title: Major beta-thalassemia, use of desferiexamine and renal proximal tubular damage.
abstract: OBJECTIVE
  Thalassemia is a quantitative hereditary hemoglobinopathy, which is one of common hereditary diseases in the Mediterranean region including Iran. Homozygotic beta-thalassemia patients have manifestations and complications such as severe anemia and multiorgan dysfunction. Recently there have been some concerns about renal complications in â-thalassemia but some controversies have been demonstrated. The aim of this study was to investigate the renal function of major â-thalassemia patients in comparison with control group.
  
  
  METHODS
  This was a historical cohort. Study population consisted of major beta-thalassemia patients who were under a management plan of thalassemia ward of Bo Ali sina hospital sari, IRAN (case group ) and uninvolved brothers or sisters of the patients (the control group). Two groups (case and control) were matched. Renal function of patients was investigated by measurement of renal and serum markers. Demographic and therapeutic data of patients were extracted from medical records. The data were analyzed using SPSS 11.
  
  
  RESULTS
  Eighty-four samples were studied in total, while 42 of them were from the case group and the rest from the control group. The case and control groups were matched according to gender and age. Mean age of patients was 21.3 +/- 5.2 years. Desferiexamin was dosed in amount of 70 +/- 19 mg/kg. There was no significant difference in the measures of 24-hour urine protein (beta-2 microglobin and fraction excretion of Na and K between case and control groups. Significant differences were however in the measures of Bun serum and creatinin as well as K (from both urine and serum). Gender, Hb, and level of ferritin related significantly with differences.
  
  
  CONCLUSIONS
  This study demonstrates no evidence of proximal tubular damage in major â-thalassemia patients. Nevertheless, the patients with severe anemia, high dose of desferal and high level of ferritin yielded an increase in the levels of Bun, serum K, and in some cases in uric acid (Tab. 4, Ref. 33).
authors.affiliation: Department of Pediatrics, Mazandaran University of Medical Sciences, Sari, Iran.
authors.forename: H M
authors.initials: HM
authors.lastname: Jafari
authors.forename: K
authors.initials: K
authors.lastname: Vahidshahi
authors.forename: M
authors.initials: M
authors.lastname: Kosaryan
authors.forename: H
authors.initials: H
authors.lastname: Karami
authors.forename: M
authors.initials: M
authors.lastname: Reza Mahdavi
authors.forename: S
authors.initials: S
authors.lastname: Ehteshami
mesh_terms: D000328:Adult; D003676:Deferoxamine / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007677:Kidney Function Tests; D007687:Kidney Tubules, Proximal / Q000503:physiopathology*; D008297:Male; D017086:beta-Thalassemia / Q000188:drug therapy* / Q000503:physiopathology
chemical_list: D003676:Deferoxamine