Retrospective clinical features and renal pathological analysis of 15 children with anti-neutrophil cytoplasmic antibody-associated vasculitis

OBJECTIVE: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a disorder with poor prognosis. This study aimed to improve the diagnosis and treatment of ANCA associated vasculitis of children, to analyze the clinical features, pathological characteristics and the prognosis of child...

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Publié dans:Zhonghua er ke za zhi = Chinese journal of pediatrics. - 1960. - 51(2013), 4 vom: 12. Apr., Seite 283-7
Auteur principal: Guan, Na (Auteur)
Autres auteurs: Yao, Yong, Yang, Ji-Yun, Xiao, Hui-Jie, Ding, Jie
Format: Article
Langue:Chinese
Publié: 2013
Accès à la collection:Zhonghua er ke za zhi = Chinese journal of pediatrics
Sujets:English Abstract Journal Article Antibodies, Antineutrophil Cytoplasmic Creatinine AYI8EX34EU
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245 1 0 |a Retrospective clinical features and renal pathological analysis of 15 children with anti-neutrophil cytoplasmic antibody-associated vasculitis 
264 1 |c 2013 
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520 |a OBJECTIVE: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a disorder with poor prognosis. This study aimed to improve the diagnosis and treatment of ANCA associated vasculitis of children, to analyze the clinical features, pathological characteristics and the prognosis of children with ANCA-associated vasculitis 
520 |a METHOD: Fifteen children with ANCA associated vasculitis who were hospitalized from 2003 to 2012 in our hospital were included. Their data of pre-diagnosis status, clinical manifestations, renal pathology, treatment and prognosis were reviewed retrospectively 
520 |a RESULT: Of the 15 children, 11 were girls and 4 boys with a mean age of 10.7 years. Fourteen children were categorized as microscopic polyangitis. The time to diagnosis varied from 0.5 month to 40 months. Hematuria and proteinuria were revealed by urine analysis in all of them, only 6 children complained with gross hematuria or edema of oliguria. Decreased glomerular filtration rate was revealed in 13 children, 8 of whom had a creatinine clearance rate of less than 15 ml/(min·1.73 m(2)). Twelve children underwent renal biopsy, crescent formation was found in 11 children. Most of the crescents were cellular fibrous crescents or fibrous crescents. Six children were diagnosed as crescentic nephritis; the process of rapidly progressive nephritis was only observed in 2 children. Segmental glomerulosclerosis or global glomerulosclerosis were found in 10 children, 3 of them were diagnosed as sclerotic glomerulonephritis. Anemia and pulmonary injury were the most common extra renal manifestations. Other extra renal manifestations included rash, pain joint, gastrointestinal symptoms, abnormal findings of cardiac ultrasonography and headache. Eight children were treated with steroid combined with cyclophosphamide, 4 were treated with steroid and mycophenolate mofetil, 2 were treated with steroid, cyclophosphamide and mycophenolate mofetil, 3 children were treated with plasma exchange. Fourteen children were followed up for 0.5 month to 4 years. The renal function did not recover in children with creatinine clearance rate of less than 30 ml/(min·1.73 m(2)), who showed crescentic glomerulonephritis or sclerotic glomerulonephritis. The children who had creatinine clearance rate of more than 30 ml/(min·1.73 m(2))had better prognosis 
520 |a CONCLUSION: More attention should be paid to ANCA-associated vasculitis among school age girls with anemia or pulmonary diseases. The renal damage was serious in children; however, the clinical manifestations were not obvious. Children with a creatinine clearance rate of less than 30 ml/(min·1.73 m(2)) had poor prognosis. Early accurate diagnosis is very important 
650 4 |a English Abstract 
650 4 |a Journal Article 
650 7 |a Antibodies, Antineutrophil Cytoplasmic  |2 NLM 
650 7 |a Creatinine  |2 NLM 
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700 1 |a Yao, Yong  |e verfasserin  |4 aut 
700 1 |a Yang, Ji-Yun  |e verfasserin  |4 aut 
700 1 |a Xiao, Hui-Jie  |e verfasserin  |4 aut 
700 1 |a Ding, Jie  |e verfasserin  |4 aut 
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