Abstract：
Sickle cell disease causes microvascular occlusion in different vascular beds. In kidneys, it leads to occult glomerular dysfunction causing asymptomatic microalbuminuria, proximal tubulopathy causing hyposthenuria and increased free water loss and distal tubulopathy causing poor urine acidification. We studied the prevalence of various types of renal dysfunction, the ability of different tests to detect it at an early stage and the correlation of these parameters in children receiving hydroxyurea (HU).
Fifty-six children (sample size calculated using SAS9.2 package) attending paediatric clinical services in a tertiary care hospital between 2 and 12 years of age diagnosed by high-performance liquid chromatography (HPLC) were enrolled. Their demographic and laboratory data including renal and urine parameters were collected. Parameters like fractional excretion of sodium (FeNa), trans tubular potassium gradient (TtKg) and free water clearance (TcH2O) were derived by calculations. Data were analysed using IBM SPSS Version 21.0 and Microsoft Office Excel 2007.
We found a significant number of children to have microalbuminuria (17.8%), hyposthenuria (30.4%) and impaired renal tubular potassium excretion (TtKg) (81.3%). A significant correlation was found between the dose of HU with urine osmolality (p < 0.0005) and free water clearance (p = 0.002), while all parameters showed a significant correlation with compliance with HU. Derangement in urine microalbumin and TcH2O correlated significantly with low mean haemoglobin levels (<9 g/dl).
Renal dysfunction is common in children with SCD and can be detected early using simple urine parameters and can be prevented with an early and appropriate dosage of HU with good compliance.
Fifty-six children (sample size calculated using SAS9.2 package) attending paediatric clinical services in a tertiary care hospital between 2 and 12 years of age diagnosed by high-performance liquid chromatography (HPLC) were enrolled. Their demographic and laboratory data including renal and urine parameters were collected. Parameters like fractional excretion of sodium (FeNa), trans tubular potassium gradient (TtKg) and free water clearance (TcH2O) were derived by calculations. Data were analysed using IBM SPSS Version 21.0 and Microsoft Office Excel 2007.
We found a significant number of children to have microalbuminuria (17.8%), hyposthenuria (30.4%) and impaired renal tubular potassium excretion (TtKg) (81.3%). A significant correlation was found between the dose of HU with urine osmolality (p < 0.0005) and free water clearance (p = 0.002), while all parameters showed a significant correlation with compliance with HU. Derangement in urine microalbumin and TcH2O correlated significantly with low mean haemoglobin levels (<9 g/dl).
Renal dysfunction is common in children with SCD and can be detected early using simple urine parameters and can be prevented with an early and appropriate dosage of HU with good compliance.
摘要：
背景：镰状细胞病会导致不同血管床中的微血管闭塞。在肾脏，它导致隐匿性肾小球功能障碍，导致无症状的微量白蛋白尿，近端肾小管病会导致尿失禁和游离水流失增加，远端肾小管病会导致尿液酸化不良。我们研究了各种类型的肾功能不全的患病率，在接受羟基脲（HU）的儿童中，不同测试在早期阶段检测到它的能力以及这些参数的相关性。
方法：纳入了56名儿童（使用SAS9.2软件包计算的样本量），这些儿童在三级保健医院接受儿科临床服务，这些儿童在2至12岁之间通过高效液相色谱（HPLC）进行诊断。收集他们的人口统计学和实验室数据，包括肾脏和尿液参数。钠（FeNa）的排泄分数等参数，通过计算得出反式管状钾梯度（TtKg）和游离水清除率（TcH2O）。使用IBMSPSS版本21.0和MicrosoftOfficeExcel2007对数据进行了分析。
结果：我们发现大量儿童有微量白蛋白尿（17.8%），神经性尿症（30.4％）和肾小管钾排泄受损（TtKg）（81.3％）。发现HU的剂量与尿渗透压（p<0.0005）和游离水清除率（p=0.002）之间存在显着相关性，而所有参数均与HU的依从性显着相关。尿微量白蛋白和TcH2O的紊乱与低平均血红蛋白水平（<9g/dl）显着相关。
结论：肾功能障碍在SCD儿童中很常见，可以通过简单的尿液参数早期发现，可以通过早期和适当剂量的HU进行预防，具有良好的依从性。
方法：纳入了56名儿童（使用SAS9.2软件包计算的样本量），这些儿童在三级保健医院接受儿科临床服务，这些儿童在2至12岁之间通过高效液相色谱（HPLC）进行诊断。收集他们的人口统计学和实验室数据，包括肾脏和尿液参数。钠（FeNa）的排泄分数等参数，通过计算得出反式管状钾梯度（TtKg）和游离水清除率（TcH2O）。使用IBMSPSS版本21.0和MicrosoftOfficeExcel2007对数据进行了分析。
结果：我们发现大量儿童有微量白蛋白尿（17.8%），神经性尿症（30.4％）和肾小管钾排泄受损（TtKg）（81.3％）。发现HU的剂量与尿渗透压（p<0.0005）和游离水清除率（p=0.002）之间存在显着相关性，而所有参数均与HU的依从性显着相关。尿微量白蛋白和TcH2O的紊乱与低平均血红蛋白水平（<9g/dl）显着相关。
结论：肾功能障碍在SCD儿童中很常见，可以通过简单的尿液参数早期发现，可以通过早期和适当剂量的HU进行预防，具有良好的依从性。
