中国小儿急救医学
中國小兒急救醫學
중국소인급구의학
CHINESE PEDIATRIC EMERGENCY MEDICINE
2007年
z1期
28-30
,共3页
韦格纳肉芽肿%病理学%抗中性粒细胞胞浆抗体
韋格納肉芽腫%病理學%抗中性粒細胞胞漿抗體
위격납육아종%병이학%항중성립세포포장항체
Wegener granulomatosis%Pathology%Antineutrophilcytoplasmic antibody
目的 探讨儿童韦格纳肉芽肿(WG)的临床和病理特点.方法 收集本院2例和全国其他7例WG患儿,对其临床表现、实验室检查、影像学特点以及治疗进行分析.结果 9例患儿中男6例,女3例,平均年龄12岁,从发病到确诊的平均时间为8.3个月.有多系统、器官受累,以肾脏和肺最为常见.7例患儿进行抗中性粒细胞胞浆抗体(ANCA)检查,c-ANCA和抗PR3抗体阳性6例.8例患儿进行了组织活检,病理表现有实质组织坏死、肉芽肿和血管炎等.多数患儿接受糖皮质激素和环磷酰胺治疗后病情明显改善.结论 WG可侵犯多个组织器官,临床表现缺乏特异性,ANCA检查是早期诊断的关键.
目的 探討兒童韋格納肉芽腫(WG)的臨床和病理特點.方法 收集本院2例和全國其他7例WG患兒,對其臨床錶現、實驗室檢查、影像學特點以及治療進行分析.結果 9例患兒中男6例,女3例,平均年齡12歲,從髮病到確診的平均時間為8.3箇月.有多繫統、器官受纍,以腎髒和肺最為常見.7例患兒進行抗中性粒細胞胞漿抗體(ANCA)檢查,c-ANCA和抗PR3抗體暘性6例.8例患兒進行瞭組織活檢,病理錶現有實質組織壞死、肉芽腫和血管炎等.多數患兒接受糖皮質激素和環燐酰胺治療後病情明顯改善.結論 WG可侵犯多箇組織器官,臨床錶現缺乏特異性,ANCA檢查是早期診斷的關鍵.
목적 탐토인동위격납육아종(WG)적림상화병리특점.방법 수집본원2례화전국기타7례WG환인,대기림상표현、실험실검사、영상학특점이급치료진행분석.결과 9례환인중남6례,녀3례,평균년령12세,종발병도학진적평균시간위8.3개월.유다계통、기관수루,이신장화폐최위상견.7례환인진행항중성립세포포장항체(ANCA)검사,c-ANCA화항PR3항체양성6례.8례환인진행료조직활검,병리표현유실질조직배사、육아종화혈관염등.다수환인접수당피질격소화배린선알치료후병정명현개선.결론 WG가침범다개조직기관,림상표현결핍특이성,ANCA검사시조기진단적관건.
Objective To analyze the clinical and pathological features of Wegener granulomatosis (WG) in children in order to improve the understanding of this disease. Methods Nine cases of WG,including two cases from our hospital and seven cases throughout the country,were analyzed in aspects of their clinical characteristics,laboratory examinations,X-ray and treatments. Results Of the nine cases,six cases were male and three cases were female with average age of twelve years old. The average time from the initiation of clinical symptom to diagnosis was 8.3 months. Patients had multiple systems and organs involvements. Lung and kidney involvement were the most common. Six out of seven were positive in c-ANCA and anti-PR3 antibody. Biopsy was conducted in eight patients,and pathological manifestations involved parenchymal necrosis,granulomatosis,vasculitis and so on. Most patients improved significantly after being treated with glucocorticoids and cyclophosphamide. Conclusion WG is a syndrome which involves many organs without distinctive clinical manifestation. An ANCA test should be performed to make an early diagnosis.
