Lakkakula Bhaskar V K S, Verma Henu Kumar, Choubey Mona, Patra Suneeta, Khodiar Prafukka Kumar, Patra Pradeep Kumar
Research Division, Sickle Cell Institute Chhattisgarh, Raipur, Chhattisgarh, India.
Department of Biochemistry, Pt. Jawahar Lal Nehru Medical College, Raipur, Chhattisgarh, India.
Saudi J Kidney Dis Transpl. 2017 May-Jun;28(3):524-531. doi: 10.4103/1319-2442.206440.
Sickle cell disease (SCD) and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin. Renal disease is one of the most frequent complications, and kidney damage starts very early and progresses throughout life causing severe complications. The present study is aimed to analyze creatinine-based estimated glomerular filtration rate (eGFR) in 616 SCD patients (507 HbSS and 109 HbSB+), receiving medical care at outpatient wing of Sickle Cell Institute, Chhattisgarh. Glomerular filtration rate (GFR) estimated using the Modification of Diet in Renal Disease (MDRD), Cockcroft-Gault, chronic kidney disease epidemiology collaboration (CKD-EPI) (<17 years analyzed with Schwartz), and SCD specific Jamaica Sickle Cell Cohort Study (JSCCS)-GFR equations were compared. Further, eGFR calculated using the CKD-EPI and Schwartz equations was used to define various stages of kidney function and compared with clinical and hematological variables. The mean age of patients was 15.8 years. Comparison of eGFR using various formulas revealed that MDRD and JSCCS formulas overestimated the GFR. Among SCD patients, prevalence of glomerular hyperfiltration (GHF) is high followed by renal insufficiency (RI) and renal failure (RF). However, no differences were found in hematological profiling among different functional stages of kidney. Age and body surface area are significantly more in SCD individuals with normal kidney function and GHF. Participants with RF showed a higher level of blood urea and fetal hemoglobin. In summary, this is the first study to analyze different functional stages of kidney among SCD patients of India. Our study revealed that the GHF and RI are the important indicators of kidney damage.
镰状细胞病（SCD）及其变异型是由血红蛋白的突变形式导致的遗传性疾病。肾脏疾病是最常见的并发症之一，肾脏损害很早就开始，并在一生中持续发展，导致严重并发症。本研究旨在分析在恰蒂斯加尔邦镰状细胞研究所门诊接受治疗的616例SCD患者（507例HbSS和109例HbSB+）基于肌酐的估计肾小球滤过率（eGFR）。比较了使用肾脏疾病饮食改良（MDRD）、Cockcroft-Gault、慢性肾脏病流行病学协作组（CKD-EPI）（<17岁用Schwartz公式分析）以及SCD特异性牙买加镰状细胞队列研究（JSCCS）-GFR公式估算的肾小球滤过率（GFR）。此外，使用CKD-EPI和Schwartz公式计算的eGFR用于定义肾功能的各个阶段，并与临床和血液学变量进行比较。患者的平均年龄为15.8岁。使用各种公式比较eGFR发现，MDRD和JSCCS公式高估了GFR。在SCD患者中，肾小球高滤过（GHF）的患病率很高，其次是肾功能不全（RI）和肾衰竭（RF）。然而，在肾脏不同功能阶段的血液学分析中未发现差异。肾功能正常和GHF的SCD个体的年龄和体表面积明显更大。RF患者的血尿素和胎儿血红蛋白水平较高。总之，这是第一项分析印度SCD患者肾脏不同功能阶段的研究。我们的研究表明，GHF和RI是肾脏损害的重要指标。
