title: Renal function in children with beta-thalassemia major and thalassemia intermedia.
abstract: In beta-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 (U(NAG)) and beta2 microglobulin, in patients with thalassemia. The object of this study was to analyze renal tubular and glomerular function in pediatric patients with beta-thalassemia and to correlate the renal findings to iron overload. Thirty-seven patients with beta-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. U(NAG) and U(NAG) to creatinine ratio (U(NAG/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 beta- 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.
authors.affiliation: Pediatric Department B, Ha'Emek Medical Center, 18101, Afula, Israel. vlsmolkin@yahoo.com
authors.forename: Vladislav
authors.initials: V
authors.lastname: Smolkin
authors.forename: Raphael
authors.initials: R
authors.lastname: Halevy
authors.forename: Carina
authors.initials: C
authors.lastname: Levin
authors.forename: Miguel
authors.initials: M
authors.lastname: Mines
authors.forename: Waheeb
authors.initials: W
authors.lastname: Sakran
authors.forename: Katzap
authors.initials: K
authors.lastname: Ilia
authors.forename: Ariel
authors.initials: A
authors.lastname: Koren
mesh_terms: D000118:Acetylglucosaminidase / Q000652:urine; D000293:Adolescent; D001806:Blood Urea Nitrogen; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000652:urine; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007668:Kidney / Q000503:physiopathology*; D008297:Male; D013789:Thalassemia / Q000503:physiopathology*; D017086:beta-Thalassemia / Q000503:physiopathology*
chemical_list: D005293:Ferritins; D003404:Creatinine; D000118:Acetylglucosaminidase