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镰状细胞病患儿的肾功能:资源极度匮乏环境下的早期检测挑战

Renal function in children suffering from sickle cell disease: challenge of early detection in highly resource-scarce settings.

作者信息

Aloni Michel Ntetani, Ngiyulu René Makwala, Gini-Ehungu Jean-Lambert, Nsibu Célestin Ndosimao, Ekila Mathilde Bothale, Lepira François Bompeka, Nseka Nazaire Mangani

机构信息

Division of Hemato-oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of Congo.

Intensive Care Division, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Kinshasa, Democratic Republic of Congo.

出版信息

PLoS One. 2014 May 8;9(5):e96561. doi: 10.1371/journal.pone.0096561. eCollection 2014.

Abstract

BACKGROUND

The prevalence of Sickle cell disease is extremely high in Democratic Republic of Congo. Despite this high prevalence of the disease, data on renal abnormalities in children are rare.

METHOD

The study proposed to assess blood pressure, glomerular function, urea and uric acid levels in 65 steady state Congolese children with homozygous sickle cell disease and 67 normal controls.

RESULTS

In Hb-SS group, blood pressure level tended to be lower than Hb-AA groups but there was no statistically significant difference (p>0.05) between the two groups. The absolute values for GFR corrected for BSA were significantly higher in Hb-SS group compared to Hb-AA group (130.5±34.1 ml/min/1.73 m2 vs 113.7±24.5 ml/min/1.73 m2; p = 0.004). Children with Hb-SS were more likely to hyperfiltrate (30.8% of subjects) than children with Hb-AA (6.1% of subjects). Proteinuria was found in 4 (6.2%) children with Hb-SS. Uric acid level was significantly increased in children with Hb-SS compared to corresponding values in control group (4.4±1.3 mg/dl vs 3.5±1.1 mg/dl; p<0.001). Urea level was significantly decreased compared to corresponding values in Hb-AA group (15.3±8.3 mg/dl vs 22.9±10.1 mg/dl; p<0.001).

CONCLUSION

Hyperfiltration, low creatinine, lower urea and high uric acid are more common in children with sickle cell disease than in normal controls.

摘要

背景

镰状细胞病在刚果民主共和国的患病率极高。尽管该病患病率很高,但关于儿童肾脏异常的数据却很少。

方法

该研究旨在评估65名患有纯合子镰状细胞病的刚果稳态儿童和67名正常对照儿童的血压、肾小球功能、尿素和尿酸水平。

结果

在血红蛋白SS组中,血压水平往往低于血红蛋白AA组,但两组之间无统计学显著差异(p>0.05)。与血红蛋白AA组相比,血红蛋白SS组经体表面积校正后的肾小球滤过率绝对值显著更高(130.5±34.1毫升/分钟/1.73平方米对113.7±24.5毫升/分钟/1.73平方米;p = 0.004)。血红蛋白SS组的儿童比血红蛋白AA组的儿童更易出现高滤过(30.8%的受试者)。在4名(6.2%)血红蛋白SS组儿童中发现蛋白尿。与对照组相应值相比,血红蛋白SS组儿童的尿酸水平显著升高(4.4±1.3毫克/分升对3.5±1.1毫克/分升;p<0.001)。与血红蛋白AA组相应值相比,尿素水平显著降低(15.3±8.3毫克/分升对22.9±10.1毫克/分升;p<0.001)。

结论

与正常对照相比,镰状细胞病患儿更常见高滤过、低肌酐、低尿素和高尿酸。

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