الفهرس 
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Abstract
Summary
β-Thalassemia syndromes are the most common inherited hemoglobinopathies in the world caused by an autosomal recessive genetic deficiency in the β-globin chain synthesis leading to accumulation of unpaired α-globin chains.
Renal dysfunction is a frequent complication in patients suffering from β-thalassemia major (β-TM). Low-molecular-weight proteinuria is found in almost all patients.
The discovery of the first microRNA (miRNA) over 20 years ago has ushered in a new era in molecular biology. There are now over 2000 miRNAs that have been discovered in humans and it is believed that they collectively regulate one third of the genes in the genome.
To assess serum levels of microRNA in children with beta-thalassemia major (β-TM) and investigate its significance as early marker of glomerular and tubular dysfunctions and hemolysis. 90 TM children (30 with chronic kidney disease,30 without chronic kidney disease group and 30 children as a control group). Candidates were recruited from the hematology clinic in Beni-Suef University Hospital.
Results:Analytical interpretation of the current study results showed a significant elevation of serum ferritin (1547.23±791.53) and serum creatinine(0.41±0.15) in beta thalassemia major patients (p<0.030 and <0.004) respectively while eGFR was significant lower in cases than controls (144.76± 48.44 , 166.53± 41.39 )(p<0.038).Albumin/ creatinine ratio was significantly higher in cases than controls (58.64± 131.58, 8.60± 3.34)(p<0.041).
Creatinine and ARC were significant higher in TM patients with CKD than patients without CKD (p=0.006& p=0.002) respectively.
The miRNA-451 expression levels were significantly higher in TM patients compared to the controls(1.14±0.55, 0.81± 0.56) (p-value, 0.011).
Splenectomized patients exhibited considerably greater plasma miR-451 levels than non-splenectomized individuals (p-value, 0.005).
The frequency of splenectomy was higher in patients with over-expressed miRNA-451 than patients with under-expression (12 (31.6%), 2 (9.1%)) ( p<0.047).
Platelet, MicroRNA-451-fold change and eGFR were significant lower in TM patients with CKD than patients without CKD (p=0.004, p=0.016& p=0.000) respectively. The frequency of CKD and ACR levels were significantly higher among patients with under-expressed miRNA-451 while eGFR was significantly lower in under-expressed miRNA-451 patients. Furthermore, miRNA-451 levels were positively significantly correlated to eGFR and negatively correlated to ACR.
MicroRNA-451 was over- expressed in 50% of TM patients with poor compliance to chelation therapy versus 78.6% of TM patients with good compliance to chelation therapy (p=0.022).