ABSTRACT

Background: The thalassemia family of genetic disorders is characterized by an abnormal synthesis of the hemoglobin chain, resulting in chronic anemia in some people and clinically asymptomatic in others. There have been very few studies conducted on the effects of thalassemia on the kidneys, which typically included patients treated with deferoxamine having both glomerular and tubular dysfunction.

Objective: This research aimed to determine whether kidney dysfunction is observed in young thalassemic individuals manipulating both conventional and primitive markers of kidney dysfunction, and to associate the results to the use of iron chelation therapy.

Methods: We measured serum cystatin C (Cys C), tubular phosphorus reabsorption (TPRA), fractional sodium excretion (FNE), and 2-microglobulin, urine calcium, protein, and glucose levels in addition to the usual renal biochemistry.

Results: A whole of 42 individuals, ranging in age from 4 to 23, were included  in this study and were split into two groups for study (group A taking deferoxamine alone, and group B taking deferiprone coupled with deferoxamine). An increased Cys C level (36%) and proteinuria (24%) were seen in a large percentage of patients, as were tubulopathy with hypercalciuria (35.5 %) and point out 2-MG excretion (33.5 %).

Conclusion: Patients with -thalassemia seem to suffer from renal dysfunction even when they are young, so it is important to monitor early signs of renal dysfunction. There is a need for further research into the effect of novel chelators on parameters tubular function.