Abstract
BackgroundRenal injury in transfusion dependent β thalassemia patients (TDT) has been attributed to iron overload, chronic anemia and iron-chelation therapy (ICT) toxicity. We studied renal function in TDT patients treated with two different ICT regimes.Patients and methodsWe studied 36 TDT patients: 26 received deferasirox (DFX) and 10 were treated with deferoxamine (DFO) +/− deferiprone (DFP).ResultsIncreased uNAG was found in 30% of the DFX group vs. 10% of the DFO+/−DFP group, the mean uNAG level in the DFX group was significantly higher than in the DFO+/−DFP group, (P < 0.05). A moderate negative correlation was found between uNAG levels and mean serum ferritin for the prior 10 years (P = 0.03), more pronounced for the DFO+/−DFP group. Twenty nine patients had had their renal function evaluated 10 years earlier; eGFR significantly declined in patients switched to DFX (P = 0.0093) but not in patients who continued DFO+/−DFP.ConclusionsA high prevalence of renal tubular damage was observed in our TDT patients, particularly those treated with DFX; uNAG was negatively associated with mean 10-year serum ferritin, suggesting ICT’s involvement in tubular injury. A significant decline in eGFR compared to a decade earlier was observed only in patients currently treated with DFX. Strict follow-up of renal function in TDT patients is warranted.
Highlights
Introduction βThalassemia major (β-TM) is a disease of hemoglobin synthesis leading to ineffective erythropoiesis and transfusion-dependent anemia from the first few months of life
Renal tubular dysfunctions reported in transfusion dependent β thalassemia patients (TDT) patients include low-molecular-weight proteinuria, found in almost all patients, and increased urinary excretion of calcium, phosphorus, magnesium and uric acid [3,4,5, 7]
N-acetyl-β-D-glucosaminidase (NAG) is a hydrolytic enzyme found in proximal tubular cells, Urinary NAG levels are considered a reliable marker for early renal tubular injury [8, 9] and were found to be elevated in 35 to 60% of TDT patients [5, 6, 8, 10, 11]
Summary
Introduction βThalassemia major (β-TM) is a disease of hemoglobin synthesis leading to ineffective erythropoiesis and transfusion-dependent anemia from the first few months of life. Regular blood transfusions and iron chelation therapy (ICT) markedly improve the survival and quality of life of Transfusion Dependent β Thalassemia (TDT) patients [1, 2], but have led to the emergence of. Hematuria has been observed in 2 to 10% of TDT patients and might be attributed to the increased incidence of nephrolithiasis [3, 7]. Renal injury in transfusion dependent β thalassemia patients (TDT) has been attributed to iron overload, chronic anemia and iron-chelation therapy (ICT) toxicity. We studied renal function in TDT patients treated with two different ICT regimes
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