Abstract
Background: 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. Methods: 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. Results: There was a significant increase in serum cystatin-C (p < 0.001) and a significant decrease in eGFR in patients with beta-TM compared with controls (p = 0.01). There was a significant increase in urinary NAG, KIM-1, UNAG/Cr, and UKIM-1/Cr (p < 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. Conclusion: 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.
Highlights
Beta thalassemia is considered the most common heterogeneous genetic disorder, resulting from reduced or absent beta globin synthesis, leading to globin chain imbalance [1].Beta thalassemia is characterized by reduced hemoglobin production with excess α-goblins.This leads to microcytic hypochromic anemia, which is associated with oxidative stress and ineffective erythropoiesis, leading to chronic hemolytic anemia [2,3]
Patients compared with the controls (p < 0.001), but they were within the normal range for both groups, and a significant decrease in Estimated glomerular filtration rate (eGFR) in patients with beta thalassemia major (beta-TM) compared with the controls (p = 0.01)
There was a significant increase in serum csytatin-C and urinary N-acetyl beta-D-glucosaminidase (NAG), kidney injury molecule 1 (KIM-1), UNAG/Cr, and UKIM-1/Cr among thalassemic patients compared with the controls (p < 0.001) (Table 3)
Summary
Beta thalassemia is characterized by reduced hemoglobin production with excess α-goblins. This leads to microcytic hypochromic anemia, which is associated with oxidative stress and ineffective erythropoiesis, leading to chronic hemolytic anemia [2,3]. Despite the increased survival of patients with thalassemia, some complications have been detected, such as renal injury or damage [5]. A good survival rate among patients with beta thalassemia major (beta-TM). 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. Results: There was a significant increase in serum cystatin-C (p < 0.001)
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