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目的调查分析我国儿童原发性 IgA 肾病(IgAN)临床与病理特征,了解 IgAN 患儿临床、病理特征,以及治疗和转归情况。方法由中华医学会儿科学分会肾脏病学组统一制订调查表格,对经肾穿刺活检确诊的原发性 IgAN 患儿进行回顾性登记,由各地初步归纳分析,然后汇集并进行统计分析。结果 1995年1月至2004年12月全国33所医院≤14岁住院 IgAN 患儿共1349例,占同期住院泌尿系疾病患儿的1.37%,肾穿刺活检患儿的11.18%。1203例完整资料分析显示,男:女为2.07:1,发病年龄和确诊年龄中位数均为9.0岁,确诊前病程中位数为4个月。55.94%患儿起病有诱因。临床表现以复发性肉眼血尿型最常见(41.15%),其次为肾病综合征型(23.77%)和血尿蛋白尿型(20.78%)。病理分级以Ⅲ级(41.40%)和Ⅱ级(28.51%)最多见。免疫荧光检查:单独 IgA沉积占34.50%,IgA 沉积强度以血尿蛋白尿型和急进性肾炎型最强(+++)。治疗与随访:无统一治疗方案,部分患儿采用皮质激素和免疫抑制剂治疗。69.24%患儿临床好转,10.39%无变化,2例恶化。仅23.35%进行随访(平均24.4个月)。结论我国儿童原发性 IgAN 发病年龄在6岁以上。临床表现以血尿和肾病综合征型为多见,病理表现以Ⅱ～Ⅲ级为主。目前国内尚无统一治疗方案,随访率低,提示治疗亟待规范化,应加强随访。
Objective To investigate the clinical and pathological features of children with primary IgA nephropathy (IgAN) in our country, and to understand the clinical and pathological features of IgAN children and the treatment and prognosis. Methods The questionnaire was made by the Nephrology Group of Pediatrics Branch of Chinese Medical Association. The primary IgAN patients diagnosed by renal biopsy were retrospectively reviewed. The data were initially summarized from different places and then collected and analyzed statistically. Results From January 1995 to December 2004, there were 1349 cases of IgAN children ≤14 years old in 33 hospitals, accounting for 1.37% of children with urinary tract diseases in hospital and 11.18% of children with renal biopsy. The complete data analysis of 1203 cases showed that the median age of men and women was 2.07: 1, and the median age at diagnosis and the median age at diagnosis were both 9.0 years. The median pre-diagnosis duration was 4 months. 55.94% of children have incentives. The most common clinical manifestations of recurrent macroscopic hematuria (41.15%), followed by nephrotic syndrome (23.77%) and urine proteinuria (20.78%). Pathological grade Ⅲ grade (41.40%) and Ⅱ grade (28.51%) the most common. Immunofluorescence: IgA deposition alone accounted for 34.50%, IgA deposition intensity of urine proteinuria type and the most aggressive type of nephritis (+++). Treatment and follow-up: no uniform treatment program, some children treated with corticosteroids and immunosuppressive agents. 69.24% of children with clinical improvement, 10.39% no change, 2 cases of deterioration. Only 23.35% were followed up (average 24.4 months). Conclusion The incidence of primary IgAN in children is over 6 years old in our country. Clinical manifestations of hematuria and nephrotic syndrome is more common, the main pathological manifestations Ⅱ ~ Ⅲ grade. At present, there is no unified treatment plan in our country. The follow-up rate is low, suggesting that the treatment needs to be standardized and should be followed up.