Service de néphrologie pédiatrique, APHP, Hôpital Universitaire Robert-Debré, 48 Boulevard Serrurier, 75019, Paris, France.
Service de Pathologie, Hôpital Universitaire Hôpital Necker, APHP, Paris, France.
Pediatr Nephrol. 2020 Oct;35(10):1897-1905. doi: 10.1007/s00467-020-04614-3. Epub 2020 May 22.
Previous reports suggest initial presentation of IgA nephropathy (IgAN) in children is different from adults. No systematic comparison of clinical, biological, and histological childhood- and adult-onset IgAN is currently available.
We compared pediatric and adult clinical and histological characteristics at IgAN diagnosis. Data on 211 consecutive patients from two different centers in Paris (82 children, 129 adults) were reviewed. Kidney biopsies were scored for Oxford classification and podocytopathic (P1) features.
We report higher eGFR at diagnosis in children compared to adults (89.5 vs. 64 ml/min/1.73 m; p = 0.0001) but no difference in proteinuria. Histological analysis of kidney biopsy found higher proportions of mesangial (M1) and endocapillary (E1) hypercellularity in children compared with adults (M1 [80.7% vs. 27.9%, p = 0.0001]; E1 [71.3% vs. 30%, p = 0.0001]). Focal glomerulosclerosis (S1), tubular atrophy/interstitial fibrosis ≥ 25% (T1), and P1 were more frequent in adults (S1 [81.5% vs. 61.3%, p = 0.0012], T1 [49.5% vs. 1.35%, p = 0.0001], P1 [33.8% vs. 16.4%, p = 0.008). Proteinuria associated with M1, E1, and C1 in children (M1, p = 0.0001; E1, p = 0.0005; C1, p = 0.0014) but S1, P1, and T1 in adults (S1, p = 0.0001; P1, p = 0.0001; T1, p = 0.001). After steroid treatment (41 children and 28 adults), proteinuria decreased in children (p < 0.001, follow-up 38 months) and adults (p < 0.001, follow-up 76.9 months), whereas eGFR remained stable in adults but increased significantly in children (90.6 to 110 ml/min/1.73m).
Proteinuria in children with IgAN is a marker of glomerular proliferative lesions whereas its presence in adults often reflects the presence of chronic lesions. This suggests the need for histological assessment.
先前的报告表明，儿童 IgA 肾病（IgAN）的初始表现与成人不同。目前尚无儿童和成人发病的 IgAN 的临床、生物学和组织学的系统比较。
我们比较了两个不同巴黎中心的 211 例连续患者的儿科和成人临床及组织学特征。回顾了 82 例儿童和 129 例成人的数据。对牛津分类和足细胞病变（P1）特征进行了肾脏活检评分。
与成人相比，我们报告儿童在诊断时的 eGFR 更高（89.5 与 64 ml/min/1.73 m；p = 0.0001），但蛋白尿无差异。肾脏活检组织学分析发现，与成人相比，儿童的系膜（M1）和内皮下（E1）细胞增多更为常见（M1 [80.7%与 27.9%，p = 0.0001]；E1 [71.3%与 30%，p = 0.0001]）。局灶性肾小球硬化（S1）、肾小管萎缩/间质纤维化≥25%（T1）和 P1 在成人中更为常见（S1 [81.5%与 61.3%，p = 0.0012]；T1 [49.5%与 1.35%，p = 0.0001]；P1 [33.8%与 16.4%，p = 0.008]）。儿童的 M1、E1 和 C1 与蛋白尿相关（M1，p = 0.0001；E1，p = 0.0005；C1，p = 0.0014），而 S1、P1 和 T1 在成人中相关（S1，p = 0.0001；P1，p = 0.0001；T1，p = 0.001）。在接受类固醇治疗（41 名儿童和 28 名成人）后，儿童的蛋白尿减少（p < 0.001，随访 38 个月）和成人（p < 0.001，随访 76.9 个月），而 eGFR 在成人中保持稳定，但在儿童中显著增加（从 90.6 增加到 110 ml/min/1.73 m）。
儿童 IgAN 的蛋白尿是肾小球增殖性病变的标志物，而其在成人中的存在往往反映了慢性病变的存在。这表明需要进行组织学评估。
