Pediatric Nephrology

, 23:1847 | Cite as

Renal function in children with β-thalassemia major and thalassemia intermedia

  • Vladislav Smolkin
  • Raphael Halevy
  • Carina Levin
  • Miguel Mines
  • Waheeb Sakran
  • Katzap Ilia
  • Ariel Koren
Original Article


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.


β-thalassemia major Thalassemia intermedia Renal tubular function Urine N-acetyl-D-glucosaminidase 


  1. 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
  2. 2.
    Khalifa AS, Sheir S, el Magd LA, el Tayeb H, el Lamie O, Khalifa A, Mokhtar G (1985) The kidney in beta-thalassaemia major. Acta Haematol 74:60PubMedGoogle Scholar
  3. 3.
    Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E (2004) Thalassemia. Hematology Am Soc Hematol Educ Program 14–34Google Scholar
  4. 4.
    Lapatsanis P, Sbyrakis S, Vertos C, Karaklis BA, Dosiadis S (1976) Phosphaturia in thalassemia. Pediatrics 58:885–892PubMedGoogle Scholar
  5. 5.
    Mastrangelo F, Lopez T, Rizzelli S, Manisco G, Corliano C, Alfonso L (1975) Function of the kidney in adult patients with Cooley’s disease. A preliminary report. Nephron 14:229–236PubMedCrossRefGoogle Scholar
  6. 6.
    Shehab M, Barakat AY (1985) Thalassemia B with distal renal tubular acidosis: a previously undescribed association. Int J Pediatr Nephrol 6:143–144PubMedGoogle Scholar
  7. 7.
    Ong-ajyooth L, Malasit P, Ong-ajyooth S, Fucharoen S, Pootrakul P, Vasuvattakul S, Siritanaratkul N, Nilwarangkur S (1998) Renal function in adult beta-thalassemia/Hb E disease. Nephron 78:156–161PubMedCrossRefGoogle Scholar
  8. 8.
    Sumboonnanonda A, Malasit P, Tanphaichitr VS, Ong-ajyooth S, Sunthornchart S, Pattanakitsakul S, Petrarat S, Assateerawatt A, Vongjirad A (1998) Renal tubular function in beta-thalassemia. Pediatr Nephrol 12:280–283PubMedCrossRefGoogle Scholar
  9. 9.
    Aldudak B, Karabay Bayazit A, Noyan A, Ozel A, Anarat A, Sasmaz I, Kilinc Y, Gali E, Anarat R, Dikmen N (2000) Renal function in pediatric patients with beta-thalassemia major. Pediatr Nephrol 15:109–112PubMedCrossRefGoogle Scholar
  10. 10.
    Link G, Athias P, Grynberg A, Pinson A, Hershko C (1989) Effect of iron loading on transmembrane potential, contraction, and automaticity of rat ventricular muscle cells in culture. J Lab Clin Med 113:103–111PubMedGoogle Scholar
  11. 11.
    Thomas CE, Morehouse LA, Aust SD (1985) Ferritin and superoxide-dependent lipid peroxidation. J Biol Chem 260:3275–3280PubMedGoogle Scholar
  12. 12.
    Sumboonnanonda A, Malasit P, Tanphaichitr VS, Ong-ajyooth S, Petrarat S, Vongjirad A (2003) Renal tubular dysfunction in alpha-thalassemia. Pediatr Nephrol 18:257–260PubMedGoogle Scholar
  13. 13.
    Landing BH, Gonick HC, Nadorra RL, Hyman CB, Wells TR, Villarreal-Engelhardt G, Mersch J, Agness CL (1989) Renal lesions and clinical findings in thalassemia major and other chronic anemias with hemosiderosis. Pediatr Pathol 9:479–500PubMedCrossRefGoogle Scholar
  14. 14.
    Cianciulli P, Sollecito D, Sorrentino F, Forte L, Gilardi E, Massa A, Papa G, Carta S (1994) Early detection of nephrotoxic effects in thalassemic patients receiving desfferioxamine therapy. Kidney Int 46:467–470PubMedCrossRefGoogle Scholar
  15. 15.
    Ali D, Mehran K, Moghaddam AG (2008) Comparative evaluation of renal findings in Beta-thalassemia major and intermedia. Saudi J Kidney Dis Transpl 19:206–209PubMedGoogle Scholar
  16. 16.
    Piscator M (1991) Early detection of tubular dysfunction. Kidney Int Suppl 34:S15–17PubMedGoogle Scholar
  17. 17.
    Koren G, Bentur Y, Strong D, Harvey E, Klein J, Baumal R, Spielberg SP, Freedman MH (1989) Acute changes in renal function associated with deferoxamine therapy. Am J Dis Child 143:1077–1080PubMedGoogle Scholar
  18. 18.
    Mohkam M, Shamsian BS, Gharib A, Nariman S, Arzanian MT (2008) Early markers of renal dysfunction in patients with beta-thalassemia major. Pediatr Nephrol 23:971–976CrossRefPubMedGoogle Scholar
  19. 19.
    De Sanctis V, Vullo C, Bagni B, Chiccoli L (1992) Hypoparathyroidism in beta-thalassemia major. Clinical and laboratory observations in 24 patients. Acta Haematol 88:105–108PubMedCrossRefGoogle Scholar
  20. 20.
    Toumba M, Sergis A, Kanaris C, Skordis N (2007) Endocrine complications in patients with thalassaemia major. Pediatr Endocrinol Rev 5:642–648PubMedGoogle Scholar
  21. 21.
    Koren G, Kochavi-Atiya Y, Bentur Y, Olivieri NF (1991) The effects of subcutaneous deferoxamine administration on renal function in thalassemia major. Int J Hematol 54:371–375PubMedGoogle Scholar
  22. 22.
    Li Volti S, Di Gregorio F, Schiliro G (1990) Acute changes in renal function associated with deferoxamine therapy. Am J Dis Child 144:1069–1070PubMedGoogle Scholar
  23. 23.
    Porter JB (2006) Deferasirox: An effective once-daily orally active iron chelator. Drugs Today (Barc) 42:623–637CrossRefGoogle Scholar
  24. 24.
    Vichinsky E (2008) Clinical application of deferasirox: Practical patient management. Am J Hematol 83:398–402PubMedCrossRefGoogle Scholar
  25. 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. 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
  27. 27.
    Lindsey WT, Olin BR (2007) Deferasirox for transfusion-related iron overload: a clinical review. Clin Ther 29:2154–2166PubMedCrossRefGoogle Scholar
  28. 28.
    Brittenham GM, Griffith PM, Nienhuis AW, McLaren CE, Young NS, Tucker EE, Allen CJ, Farrell DE, Harris JW (1994) Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major. N Engl J Med 331:567–573PubMedCrossRefGoogle Scholar

Copyright information

© IPNA 2008

Authors and Affiliations

  • Vladislav Smolkin
    • 1
    • 2
  • Raphael Halevy
    • 1
    • 2
  • Carina Levin
    • 3
  • Miguel Mines
    • 4
  • Waheeb Sakran
    • 1
    • 5
  • Katzap Ilia
    • 1
  • Ariel Koren
    • 1
    • 3
    • 5
  1. 1.Pediatric Department “B”Ha’Emek Medical CenterAfulaIsrael
  2. 2.Pediatric Nephrology UnitHa’Emek Medical CenterAfulaIsrael
  3. 3.Pediatric Hematology UnitHa’Emek Medical CenterAfulaIsrael
  4. 4.Biochemistry LaboratoryHa’Emek Medical CenterAfulaIsrael
  5. 5.Baruch Rappaport School of Medicine, TechnionIsrael Institute of TechnologyHaifaIsrael

Personalised recommendations