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Koomesh  2009 

Impaired renal tubular function in pediatric patients with β-thalassemia major

Keywords: β-thalassemia major , Renal tubular function , NAG (N.Acetyl-β.D.Glucosaminidase).

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Abstract:

Introduction: β-thalassemia major due to ineffective erythrocyte life span leads to severe anemiarequiring regular transfusion, which it can lead to major organs damage. Renal damage can beattributed to chronic anemia, iron over load and or defferoxamin toxicity. The object of this study wasto analyze renal tubular and glomerular function in pediatric patients with beta-thalassemia major.Materials and Methods: 39 patients with β -thalassemia major whose mean ages were 11.8 yr and22 sex and age matched healthy children as control group were studied. Blood rine samplesand uwereobtained for measuring biochemical markers. Urinary N-Acetyl-β.D.glucosaminidase (NAG) andurine NAG to creatine ratio (U Cr /NAGratio) were measuredResults: No significant differences were found between serum BUN, creatinine, and also creatinineclearance between both groups. Also there were no significant differences in urine osmolality betweenpatients and controls. Urine NAG was significantly higher in thalassemic patients than control group(p< 0.000). There was positive relation between urinary NAG and duration of illness(r = 0314,p<0.01). There was no significant relationship between urinary NAG and serum ferritin. Finally, nosignificant difference was found in GFR of both groups.Conclusion: Our study showed renal tubular function is impaired in in patients with β-thalassemiamajor as indicated with increased urinary NAG. Further, we have found a positive relationshipbetween urinary NAG and duration of disease. Thus, in spite of normal GFR and other biochemicalmarker such as BUN and creatinine, increased urinary NAG may be as an early marker of renaldisease in patients with β-thalassemia majo.

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