中国儿童IgA肾病治疗现状多中心回顾性研究

Multicenter investigation of therapeutic status of children with IgA nephropathy in China

目的 中国儿童IgA肾病治疗现状尚不明确,本研究探讨中国儿童IgA肾病治疗现状,为本病的治疗以及进一步研究提供基本数据.方法 由中华医学会儿科学分会肾脏病学组组织全国多家三级甲等医院,对2008年7月1日至2011年6月30日收治的18岁及以下经肾脏病理确诊的IgA肾病患儿进行调查,收集一般信息、临床表型及治疗状况,分析治疗状况.结果 共35家三级甲等医院参加了此次调研,共纳入1417例,男952例（67.2％）,女465例（32.8％）,年龄0.5～18.0岁,中位年龄10.0岁.临床表型依次为血尿和蛋白尿型524例（37.0％）、肾病综合征型433例（30.6％）、孤立性血尿型224例（15.8％）、急性肾小球肾炎型180例（12.7％）、慢性肾小球肾炎型26例（1.8％）、急进性肾小球肾炎19例（1.3％）、孤立性蛋白尿11例（0.8％）.血尿和蛋白尿型的主要用药依次为血管紧张素转换酶抑制剂（ACEI）和（或）血管紧张素受体拮抗剂（ARB）类药物（49.6％）、口服激素（41.4％）、静脉甲泼尼龙（20.8％）、雷公藤制剂（18.7％）、环磷酰胺（11.3％）等.肾病综合征型的主要用药依次为口服激素（76.2％）、ACEI和（或）ARB类药物（60.5％）、环磷酰胺（40.0％）、静脉甲泼尼龙（31.2％）和雷公藤制剂（10.6％）等.结论 本研究为国内首次进行的全国范围儿童IgA肾病治疗现状调研,对于肾病综合征型的治疗分别以激素、ACEI和（或）ARB和环磷酰胺为主,激素和免疫抑制剂对于其他类型IgA肾病的应用和适应证有待研究.

Objective Data of general status of clinical presentation and therapy of IgA nephropathy in children in China are lacking.This study aimed to investigate the clinical presentations and treatment status of children with IgA nephropathy in China.Method The investigation was organized by Chinese Society of Pediatric Nephrology.Thirty five centers from 23 provinces/municipalities of China participated in the study.Children no more than 18 years old who were diagnosed by renal biopsy as IgA nephropathy from July 1,2008 to June 20,2011 were included.The data on clinical presentations and treatment methods were collected.Descriptive method was used for data analysis.Result Totally 1417 children aged from 0.5 to 18 years old were included.The median age was 10.0 years old.Children more than 6 years old accounted for 89.8%.Most of the children presented with hematuria and proteinuria （37.0%）,nephrotic syndrome type accounted for 30.6%,isolated hematuria type accounted for 15.8%,acute glomerulonephritis type accounteed for 12.7%,chronic glomerulonephritis type accounted for 1.8%,acute rapidly progressive glomerulonephritis type accounted for 1.3%,isolated proteinuria type accounted for 0.8%.The common drug used for children with hematuria and proteinuria type included angiotensin-converting enzyme inhibitor （ACEI）/angiotensin Ⅱ receptor antagonists （ARB） （49.6%）,oral prednisone （41.4%）,intravenous methylprednisolone （20.8%）,etc.The therapies with oral prednisone （76.2%）,ACEI/ARB （60.5%）,cyclophosphamide （40.0%）,intravenous methylprednisolone （31.2%） were more often used for children with nephrotic syndrome type.Conclusion This is the first nationwide investigation on therapy status of children with IgA nephropathy in China.Steroid combined with ACEI/ARB and cyclophosphamide was the main treatment for children with nephrotic syndrome type.The effect of steroid combined with immunosuppressant on other types of IgA nephropathy needs further investigation.