FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2000378313> ?p ?o ?g. }

• W2000378313 abstract "Data on anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis are scarce in children. The current study is aimed at describing the clinical features and outcomes of childhood-onset ANCA-associated vasculitis (AAV). We conducted a retrospective French multicentre study involving patients in whom AAV was diagnosed before the age of 18 years. Inclusion criteria were (i) granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) according to classification criteria of the European League Against Rheumatism/Paediatric Rheumatology European Society, and (ii) ANCA positivity. Patient and renal survival were analysed. Among 66 children included, 80% were female, 42% had GPA and 58% MPA including renal-limited vasculitis, 67% were pANCA+ and 33% cANCA+. The mean incidence of reported cases increased to 0.45 per million children/year in the period 2006–10. Median age at diagnosis was 11.5 years, and median time to diagnosis was 1 month. Initial symptoms included fever and fatigue (79%), skin lesions (41%), arthritis (42%), pulmonary (45%) and renal involvement (88%). Clinical features were similar between GPA and MPA with the exception of upper airway impairment (28%) specific of GPA. Ninety percent of the patients achieved remission after induction treatment. After a median follow-up of 5.2 years, 4 patients (6%) died, corresponding to a mortality rate of 1.2 per 100 person-years, and 22 patients (34%) developed end-stage renal disease (ESRD). Renal survival was 74, 70 and 59% at 1, 5 and 10 years, respectively. In a multivariable Cox regression model, baseline glomerular filtration rate, ethnic origin, histopathological classification and era of treatment were associated with the occurrence of ESRD. Relapse-free survival was 57% at 5 years and 34% at 10 years of follow-up. Patient and renal outcome did not significantly differ between GPA and MPA. Childhood-onset AAV is a rare disease characterized by female predominance, delayed diagnosis, frequent renal impairment and a high remission rate. Baseline GFR and new histopathological classification system are strong predictors of ESRD. Renal survival in childhood AAV has improved over time." @default.
• W2000378313 created "2016-06-24" @default.
• W2000378313 creator A5000227486 @default.
• W2000378313 creator A5005438847 @default.
• W2000378313 creator A5015013487 @default.
• W2000378313 creator A5016762535 @default.
• W2000378313 creator A5017688158 @default.
• W2000378313 creator A5018486655 @default.
• W2000378313 creator A5024527923 @default.
• W2000378313 creator A5030656677 @default.
• W2000378313 creator A5043853010 @default.
• W2000378313 creator A5045427122 @default.
• W2000378313 creator A5046470677 @default.
• W2000378313 creator A5056689111 @default.
• W2000378313 creator A5064426652 @default.
• W2000378313 creator A5067851551 @default.
• W2000378313 creator A5071280031 @default.
• W2000378313 creator A5072330243 @default.
• W2000378313 creator A5073872127 @default.
• W2000378313 creator A5077369264 @default.
• W2000378313 creator A5079738832 @default.
• W2000378313 creator A5082190748 @default.
• W2000378313 creator A5084629812 @default.
• W2000378313 creator A5085454131 @default.
• W2000378313 creator A5086162029 @default.
• W2000378313 creator A5089769251 @default.
• W2000378313 creator A5091575574 @default.
• W2000378313 date "2015-02-12" @default.
• W2000378313 modified "2023-10-18" @default.
• W2000378313 title "Clinical characteristics and outcomes of childhood-onset ANCA-associated vasculitis: a French nationwide study" @default.
• W2000378313 cites W112413324 @default.
• W2000378313 cites W1492885539 @default.
• W2000378313 cites W1545696043 @default.
• W2000378313 cites W1596300271 @default.
• W2000378313 cites W1963501428 @default.
• W2000378313 cites W1963941288 @default.
• W2000378313 cites W1969171525 @default.
• W2000378313 cites W1969399531 @default.
• W2000378313 cites W1969760113 @default.
• W2000378313 cites W1979398789 @default.
• W2000378313 cites W1981535363 @default.
• W2000378313 cites W1981991010 @default.
• W2000378313 cites W1999520120 @default.
• W2000378313 cites W2001904865 @default.
• W2000378313 cites W2002680856 @default.
• W2000378313 cites W2005655317 @default.
• W2000378313 cites W2011596425 @default.
• W2000378313 cites W2019404620 @default.
• W2000378313 cites W2024564671 @default.
• W2000378313 cites W2025568493 @default.
• W2000378313 cites W2026118440 @default.
• W2000378313 cites W2037547791 @default.
• W2000378313 cites W2055864845 @default.
• W2000378313 cites W2059058890 @default.
• W2000378313 cites W2063340779 @default.
• W2000378313 cites W2064011553 @default.
• W2000378313 cites W2068895295 @default.
• W2000378313 cites W2070532418 @default.
• W2000378313 cites W2080567030 @default.
• W2000378313 cites W2083728839 @default.
• W2000378313 cites W2084056567 @default.
• W2000378313 cites W2085003457 @default.
• W2000378313 cites W2088972947 @default.
• W2000378313 cites W2090622672 @default.
• W2000378313 cites W2092644774 @default.
• W2000378313 cites W2099182695 @default.
• W2000378313 cites W2099989381 @default.
• W2000378313 cites W2101285203 @default.
• W2000378313 cites W2110189582 @default.
• W2000378313 cites W2120427700 @default.
• W2000378313 cites W2122855609 @default.
• W2000378313 cites W2126960177 @default.
• W2000378313 cites W2127458626 @default.
• W2000378313 cites W2128348682 @default.
• W2000378313 cites W2130053620 @default.
• W2000378313 cites W2130911389 @default.
• W2000378313 cites W2132795660 @default.
• W2000378313 cites W2132910340 @default.
• W2000378313 cites W2140431074 @default.
• W2000378313 cites W2145583696 @default.
• W2000378313 cites W2155005915 @default.
• W2000378313 cites W2165269524 @default.
• W2000378313 cites W2169096025 @default.
• W2000378313 cites W4229875030 @default.
• W2000378313 cites W4252638651 @default.
• W2000378313 doi "https://doi.org/10.1093/ndt/gfv011" @default.
• W2000378313 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/25676121" @default.
• W2000378313 hasPublicationYear "2015" @default.
• W2000378313 type Work @default.
• W2000378313 sameAs 2000378313 @default.
• W2000378313 citedByCount "39" @default.
• W2000378313 countsByYear W20003783132015 @default.
• W2000378313 countsByYear W20003783132016 @default.
• W2000378313 countsByYear W20003783132017 @default.
• W2000378313 countsByYear W20003783132018 @default.
• W2000378313 countsByYear W20003783132019 @default.
• W2000378313 countsByYear W20003783132020 @default.
• W2000378313 countsByYear W20003783132021 @default.
• W2000378313 countsByYear W20003783132022 @default.
• W2000378313 countsByYear W20003783132023 @default.

• next