Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major

Asmaa A Mahmoud; Doaa M Elian; Nahla Ms Abd El Hady; Heba M Abdallah; Shimaa Abdelsattar; Fatma O Khalil; Sameh A Abd El Naby

doi:10.3390/children8020100

Assessment of Subclinical Renal Glomerular and Tubular Dysfunction in Children with Beta Thalassemia Major

Children (Basel). 2021 Feb 3;8(2):100. doi: 10.3390/children8020100.

Authors

Asmaa A Mahmoud¹, Doaa M Elian^{1

2}, Nahla Ms Abd El Hady¹, Heba M Abdallah³, Shimaa Abdelsattar⁴, Fatma O Khalil⁵, Sameh A Abd El Naby¹

Affiliations

¹ Department of Pediatrics, Faculty of Medicine, Menoufia University, Shebin Elkom 32511, Egypt.
² Department of Pediatrics, College of Medicine, King Faisal University, Al-Ahsa 31982, Saudi Arabia.
³ Department of Clinical Pathology, National Liver Institute, Menoufia University, Shebin Elkom 32511, Egypt.
⁴ Department of Clinical Biochemistry and Molecular Diagnostics, National Liver Institute, Menoufia University, Shebin Elkom 32511, Egypt.
⁵ Department of Clinical and Molecular Microbiology and Immunology, National Liver Institute, Menoufia University, Shebin Elkom 32511, Egypt.

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.

Keywords: N-acetyl beta-D-glucosaminidase (NAG); beta thalassemia major; cystatin C; iron overload; kidney injury molecule 1 (KIM-1).