摘要
目的探讨抗中性粒细胞胞浆抗体(ANCA)相关小血管炎的临床表现。方法回顾性分析3例ANCA相关小血管炎患儿的临床资料。结果 3例患儿均为女性。2例环核型ANCA(P-ANCA)、抗髓过氧化物酶(MPO)抗体均为阳性;1例P-ANCA阳性,但未作MPO检查。3例患儿均出现肾损害,其中例1患儿出现严重肺出血。例1患儿接受大剂量甲基泼尼松龙及环磷酰胺治疗,但因发生慢性肾衰竭而只能维持肾脏替代治疗;例2经皮质激素联合吗替麦考酚酯治疗,病情得到控制;例3停用丙基硫氧嘧啶并予小剂量激素口服,病情得到控制。结论儿童ANCA相关性血管炎以女性多见,以P-ANCA阳性为主。早期诊断、合理使用皮质激素及联合免疫抑制剂是改善其预后的重要措施。
Objective To explore the clinical features of anti-neutrophil cytoplasmic antibody(ANCA)associated vasculitis.Methods The clinical records of 3 children with ANCA-associated vasculitis who were hospitalized after 2007 were retrospectively reviewed.Results The 3 children were all girls.Two children were both perinuclear ANCA(P-ANCA)and myeloperoxidase ANCA(MPO-ANCA)positive by serological test.Another child was P-ANCA positive,but MPO-ANCA was not checked.All 3 children suffered with kidney damage.Furthermore,case 1 showed severe pulmonary hemorrhage.After the treatment,although the fatal pulmonary hemorrhage was resolved,case 1 displayed chronic kidney disease which necessitated renal replacement therapy.Case 2 entered into remission by the treatment of steroids combined with Mycophenolate Mofetil.Case 3 also entered into remission by withdraw of propylthi-ouracil and resorting to low-dose steroids.Conclusions Girls account for a large part of ANCA-associated vasculitis in children,and the serological results was most likely to be P-ANCA positive.Early diagnosis,rational use of corticosteroids and immunosuppressive agents are important measures to improve the prognosis of ANCA-associated vasculitis.
出处
《临床儿科杂志》
CAS
CSCD
北大核心
2012年第4期312-315,共4页
Journal of Clinical Pediatrics
基金
国家自然科学基金资助项目(No.30971365
81070561
81170664)
卫生部科学研究基金-浙江省医药卫生重大科技计划(No.WKJ2010-2-014)
关键词
抗中性粒细胞胞浆抗体
血管炎
激素
免疫抑制剂
儿童
anti-neutrophil cytoplasmic antibody
vasculitis
corticosteroids
immuno-suppressive agents
child