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Renal tubular dysfunction in α-thalassemia

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Abstract.

Shortened red cell life span and excess iron cause functional and physiological abnormalities in various organ systems in thalassemia patients. In an earlier study, we showed that β–thalassemia patients have a high prevalence of renal tubular abnormalities. The severity correlated with the degree of anemia, being least severe in patients on hypertransfusion and iron chelation therapy, suggesting that the damage might be caused by the anemia and increased oxidation induced by excess iron deposits. This study was designed to define the renal abnormalities associated with α–thalassemia and to correlate the renal findings with clinical parameters. Thirty-four pediatric patients (mean age 8.2±2.8 years) with Hb H disease or Hb H/Hb CS were studied. Ten patients (group 1) were splenectomized, with a mean duration post splenectomy of 3.5±1.4 years; 24 patients (group 2) had intact spleens. The results were compared with 15 normal children. Significantly higher levels of urine N-acetyl-β-d-glycosaminidase, malondialdehyde (MDA), and β2-microglobulin were found in both groups compared with normal children. An elevated urine protein/creatinine ratio was recorded in 60% of group 1 and 29% of group 2. Two patients (5.9%), 1 in each group, had generalized aminoaciduria. We found proximal tubular abnormalities in α–thalassemia patients. Increased oxidative stress, possibly iron induced, may play an important role, since urine MDA levels were significantly increased in both groups of patients.

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Acknowledgement.

This study was funded by the Siriraj Grant for Research Development and Medical Education, Siriraj Hospital, Thailand (grant no. 75–348–292). P. Malasit is a recipient of the Senior Scholar Grant from the Thailand Research Fund. The Medical Molecular Biology Laboratory also operates as the Medical Biotechnology Unit funded by the National Center for Genetic Engineering (BIOTEC) of the National Science and Technology Development Agency (NSTDA), Thailand.

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Authors and Affiliations

  1. Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Prannok Road, Bangkok 10700, Thailand

    Achra Sumboonnanonda, Voravarn S. Tanphaichitr, Siripan Petrarat & Arun Vongjirad

  2. Medical Molecular Biology Unit, Office for Research and Development, Siriraj Hospital, Mahidol University, Prannok Road, Bangkok 10700, Thailand

    Prida Malasit

  3. Department of Biochemistry, Siriraj Hospital, Mahidol University, Prannok Road, Bangkok 10700, Thailand

    Sompong Ong–ajyooth

Authors
  1. Achra Sumboonnanonda
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  2. Prida Malasit
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  3. Voravarn S. Tanphaichitr
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  4. Sompong Ong–ajyooth
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  5. Siripan Petrarat
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  6. Arun Vongjirad
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Correspondence to Achra Sumboonnanonda.

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Sumboonnanonda, A., Malasit, P., Tanphaichitr, V.S. et al. Renal tubular dysfunction in α-thalassemia. Pediatr Nephrol 18, 257–260 (2003). https://doi.org/10.1007/s00467-003-1067-7

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  • DOI: https://doi.org/10.1007/s00467-003-1067-7

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