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• W1981193243 abstract "Background/Aims: Renal manifestations have been described in β-thalassemia major and were attributed to transfusional iron overload and chelation therapy. Patients with the milder phenotype, β-thalassemia intermedia (TI), remain largely transfusion and iron chelation independent while enduring a chronic hemolytic anemia and primary iron overload. Data on renal function in patients with TI is lacking. Methods: In this cross-sectional study of 50 TI patients, we evaluated the association of estimated glomerular filtration rate (eGFR) and urinary protein to creatinine (UPr/UCr) ratio with relevant patient, disease and laboratory indices. Results: The median age of patients was 28 years (44% males). The eGFR was >90 ml/min/1.73 m2 in all patients, with a median value of 142.3 ml/min/1.73 m2. The median UPr/UCr ratio was 213.2 mg/g. There was a negative correlation between age and eGFR, while the UPr/UCr ratio correlated positively with markers of anemia, hemolysis and iron overload. A total of 24 (48%) patients had evidence of glomerular hyperfiltration, while 7 (14%) had proteinuria (UPr/UCr ratio >500 mg/g). Patients with proteinuria were characterized by elevated liver iron concentration (>7 mg Fe/g dry weight), non-transferrin-bound iron levels and nucleated red blood cell counts. Conclusions: A considerable proportion of TI patients show evidence of abnormally elevated eGFR, with a declining trend towards advancing age. The occurrence of proteinuria is associated with anemia, hemolysis and iron toxicity." @default.
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• W1981193243 date "2012-12-08" @default.
• W1981193243 modified "2023-09-26" @default.
• W1981193243 title "Glomerular Hyperfiltration and Proteinuria in Transfusion-Independent Patients with β-Thalassemia Intermedia" @default.
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• W1981193243 doi "https://doi.org/10.1159/000339787" @default.
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