Clinical and pathological analysis of Wegener granulomatosis in children
- VernacularTitle:儿童韦格纳肉芽肿的临床和病理分析
- Author:
Yuhong TAO
;
Songhui ZHAI
;
Zheng WANG
- Publication Type:Journal Article
- Keywords:
Wegener granulomatosis;
Pathology;
Antineutrophilcytoplasmic antibody
- From:
Chinese Pediatric Emergency Medicine
2007;14(z1):28-30
- CountryChina
- Language:Chinese
-
Abstract:
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.
