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学科主题: 临床医学
题名:
The clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in Chinese children
作者: Yu, F; Huang, JP; Zou, WZ; Zhao, MH
关键词: children ; vasculitis ; anti-neutrophil cytoplasmic antibodies (ANCAs)
刊名: PEDIATRIC NEPHROLOGY
发表日期: 2006-04-01
DOI: 10.1007/s00467-006-0028-3
卷: 21, 期:4, 页:497-502
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Pediatrics ; Urology & Nephrology
研究领域[WOS]: Pediatrics ; Urology & Nephrology
关键词[WOS]: CRESCENTIC GLOMERULONEPHRITIS ; MICROSCOPIC POLYANGIITIS ; NECROTIZING VASCULITIS ; AUTOANTIBODIES
英文摘要:

Anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis is reported mainly in adults. Studies in children are limited. The current study retrospectively analyzed the clinical characteristics and pathology of ANCA-associated systemic vasculitis in children in our hospital during the past 7 years. Twenty-four pediatric patients were diagnosed as having ANCA-associated systemic vasculitis, including 19 patients with microscopic polyangiitis (MPA), one with Wegener′s granulomatosis (WG), three with propylthiouracil (PTU)-induced ANCA-positive vasculitis and one with anti-glomerular basement membrane (GBM) disease. Of patients with primary ANCA-associated systemic vasculitis (MPA and WG), with an average age of 10.8 +/- 2.8 (6-14) years, 18 patients (90%) were female and two (10%) were male. Nineteen patients (95%) were p-ANCA/MPO-ANCA positive and one (5%) was c-ANCA/PR3-ANCA positive. The interval between onset and diagnosis was 8.5 +/- 24.3 (0.2-108) months. The majority of the patients (85%) had multi-organ involvement. All patients had clinical evidence of renal involvement and presented with hematuria and proteinuria. Of 20 patients, 16 (80%) also had acute renal failure, and five patients were dialysis dependent. Nine patients underwent renal biopsy and were diagnosed with necrotizing and crescentic glomerulonephritis. However, six biopsies showed immune complex deposition. All patients received immunosuppressive therapy including prednisone and cyclophosphamide, and ten patients also received intravenous administration of methylprednisone pulse therapy according to their clinical situation and renal pathology. Sixteen patients achieved clinical remission, and four patients presented as treatment failure. Patients were followed up for 12.3 +/- 5.1 months (median 12 months; range 1 to 91 months). Ten patients maintained their clinical remission, and ten progressed to renal failure requiring dialysis. Our study showed that the clinical features and pathology of primary ANCA-associated systemic vasculitis in children were similar to those of adults, but there were a predominance of female patients and late diagnoses. We suggest that early recognition and prompt aggressive treatment might improve outcome.

语种: 英语
WOS记录号: WOS:000236417600008
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/57628
Appears in Collections:北京大学第一临床医学院_肾脏内科_期刊论文

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作者单位: 1.Peking Univ, First Hosp, Dept Pediat, Beijing, Peoples R China
2.Peking Univ, First Hosp, Dept Nephrol, Beijing, Peoples R China
3.Peking Univ, First Hosp, Div Renal, Beijing 100034, Peoples R China
4.Peking Univ, First Hosp, Inst Nephrol, Beijing 100034, Peoples R China

Recommended Citation:
Yu, F,Huang, JP,Zou, WZ,et al. The clinical features of anti-neutrophil cytoplasmic antibody-associated systemic vasculitis in Chinese children[J]. PEDIATRIC NEPHROLOGY,2006,21(4):497-502.
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