基于单中心5年IgA肾病和紫癜性肾炎的临床和病理分型—对IgA肾病和紫癜性肾炎临床和病理分型的商榷

目的 总结儿童IgA肾病(IgAN)和紫癜性肾炎(HSPN)的临床资料,并探讨国内儿童IgAN和HSPN的临床和病理分型。方法 纳入2014年1月1日至2018年12月31日北京大学第一医院儿科初治的诊断明确的IgAN和HSPN患儿,临床和病理分型参照中华医学会儿科学分会肾脏病学组标准。截取患儿入院时一般情况、临床特点、最终诊断临床和病理分型。结果 共纳入IgAN患儿255例,HSPN患儿212例。IgAN肾病综合征型45.5%、血尿和蛋白尿型41.2%、急性肾炎型11.0%、急进性肾炎型2.4%,无孤立性血尿型、孤立性蛋白尿型和慢性肾炎型;HSPN血尿和蛋白型42.0%、肾病综合征型39.2%、急性肾炎型12.3%、孤立性血尿型5.7%、急进性肾炎型0.9%,无孤立性蛋白尿型和慢性肾炎型。除了孤立性血尿型(因IgA肾活检标准未包括外),其余临床分型IgAN和HSPN差异无统计学意义(P>0.05)。IgAN和HSPN不同临床分型病理分型均以Ⅲ型(分别为52.4%~53.6%和51.8%~53.9%)和Ⅱ型(分别为35.3%~36.2%和34.6%~36.3%)最为常见,不同临床分型间病理类型差异无统计学意义(P>0.05)。IgAN和HSPN不同临床分型总有效率分别为99.1%~100%和97.6%~100%,不同临床分型间预后差异无统计学意义(P>0.05)。结论 目前国内有关儿童IgAN和HSPN的临床和病理分型依据和标准有待商榷,值得进一步探索。

Discussion on the clinical and pathological classification of IgA nephropathy and Henoch-Schonlein purpura nephritis in children

Objective To analyze the clinical characteristics of IgA nephropathy and Henoch-Schonlein purpura nephritis in children, and to explore the value of its clinical and pathological classification.Methods Patients with the first onset of the IgA nephropathy or Henoch-Schonlein purpura nephritis , who hospitalized in our hospital from January 2014 to December 2018 were studied retrospectively to summarize their clinical data. The clinical and pathological classification of IgA nephropathy and Henoch-Schonlein was according to the criterion of Kidney Pathology Group of pediatric Branch of Chinese Medical Association.Results There were 255 cases of first IgA nephropathy and 212 cases of Henoch-Schonlein purpura nephritis included in this study. Nephritic syndrome type (45.5% vs 39.2%), hematuria and proteinuria type (41.2% vs 42.5%) were most common, while isolated proteinuria type and chronic nephritis were not seen in both IgA nephropathy and Henoch-Schonlein purpura nephritis group. Grades Ⅲ (52.4%~53.6% vs 51.8%~53.9%) and Ⅱ (35.3%~36.2% vs 34.6%~36.3%) were the most common pathological types in different clinical types (P>0.05). The prognosis between different clinical types was the same (99.1%~100% vs 97.6%~100%) (P>0.05).Conclusion The clinical and pathological classification of childhood IgA nephropathy and Henoch-Schonlein purpura nephritis in China is useless and it should be discussed further.