Document Detail

Renal tubular dysfunction in alpha-thalassemia.
MedLine Citation:
PMID:  12644919     Owner:  NLM     Status:  MEDLINE    
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 beta-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 alpha-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-beta- d-glycosaminidase, malondialdehyde (MDA), and beta(2)-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 alpha-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.
Achra Sumboonnanonda; Prida Malasit; Voravarn S Tanphaichitr; Sompong Ong-ajyooth; Siripan Petrarat; Arun Vongjirad
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2003-02-26
Journal Detail:
Title:  Pediatric nephrology (Berlin, Germany)     Volume:  18     ISSN:  0931-041X     ISO Abbreviation:  Pediatr. Nephrol.     Publication Date:  2003 Mar 
Date Detail:
Created Date:  2003-03-19     Completed Date:  2003-12-03     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  8708728     Medline TA:  Pediatr Nephrol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  257-60     Citation Subset:  IM    
Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Prannok Road, Bangkok 10700, Thailand.
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MeSH Terms
Acetylglucosaminidase / urine
Blood Urea Nitrogen
Child, Preschool
Creatinine / blood
Kidney Diseases / etiology*,  physiopathology*
Kidney Tubules / physiopathology*
Malondialdehyde / urine
Oxidative Stress
Renal Aminoacidurias / etiology,  physiopathology
alpha-Thalassemia / complications*,  physiopathology*
beta 2-Microglobulin / urine
Reg. No./Substance:
0/beta 2-Microglobulin; 542-78-9/Malondialdehyde; 60-27-5/Creatinine; EC

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