Shen Huijun, Gu Weizhong, Mao Jianhua, Zhu Xiujuan, Jin Xia, Fu Haidong, Liu Aimin, Shu Qiang, Du Lizhong
Department of Nephrology, The Children's Hospital of Zhejiang University School of Medicine, Hangzhou, China.
Nephron. 2015;130(1):21-8. doi: 10.1159/000382035. Epub 2015 Apr 22.
IgA nephropathy (IgAN) is the most common primary glomerulonephritis in children and adolescents worldwide. Meanwhile, minimal change disease (MCD) is the most common cause of nephrotic syndrome in children. Recently, several case reports have revealed that IgAN can be complicated by MCD in adults. Here, we report our experience concerning the features of such patients in pediatrics.
The clinical manifestations and pathological features of 197 children who presented with IgAN from December 2007 to November 2013 were analyzed retrospectively based on the criteria for nephrotic syndrome and MCD.
Among the 197 children diagnosed with primary IgAN, 25 (12.7%) patients presented with nephrotic syndrome, and 7 patients (2.8%) presented with MCD-like pathological features and nephrotic syndrome simultaneously. The cohort of 7 patients included 5 boys, and the median age was 8.9. All of the patients who were diagnosed with primary nephrotic syndrome were treated initially with corticosteroids. Except for 1 patient with steroid resistance who was lost to follow-up, the other 6 cases presented were steroid sensitive and remained in complete remission for the last follow-up, with median and mean follow-up durations of 30.5 and 34.5 months, respectively (range 10-65 months).
This study revealed that IgAN and MCD may also coexist in children. Moreover, most of these patients who presented with nephrotic syndrome responded well to steroids and had a favorable prognosis. Large-scale studies are required, and careful attention should be paid to such complicated cases.
IgA肾病(IgAN)是全球儿童和青少年中最常见的原发性肾小球肾炎。同时,微小病变病(MCD)是儿童肾病综合征最常见的病因。最近,几例病例报告显示成人IgAN可合并MCD。在此,我们报告我们在儿科对此类患者特征的经验。
回顾性分析2007年12月至2013年11月期间出现IgAN的197例儿童的临床表现和病理特征,依据肾病综合征和MCD的标准进行分析。
在197例诊断为原发性IgAN的儿童中,25例(12.7%)患者出现肾病综合征,7例(2.8%)患者同时出现MCD样病理特征和肾病综合征。7例患者队列包括5名男孩,中位年龄为8.9岁。所有诊断为原发性肾病综合征的患者最初均接受皮质类固醇治疗。除1例激素抵抗患者失访外,其他6例患者对激素敏感,最后一次随访时仍完全缓解,中位随访时间和平均随访时间分别为30.5个月和34.5个月(范围10 - 65个月)。
本研究表明IgAN和MCD在儿童中也可能共存。此外,这些出现肾病综合征的患者大多数对类固醇反应良好,预后良好。需要进行大规模研究,并且应密切关注此类复杂病例。