Zentrum Kinder & Jugendrheumatol, Garmisch Partenkirchen, Germany Wilhelmina Childrens Hosp, Dept Pediat Immunol & Rheumatol, Utrecht, Netherlands Benghazi Children Hosp Benghazi, MUB Rheumatol Clin, Benghazi, Libya Univ Genoa, Dipartimento Pediat, Genoa, Italy Pediatrics, Universidade Federal de São Paulo, São Paulo, Brazil Charles University Grant Agency: GAUK 52608/2008 Web of Science
Databáze: OpenAIRE
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