title: Renal dysfunction in patients with beta-thalassemia major receiving iron chelation therapy either with deferoxamine and deferiprone or with deferasirox.
abstract: There are limited studies on renal involvement in beta-thalassemia, mainly involving patients on deferoxamine, reporting both glomerular and tubular dysfunction. The aim of the present study was to investigate renal involvement in young thalassemia patients, using both conventional and early markers of renal dysfunction, and to correlate findings to iron chelation therapy. Forty-two patients aged 4-23 years were studied and, for analysis purposes, were divided into two groups based on chelation therapy (group A receiving deferasirox and group B receiving deferoxamine and deferiprone combination therapy). In addition to conventional renal biochemistries, creatinine clearance, estimated glomerular filtration rate, serum cystatin C (Cys C), fractional excretion of sodium, tubular phosphorus reabsorption and urine calcium, protein, beta(2)-microglobulin (beta(2)-MG) and glucose levels were measured. A considerable number of patients demonstrated impaired renal function with elevated Cys C levels (36%), glomerular dysfunction with proteinuria (24%) and tubulopathy with hypercalciuria (35.5%) and elevated excretion of beta(2)-MG (33.5%). Renal involvement seems to be present even in young patients with beta-thalassemia, therefore, routine use of early markers of renal dysfunction is recommended. Further studies are needed in order to investigate the role of new chelators in tubular function parameters.
authors.affiliation: First Department of Pediatrics, Aristotle University of Thessaloniki, 'Hippokration' General Hospital, Thessaloniki, Greece. marina@med.auth.gr
authors.forename: Marina
authors.initials: M
authors.lastname: Economou
authors.forename: Nikoletta
authors.initials: N
authors.lastname: Printza
authors.forename: Aikaterini
authors.initials: A
authors.lastname: Teli
authors.forename: Vassiliki
authors.initials: V
authors.lastname: Tzimouli
authors.forename: Ioanna
authors.initials: I
authors.lastname: Tsatra
authors.forename: Fotis
authors.initials: F
authors.lastname: Papachristou
authors.forename: Miranda
authors.initials: M
authors.lastname: Athanassiou-Metaxa
mesh_terms: D000293:Adolescent; D000328:Adult; D001565:Benzoates / Q000009:adverse effects* / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood / Q000652:urine; D015913:Chelation Therapy / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D055316:Cystatin C / Q000097:blood; D000077588:Deferasirox; D000077543:Deferiprone; D003676:Deferoxamine / Q000009:adverse effects* / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D053565:Hypercalciuria; D007502:Iron Chelating Agents / Q000009:adverse effects* / Q000627:therapeutic use; D007674:Kidney Diseases / Q000097:blood / Q000139:chemically induced / Q000150:complications* / Q000652:urine; D007677:Kidney Function Tests; D008297:Male; D011507:Proteinuria; D011728:Pyridones / Q000009:adverse effects* / Q000627:therapeutic use; D014230:Triazoles / Q000009:adverse effects* / Q000627:therapeutic use; D055815:Young Adult; D001613:beta 2-Microglobulin / Q000652:urine; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications / Q000188:drug therapy* / Q000652:urine
chemical_list: D001565:Benzoates; D015415:Biomarkers; D055316:Cystatin C; D007502:Iron Chelating Agents; D011728:Pyridones; D014230:Triazoles; D001613:beta 2-Microglobulin; D000077543:Deferiprone; D003676:Deferoxamine; D000077588:Deferasirox