title: Renal functions in pediatric patients with beta-thalassemia major: relation to chelation therapy: original prospective study.
abstract: BACKGROUND
  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 mainly in adult demonstrating renal involvement in beta-thalassemia. This prospective study was aimed to investigate renal involvement in pediatric patients with transfusion dependent beta-thalassemia major (TD-betaTM), using both conventional and early markers of glomerular and tubular dysfunctions, and to correlate findings to oxidative stress and iron chelation therapy.
  
  
  METHODS
  Sixty-nine TD-betaTM patients (aged 1-16 years) and 15 healthy controls (aged 3-14 years) were enrolled in this study. Based on receiving chelation therapy (deferoxamine, DFO), patients were divided into two groups: group [I] with chelation (n=34) and group [II] without chelation (n=35). Levels of creatinine (Cr), calcium (Ca), inorganic phosphorus (PO4), uric acid (UA) and albumin were measured by spectrophotometer. Serum (S) levels of cystatin-C (SCysC) and total antioxidant capacity (STAC) and urinary (U) levels of beta2-microglobulin (Ubeta2MG) were measured by immunosorbent assay (ELISA). Urinary N-acetyl-beta-D-glucosaminidase (UNAG) activity and malondialdehyde (UMDA) were measured by chemical methods. Estimated glomerular filtration rate (eGFR) was determined from serum creatinine.
  
  
  RESULTS
  In patient with and without chelation, glomerular [elevated SCysC, SCr, Ualbumin/Cr and diminished eGFR]; and tubular dysfunctions [elevated SUA, SPO4, UNAG/Cr, Ubeta2MG/Cr] and oxidative stress marker disturbances [diminished STAC and elevated UMDA/Cr] were reported than controls. In patients with chelation, SCysC was significantly higher while, STAC was significantly lower than those without chelation. In all patients, SCysC showed significant positive correlation with SCr and negative correlation with eGFR; STAC showed significant positive correlation with eGFR and negative correlation with SCysC, SCr, UNAG/Cr; UMDA/Cr showed significant positive correlation with Ualbumin/Cr, Ubeta2MG/Cr, UNAG/Cr.
  
  
  CONCLUSIONS
  Our data confirm high frequency of glomerular and tubular dysfunctions in TD-betaTM pediatric patients which could be attributed to oxidative stress and DFO therapy.
authors.affiliation: Department of Physiology, Faculty of Medicine, Assiut University, Assiut, Egypt. eah3a2010@yahoo.com
authors.forename: Enas A
authors.initials: EA
authors.lastname: Hamed
authors.forename: Nagla T
authors.initials: NT
authors.lastname: ElMelegy
mesh_terms: D000293:Adolescent; D002614:Chelating Agents / Q000009:adverse effects* / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005500:Follow-Up Studies; D005919:Glomerular Filtration Rate / Q000187:drug effects*; D006801:Humans; D007223:Infant; D007674:Kidney Diseases / Q000139:chemically induced* / Q000378:metabolism / Q000503:physiopathology; D008297:Male; D018384:Oxidative Stress; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors; D017086:beta-Thalassemia / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism
chemical_list: D002614:Chelating Agents; D003404:Creatinine