33所医院儿童原发性IgA肾病临床和病理表现调查分析

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。目前国内尚元统一治疗方案,随访率低,提示治疗亟待规范化,应加强随访。

Clinical and pathological manifestations of Chinese childhood patients with primary IgA nephropathy: a national collaborative study of 33 hospitals

OBJECTIVE: Primary IgA nephropathy (IgAN) is characterized by a highly variable course ranging from a totally benign condition to rapidly progressive renal failure. About 30% of children with IgAN will eventually have end-stage renal failure after 20 years from onset. A nation-wide survey was conducted and data of hospitalized children (younger than 14 years old) with IgAN during the period of 1995 to 2004 were analyzed. The aim was to investigate the clinical and pathological characteristics, treatment and outcome of the hospitalized children with IgAN. METHODS: Questionnaires concerning children with IgAN were designed by the Subspecialty Group of Nephrology, Chinese Society of Pediatrics and distributed to the doctors of 33 hospitals in China. The criterion of IgAN was prominent and diffuse IgA deposition and to a lesser extent, other immunoglobulins in the glomerular mesangium and/or capillary loops, and purpura nephritis, lupus nephritis and hepatic disease were excluded. The data were collected and analyzed. RESULTS: From January 1, 1995 to December 31, 2004, 1349 hospitalized children were diagnosed as IgAN. The cases of childhood IgAN accounted for 1.37% of the hospitalized cases with urologic-kidney diseases and 11.18% of those who underwent renal biopsies. Complete records were available for 1203 patients. The male to female ratio was 2.07:1. Both the median ages at the disease onset and diagnosis were 9.0 years. The median duration from onset to diagnosis of IgAN was 4 months; 55.94% of patients had predisposing causes, especially infection. Recurrent macroscopic hematuria was the most common clinical manifestation (41.15%), followed by nephritic syndrome (23.77%) and hematuria and proteinuria (20.78%). Subclass III (41.40%) and II (28.51%) were the most common histologic types. The main type of immunofluorescence examination was IgA deposition (34.50%). The intensity of IgA deposition in patients with hematuria and proteinuria and in acute rapidly progressive nephritis was the strongest (+++). There was no unified treatment scheme. Some patients were treated with corticosteroids and immunosuppressants, and 69.24% of the patients with IgAN showed clinical improvement, 10.39% remained unchanged, and 2 cases presented deterioration. The rate of follow-up was 23.35%, the mean duration of follow-up was 24.4 months. CONCLUSION: The mean age of onset of the primary childhood IgAN was after 6 years. Hematuria was the most common clinical manifestation. Subclass III and II were the most common histologic type. There was no unified treatment scheme. The rate of follow-up was lower and the rate of lost follow-up was high. It is necessary to establish a normalized management, treatment and follow-up system for childhood IgAN in China.