Department of Nephrology, Children's Hospital of Soochow University, Suzhou, China.
Department of Pediatrics, Taixing Hospital Affiliated to Yangzhou University, Taixing, Jiangsu, China.
Biomed Res Int. 2018 May 30;2018:4386438. doi: 10.1155/2018/4386438. eCollection 2018.
This study aimed to investigate the clinical significance of complement 3 (C3) deposition in renal tubules of children with primary nephrotic syndrome (PNS).
The clinical and pathological characteristics of PNS were retrospectively reviewed in 99 PNS pediatric patients, who were divided into the C3 deposition and the non-C3 deposition groups.
A total of 39 patients (39.39%) had renal tubule C3 deposition. In the C3 deposition group, the ratios of urine N-acetylglucosaminidase/creatinine (UNAG/Cr), urine 2 microglobulin/creatinine (U2MG/Cr), and urine transferrin/creatinine (UTRF/Cr) were significantly higher than those of the non-C3 deposition group. The patients of the C3 deposition group had lower serum total protein and albumin, higher cholesterol and D-dimer (DD), lower proportion of CD3+CD8+ cells, and higher proportion of CD19+CD23+ cells. The number of the patients with interstitial fibrosis, renal cell vacuolar degeneration, renal tubular immunoglobulin deposition, and severe tubulointerstitial injury in the C3 deposition group was higher than that of the non-C3 deposition group. The C3 deposition intensity was positively correlated with the number of recurrences.
PNS pediatric patients with C3 deposition in renal tubules have more severe disease condition, tubulointerstitial injury, and recurrence suggesting a worse long-term prognosis.
本研究旨在探讨补体 3(C3)在儿童原发性肾病综合征(PNS)肾小管沉积的临床意义。
回顾性分析 99 例 PNS 患儿的临床和病理特征,将其分为 C3 沉积组和非 C3 沉积组。
共 39 例(39.39%)患儿存在肾小管 C3 沉积。在 C3 沉积组,尿 N-乙酰氨基葡萄糖苷酶/肌酐(UNAG/Cr)、尿 2 微球蛋白/肌酐(U2MG/Cr)和尿转铁蛋白/肌酐(UTRF/Cr)比值明显高于非 C3 沉积组。C3 沉积组患者血清总蛋白和白蛋白较低,胆固醇和 D-二聚体(DD)较高,CD3+CD8+细胞比例较低,CD19+CD23+细胞比例较高。C3 沉积组患者间质纤维化、肾小管空泡变性、肾小管所沉积免疫球蛋白和严重肾小管间质损伤的例数多于非 C3 沉积组。C3 沉积强度与复发次数呈正相关。
伴有肾小管 C3 沉积的 PNS 患儿病情更严重,存在更严重的肾小管间质损伤和复发,提示预后较差。