OBJECTIVES Crescentic IgA nephropathy (C-IgAN) is defined as IgAN with more than 50% of glomeruli showing crescents. C-IgAN in children is rare; we investigate in detail for the first time. METHODS We retrospectively analyzed the 515 consecutive children who were newly diagnosed with biopsy-proven IgAN between June 1976 and May 2010. We compared clinical and pathological findings between C-IgAN and non-C-IgAN. RESULTS Among 515 cases of childhood IgAN, 25 children (4.9%) had C-IgAN. Of these 25, 16 children (64%) were referred to hospitals by annual school screening. Clinical findings showed significant differences in gross hematuria (76 vs. 50%, p = .03), excretion of proteinuria (1.9 vs. 0.5 g/day/m2, p < .0001), eGFR (102 vs. 108 ml/min/1.73 m2, p = .03), and duration from onset to renal biopsy (4.0 vs. 8.0 months, p = .04) between groups. Pathological findings showed significant differences in M1 (88 vs. 55%, p = .02), E1 (83 vs. 53%, p = .008), and presence of tubular atrophy/interstitial fibrosis (88 vs. 53%, p < .0001) between groups. The 16 children with C-IgAN were treated with prednisolone and immunosuppressant. Four cases (16%) reached chronic renal failure (eGFR < 60) at the latest observation (mean observation period: 6.0 ± 3.6 years). Patients with C-IgAN had significantly lower renal survival curve than non-C-IgAN patients according to Kaplan-Meier analysis (77.1% vs. 92.6% at 13 years, p < .0001). Compared with previous reports, however, they had better renal outcome. CONCLUSIONS We confirmed the importance of school screening to find C-IgAN. Although most crescents (mean: 98.1%) of C-IgAN were cellular/fibrocellular, and acute lesions were well modified with combination therapy, the presence of tubular atrophy in C-IgAN may be the reason for poorer prognosis.

中文翻译：

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小儿新月型IgA肾病。

目标新月型IgA肾病（C-IgAN）定义为IgAN，其中肾小球的50％以上显示新月。儿童中的C-IgAN很少；我们将进行首次详细调查。方法我们回顾性分析了1976年6月至2010年5月间新诊断为经活检证实为IgAN的515例连续儿童。我们比较了C-IgAN和非C-IgAN的临床和病理学发现。结果在515例儿童IgAN中，有25例儿童（4.9％）患有C-IgAN。在这25名儿童中，有16名儿童（64％）通过年度学校筛查被转介到医院。临床发现显示，血尿明显（76％vs. 50％，p = .03），蛋白尿排泄（1.9 vs. 0.5 g / day / m2，p <.0001），eGFR（102 vs. 108 ml / min）有显着差异。 /1.73平方米，p = .03），两组之间从发病至肾活检的持续时间（4.0 vs. 8.0个月，p = .04）。病理结果显示M1（88 vs. 55％，p = .02），E1（83 vs. 53％，p = .008）和肾小管萎缩/间质纤维化存在显着差异（88 vs. 53％，p <.0001）。用强的松龙和免疫抑制剂治疗16例C-IgAN儿童。最近的一次观察（平均观察期：6.0±3.6年）有4例（16％）达到了慢性肾衰竭（eGFR <60）。根据Kaplan-Meier分析，C-IgAN患者的肾脏生存曲线明显低于非C-IgAN患者（13岁时分别为77.1％和92.6％，p <.0001）。但是，与以前的报道相比，他们的肾脏预后更好。结论我们证实了进行C-IgAN的学校筛查的重要性。尽管C-IgAN的大多数新月形（平均占98.1％）是细胞/纤维细胞，