Abstract:
BACKGROUND/PURPOSE:To assess EDI-OCT (enhanced depth imaging optical coherence tomography) of choroid for inflammatory signs in children with polyarteritis nodosa (PAN) and adenosine deaminase-2 deficiency (DADA-2). METHODS:In this cross-sectional study conducted between June 2017 and September 2018, we evaluated children diagnosed with PAN (n = 11) and DADA-2 (n = 4) and an age- and sex-matched control group (n = 15). Demographic and laboratory data were retrospectively analyzed from patient charts. Disease activity was assessed using the pediatric vasculitis activity score (PVAS). Choroidal images were obtained with spectral domain-OCT to measure choroidal thickness (ChT) at 5 points (750 and 1500 μm from the foveal center in the temporal and nasal quadrants and beneath the fovea), and to calculate the total subfoveal choroidal area (TCA), luminal area (LA), stromal area (SA), and the choroidal vascularity index (CVI). RESULTS:The median (min-max) age was 8 (4-16) years in PAN patients, 6 (5-16) years in DADA-2 patients and 8 (8-10) years in control group at the OCT visit (p = 0.214). The ChT at 3 points and the TCA, LA, and SA were higher in children with both PAN and DADA-2 patients compared to those of the control group (p < 0.0001, p = 0.049, p = 0.007, p = 0.007, p = 0.006, p = 0.033, respectively). The CVI was similar in both groups. No association was observed between the OCT findings, PVAS, and the erythrocyte sedimentation rate, and serum leukocyte and C-reactive protein levels. CONCLUSION:Similar CVI scores were obtained from PAN and DADA2 patients under treatment and from healthy controls. Increased subfoveal ChT without any other signs of ocular involvement may suggest choroidal thickening as a sign of mild subclinical inflammation.
journal_name
Pediatr Rheumatol Online Jjournal_title
Pediatric rheumatology online journalauthors
Baytaroğlu A,Kadayifçilar S,Ağin A,Deliktaş Ö,Demir S,Bilginer Y,Karakaya J,Özen S,Eldem Bdoi
10.1186/s12969-020-0417-3subject
Has Abstractpub_date
2020-04-03 00:00:00pages
29issue
1issn
1546-0096pii
10.1186/s12969-020-0417-3journal_volume
18pub_type
杂志文章abstract:BACKGROUND:Due to an increased frequency of vasculitis in FMF patients, many investigators have studied MEFV mutations in patients with HSP. The aim of the study is to investigate the frequency and clinical significance of MEFV mutations in Egyptian children with Henoch-Schonlein purpura (HSP). Investigating MEFV mutat...
journal_title:Pediatric rheumatology online journal
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journal_title:Pediatric rheumatology online journal
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pub_type: 杂志文章,多中心研究
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journal_title:Pediatric rheumatology online journal
pub_type: 杂志文章
doi:10.1186/1546-0096-7-21
更新日期:2009-12-22 00:00:00
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journal_title:Pediatric rheumatology online journal
pub_type: 杂志文章
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journal_title:Pediatric rheumatology online journal
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doi:10.1186/s12969-017-0139-3
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journal_title:Pediatric rheumatology online journal
pub_type: 杂志文章
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pub_type: 杂志文章,评审
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pub_type: 杂志文章,评审
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更新日期:2021-01-06 00:00:00
abstract:BACKGROUND:Periodic fever syndromes (PFS) are an emerging group of autoinflammatory disorders. Clinical overlap exists and multiple genetic analyses may be needed to assist diagnosis. We evaluated the diagnostic value of a 5-gene sequencing panel (5GP) in patients with undiagnosed PFS. METHODS:Simultaneous double stra...
journal_title:Pediatric rheumatology online journal
pub_type: 杂志文章
doi:10.1186/s12969-015-0006-z
更新日期:2015-04-10 00:00:00
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更新日期:2015-12-09 00:00:00