Renal function in children with β-thalassemia major and thalassemia intermedia
- 352 Downloads
In β-thalassemia, profound anemia and severe hemosiderosis cause functional and physiological abnormalities in various organ systems. In recent years, there have been few published studies demonstrating proteinuria, aminoaciduria, low urine osmolality, and excess secretion of the tubular damage markers, such as urinary N-acetyl-D-glucosaminidase (UNAG) and β2 microglobulin, in patients with thalassemia. The object of this study was to analyze renal tubular and glomerular function in pediatric patients with β-thalassemia and to correlate the renal findings to iron overload. Thirty-seven patients with β-thalassemia major and 11 with thalassemia intermedia were studied. Twelve children without iron metabolism disorders or renal diseases served as a control group. No difference in blood urea nitrogen (BUN), serum creatinine, creatinine clearance, electrolytes, fractional excretion of sodium and potassium, and tubular phosphorus reabsorption was found. Serum uric acid was equal in the two groups, but its urine excretion was significantly higher in the thalassemic group. UNAG and UNAG to creatinine ratio (UNAG/CR) were elevated in all patients with thalassemia compared with the control group (p < 0.001) and were directly correlated to the amount of transfused iron but not to actual ferritin level. We found that renal tubular function is impaired in children with β- thalassemia major and intermedia. It is not known whether these functional abnormalities would have any long-term effects on the patients. Further studies are needed, and means of preventing these disturbances should be sought.
Keywordsβ-thalassemia major Thalassemia intermedia Renal tubular function Urine N-acetyl-D-glucosaminidase
- 1.Orkin SH, Nathan DG (1998) The thalassemias. In: Nathan DG, Orkin SH (eds) Nathan and Oski’s hematology of infancy and childhood. 5th edn. Saunders, Philadelphia, pp 811–886Google Scholar
- 3.Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E (2004) Thalassemia. Hematology Am Soc Hematol Educ Program 14–34Google Scholar
- 25.Shashaty G, Frankewich R, Chakraborti T, Choudary J, Al-Fayoumi S, Kacuba A, Castillo S, Robie-Suh K, Rieves D, Weiss K, Pazdur R (2006) Deferasirox for the treatment of chronic iron overload in transfusional hemosiderosis. Oncology (Williston Park) 20:1799–1806, 1811; discussion 1811–1713, 1817Google Scholar
- 26.Vichinsky E, Onyekwere O, Porter J, Swerdlow P, Eckman J, Lane P, Files B, Hassell K, Kelly P, Wilson F, Bernaudin F, Forni GL, Okpala I, Ressayre-Djaffer C, Alberti D, Holland J, Marks P, Fung E, Fischer R, Mueller BU, Coates T (2007) A randomised comparison of deferasirox versus deferoxamine for the treatment of transfusional iron overload in sickle cell disease. Br J Haematol 136:501–508PubMedCrossRefGoogle Scholar