Department of Pediatrics, Niigata University Medical and Dental Hospital, Niigata, Japan.
Am J Nephrol. 2013;38(2):149-57. doi: 10.1159/000353898. Epub 2013 Aug 6.
Recent reports suggest that low birthweight (LBW) is a risk factor for kidney diseases, including focal segmental glomerulosclerosis (FSGS), although the underlying pathological mechanism remains unknown. Podocyte loss triggers glomerulosclerosis; however, whether FSGS in LBW children is associated with podocytopenia is unclear.
We reviewed the birthweights and gestational age of all patients who underwent renal biopsies from 1995 to 2011 at our Institute. Sixteen patients had FSGS, of which 6 (37.5%) had LBW; this LBW rate was significantly higher than the overall LBW rate in Japan (9.7%). The incidence of LBW was also high in patients with minimal change nephrotic syndrome (MCNS; 12.5%). The glomerular cell numbers in biopsy sections were calculated using computer image analysis and compared with FSGS of normal birthweight (NBW-FSGS). Biopsy specimens from age-matched patients with MCNS were also compared. Wilms' tumor-1 (WT1) immunohistochemistry was performed to enumerate the podocytes.
All patients in the LBW-FSGS group were also preterm, with an average gestational age of 25.8 weeks. The number of podocytes per glomerulus in the LBW-FSGS patients was 34 and 24% lower as compared to that in the MCNS patients (p < 0.01) and the NBW-FSGS patients (p < 0.05), respectively. Similar results were observed for the WT1-positive glomerular cell number.
LBW and premature birth were associated with FSGS development. The possibility that LBW and premature birth may be predisposing factors for severe podocytopenia in children with FSGS warrants further investigation.
最近的报告表明,低出生体重(LBW)是肾脏疾病的一个危险因素,包括局灶节段性肾小球硬化症(FSGS),尽管其潜在的病理机制尚不清楚。足细胞丢失引发肾小球硬化症;然而,LBW 儿童的 FSGS 是否与足细胞减少症有关尚不清楚。
我们回顾了 1995 年至 2011 年在我们研究所进行肾活检的所有患者的出生体重和胎龄。16 例患者患有 FSGS,其中 6 例(37.5%)为 LBW;LBW 发生率明显高于日本的总体 LBW 发生率(9.7%)。微小病变肾病综合征(MCNS;12.5%)患者的 LBW 发生率也很高。使用计算机图像分析计算活检切片中的肾小球细胞数量,并与正常出生体重(NBW-FSGS)的 FSGS 进行比较。还比较了年龄匹配的 MCNS 患者的活检标本。进行 Wilms' 肿瘤-1(WT1)免疫组化以计数足细胞。
LBW-FSGS 组的所有患者均为早产儿,平均胎龄为 25.8 周。LBW-FSGS 患者每个肾小球的足细胞数量比 MCNS 患者(p < 0.01)和 NBW-FSGS 患者(p < 0.05)分别低 34%和 24%。WT1 阳性肾小球细胞数量也观察到类似结果。
LBW 和早产与 FSGS 发展有关。LBW 和早产可能是 FSGS 儿童严重足细胞减少症的易患因素,值得进一步研究。