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足细胞 CD44 是否为原发性局灶节段性肾小球硬化症肾功能恶化的病理标志物?

Is CD44 in glomerular parietal epithelial cells a pathological marker of renal function deterioration in primary focal segmental glomerulosclerosis?

机构信息

Department of Pediatrics, Pediatric Nephrourology Unit, Faculty of Medicine, Federal University of Minas Gerais, Avenida Alfredo Balena 190/sala 331, Belo Horizonte, Minas Gerais, 30130-100, Brazil.

Division of Renal Pathology, Clinics Hospital, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil.

出版信息

Pediatr Nephrol. 2017 Nov;32(11):2165-2169. doi: 10.1007/s00467-017-3775-4. Epub 2017 Aug 10.

Abstract

BACKGROUND

The search for risk factors for chronic kidney disease in children with focal segmental glomerulosclerosis (FSGS) is important in defining prognosis and individualized treatment. This study preliminarily investigated whether CD44 immunostaining in glomerular parietal epithelial cells (PECs) is a prognostic marker in pediatric FSGS.

METHODS

In this retrospective study, 26 patients with FSGS, biopsied from 1985 to 2010, were evaluated. Immunohistochemistry for CD44 was performed in all cases. For analysis purposes, patients were grouped according to whether or not they were positive for CD44 in PECs. The primary outcome was a decline in baseline estimated glomerular filtration rate (eGFR) of 50% or more.

RESULTS

Median follow-up was 6.9 years. Median renal survival was 14.5 years and probability of a 50% decline of eGRF was 30% in 10 years. Nine children exhibited the primary outcome and 7 developed end-stage renal disease (ESRD). In comparison with PEC CD44-negative patients (n = 18), PEC CD44-positive patients (n = 8) presented lower baseline eGFR (99 ± 41 versus 141 ± 44 ml/min/1.73 m, p = 0.035) and a significant decline in eGFR (-38.6 ± 39.5 versus -5.6 ± 25.3 ml/min/1.73 m/year, p = 0.018). No difference was observed in FSGS subtypes or other glomerular features. Presence of CD44 staining in PECs was significantly associated with the decline in baseline eGFR of 50% or more. Renal survival was significantly reduced in PEC CD44-positive patients (3.8 vs 14.6 years in C4d-negative, p < 0.05).

CONCLUSION

Our preliminary findings indicate, for the first time, that positivity for CD44 in PECs seems to be a pathological marker of renal function deterioration in pediatric patients with FSGS.

摘要

背景

在局灶节段性肾小球硬化症(FSGS)患儿中寻找慢性肾脏病的风险因素对于确定预后和个体化治疗非常重要。本研究初步探讨了肾小球壁细胞(PEC)中 CD44 免疫染色是否为儿科 FSGS 的预后标志物。

方法

在这项回顾性研究中,评估了 1985 年至 2010 年间活检的 26 例 FSGS 患者。所有病例均行 CD44 免疫组化检查。为了分析目的,根据 PEC 中是否存在 CD44 阳性将患者分为两组。主要结局是基线估算肾小球滤过率(eGFR)下降 50%或更多。

结果

中位随访时间为 6.9 年。中位肾脏存活率为 14.5 年,10 年内 eGRF 下降 50%的概率为 30%。9 名儿童出现主要结局,7 名儿童发展为终末期肾病(ESRD)。与 PEC CD44 阴性患者(n=18)相比,PEC CD44 阳性患者(n=8)的基线 eGFR 更低(99±41 与 141±44 ml/min/1.73 m,p=0.035),eGFR 显著下降(-38.6±39.5 与-5.6±25.3 ml/min/1.73 m/年,p=0.018)。FSGS 亚型或其他肾小球特征无差异。PEC 中 CD44 染色的存在与基线 eGFR 下降 50%或更多显著相关。PEC CD44 阳性患者的肾脏存活率明显降低(3.8 与 C4d 阴性患者的 14.6 年,p<0.05)。

结论

我们的初步研究结果首次表明,PEC 中 CD44 的阳性似乎是儿科 FSGS 患者肾功能恶化的病理标志物。

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