Mahmoud Asmaa A, Elian Doaa M, Abd El Hady Nahla Ms, Abdallah Heba M, Abdelsattar Shimaa, Khalil Fatma O, Abd El Naby Sameh A
Department of Pediatrics, Faculty of Medicine, Menoufia University, Shebin Elkom 32511, Egypt.
Department of Pediatrics, College of Medicine, King Faisal University, Al-Ahsa 31982, Saudi Arabia.
Children (Basel). 2021 Feb 3;8(2):100. doi: 10.3390/children8020100.
A good survival rate among patients with beta thalassemia major (beta-TM) has led to the appearance of an unrecognized renal disease. Therefore, we aimed to assess the role of serum cystatin-C as a promising marker for the detection of renal glomerular dysfunction and N-acetyl beta-D-glucosaminidase (NAG) and kidney injury molecule 1 (KIM-1) as potential markers for the detection of renal tubular injury in beta-TM children.
This case-control study was implemented on 100 beta-TM children receiving regular blood transfusions and undergoing iron chelation therapy and 100 healthy children as a control group. Detailed histories of complete physical and clinical examinations were recorded. All subjected children underwent blood and urinary investigations.
There was a significant increase in serum cystatin-C ( < 0.001) and a significant decrease in eGFR in patients with beta-TM compared with controls ( = 0.01). There was a significant increase in urinary NAG, KIM-1, UNAG/Cr, and UKIM-1/Cr ( < 0.001) among thalassemic children, with a significant positive correlation between serum cystatin-C, NAG and KIM-1 as regards serum ferritin, creatinine, and urea among thalassemic patients. A negative correlation between serum cystatin-C and urinary markers with eGFR was noted.
Serum cystatin-C is a good marker for detection of glomerular dysfunction. NAG and KIM-1 may have a predictive role in the detection of kidney injury in beta-TM children.
重型β地中海贫血（β-TM）患者的良好生存率导致了一种未被认识的肾脏疾病的出现。因此，我们旨在评估血清胱抑素-C作为检测肾小球功能障碍的有前景标志物的作用，以及N-乙酰-β-D-氨基葡萄糖苷酶（NAG）和肾损伤分子1（KIM-1）作为检测β-TM儿童肾小管损伤的潜在标志物的作用。
本病例对照研究对100名接受定期输血和铁螯合治疗的β-TM儿童以及100名健康儿童作为对照组进行。记录详细的全面体格检查和临床检查病史。所有受试儿童均接受血液和尿液检查。
与对照组相比，β-TM患者的血清胱抑素-C显著升高（<0.001），估算肾小球滤过率（eGFR）显著降低（=0.01）。地中海贫血儿童的尿NAG、KIM-1、尿NAG/肌酐（UNAG/Cr）和尿KIM-1/肌酐（UKIM-1/Cr）显著升高（<0.001），地中海贫血患者的血清胱抑素-C、NAG和KIM-1与血清铁蛋白、肌酐和尿素之间存在显著正相关。血清胱抑素-C和尿标志物与eGFR之间呈负相关。
血清胱抑素-C是检测肾小球功能障碍的良好标志物。NAG和KIM-1可能在检测β-TM儿童肾损伤方面具有预测作用。
