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常染色体显性多囊肾病患儿的蛋白尿

Proteinuria in children with autosomal dominant polycystic kidney disease.

作者信息

Seeman Tomáš, Pohl Michael, John Ulrike

机构信息

Section of Pediatric Nephrology, Department of Pediatrics -

nd Faculty of Medicine, Charles University Prague, Motol University Hospital, Prague, Czech Republic.

出版信息

Minerva Pediatr. 2018 Oct;70(5):413-417. doi: 10.23736/S0026-4946.16.04404-2.

Abstract

BACKGROUND

Proteinuria is a common complication in adults with autosomal dominant polycystic kidney disease (ADPKD) and serves as a risk factor for progression. However, proteinuria has rarely been examined in children with ADPKD and the type of proteinuria has not yet been investigated. The aim of the study was to assess the prevalence and to analyse the types of proteinuria in children with ADPKD.

METHODS

Children with ADPKD followed-up in our tertiary centres during the years 2012-2013 were investigated in a cross-sectional study. Morning urine was tested for total protein (PROT), albumin (ALB) and alpha-1-microglobulin (AMG). Renal function was assessed from serum creatinine as estimated glomerular filtration rate.

RESULTS

Thirty-seven children of median age 11.2 (2.0-18.0) years were investigated. Median (range) PROT, ALB and AMG (in mg/mmol creatinine) were 15.1 (6.2-64.8), 2.54 (0.54-37.25) and 3.22 (0.04-10.16), respectively. Pathological total proteinuria (>22) was found in 30% of children, albuminuria (>2.2) in 49% of children and alpha-1-microglobulinuria (>0.55) in 65% of children. No correlation was found between PROT, ALB or AMG and office blood pressure, kidney size or estimated glomerular filtration rate.

CONCLUSIONS

Proteinuria in children with ADPKD is a frequent finding, the most common type is tubular proteinuria. It should be measured in all ADPKD children.

摘要

背景

蛋白尿是常染色体显性遗传性多囊肾病(ADPKD)成人患者的常见并发症,也是疾病进展的危险因素。然而,ADPKD儿童患者的蛋白尿情况鲜有研究,蛋白尿类型也尚未明确。本研究旨在评估ADPKD儿童患者蛋白尿的患病率并分析其类型。

方法

在一项横断面研究中,对2012年至2013年期间在我们三级中心接受随访的ADPKD儿童患者进行调查。检测晨尿中的总蛋白(PROT)、白蛋白(ALB)和α-1-微球蛋白(AMG)。根据血清肌酐评估肾功能,计算估计肾小球滤过率。

结果

共调查了37名儿童,中位年龄为11.2(2.0 - 18.0)岁。PROT、ALB和AMG的中位数(范围)(单位:mg/mmol肌酐)分别为15.1(6.2 - 64.8)、2.54(0.54 - 37.25)和3.22(0.04 - 10.16)。30%的儿童存在病理性总蛋白尿(>22),49%的儿童存在白蛋白尿(>2.2),65%的儿童存在α-1-微球蛋白尿(>0.55)。未发现PROT、ALB或AMG与诊室血压、肾脏大小或估计肾小球滤过率之间存在相关性。

结论

ADPKD儿童患者中蛋白尿很常见,最常见的类型是肾小管性蛋白尿。所有ADPKD儿童患者均应检测蛋白尿。

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