Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F14%3A10286343" target="_blank" >RIV/00216208:11110/14:10286343 - isvavai.cz</a>
Alternative codes found
RIV/00064165:_____/14:10286343
Result on the web
<a href="http://dx.doi.org/10.1186/1546-0096-12-18" target="_blank" >http://dx.doi.org/10.1186/1546-0096-12-18</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1186/1546-0096-12-18" target="_blank" >10.1186/1546-0096-12-18</a>
Alternative languages
Result language
angličtina
Original language name
Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)
Original language description
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 {= 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 atthe 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%) witha 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 los
Czech name
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Czech description
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Classification
Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
FG - Paediatrics
OECD FORD branch
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Result continuities
Project
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Continuities
S - Specificky vyzkum na vysokych skolach
Others
Publication year
2014
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Pediatric Rheumatology
ISSN
1546-0096
e-ISSN
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Volume of the periodical
12
Issue of the periodical within the volume
May
Country of publishing house
US - UNITED STATES
Number of pages
5
Pages from-to
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UT code for WoS article
000336749400001
EID of the result in the Scopus database
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