儿童抗中性细胞胞浆抗体相关的急进性肾炎临床分析

目的：了解儿童抗中性细胞胞浆抗体（ANCA）相关的急进性肾炎的临床特点。方法：对2001~2006年我院诊断的7例ANCA相关的急进性肾炎临床、实验室检查和肾脏活检病理资料进行分析。结果：7例病例中，女6例，男1例，平均年龄9岁。主诉不典型，临床症状呈多样性、非特异性。实验室普通检查缺乏诊断特异性：全部病人血沉增快，不同程度的尿素氮（BUN）、血肌酐（SCr）升高, 有贫血、血尿、蛋白尿；C3正常3例、C3轻微下降4例；尿蛋白电泳分析呈混合性蛋白尿。实验室特殊检查：7例患儿血髓过氧化物酶（MPO），p-ANCA阳性，1例同时合并蛋白酶3-（PR3），c-ANCA阳性。肾活检病理特点：广泛的新月体形成,伴有肾小球毛细血管袢坏死。间质大量炎细胞浸润,间质小血管内皮细胞肿胀，管壁水肿或坏死。免疫荧光肾小球及血管壁无或有少量免疫复合物沉积。3例经甲基泼尼松及环磷酰胺冲击等治疗后追踪观察肾功能正常，血尿、蛋白尿消失或微量。结论：该病就诊症状多样、呈非特异性，易误诊。积极开展血清学ANCA检查及肾活检有助于早期诊断,早期治疗,改善预后。

Antineutrophil cytoplasmic autoantibody-associated rapidly progressive glomerulonephritis in children

Objective To investigate the clinical characteristics of childhood antineutrophil cytoplasmic autoantibody (ANCA)-associated rapidly progressive glomerulonephritis.Methods The medical data, including clinical manifestations, laboratory findings, and kidney pathology, of 7 children with ANCA-associated rapidly progressive glomerulonephritis were retrospectively studied. Results The 7 patients (6 girls and 1 boy) ranged in age from 3.5-14 years, with a mean age of 9 years. A diversity of major complaints and clinical symptoms was presented in the patients. Laboratory findings were not specific. All patients had elevated ESR, BUN and serum creatinine levels as well as anaemia, hematuria and proteinuria. Urinary protein electrophoresis showed mixed proteinuria in the 7 cases. C3 was normal in 3 cases and slightly decreased in 4 cases. All were MPO-ANCA positive, and 1 out of the 7 cases was positive for PR3-ANCA. Renal biopsy displayed extensive crescentic formations and necrotic glomerulus capillary loop. A great quantity of inflammatory cell infiltration and swollen endotheliocytes of small vessels as well as vessel wall edema or necrosis were found in the interstitium. Immunofluorescence showed no or little amounts of immune complex depositions in the renal glomeruli and vessel walls. Renal function was recovered and hematuria/proteinuria disappeared or greatly relieved in 3 patients after methylprednisone and cyclophosphamide pulse therapy.Conclusions Children with ANCA-positive rapidly progressive glomerulonephritis present with various clinical manifestations. The diagnosis of this disorder may be difficult due to a lack of specificity in its clinical manifestations. It is important to enhance our understanding of this disorder to effectively make an early diagnosis.