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Saudi Journal of Kidney Diseases and Transplantation
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ORIGINAL ARTICLE Table of Contents   
Year : 2008  |  Volume : 19  |  Issue : 2  |  Page : 206-209
Comparative Evaluation of Renal Findings in Beta-Thalassemia Major and Intermedia


1 Shiraz Nephro-Urology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
2 Hematology Oncology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran
3 Department of Pediatrics, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran

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   Abstract 

Thalassemia is a systematic disease in which the renal involvement has not yet been scrupulously studied. In a cross-sectional study, the renal findings of 50 cases of thalassemia intermedia (group 1) were compared to 58 patients with thalassemia major (group 2). Blood urea nitrogen, serum creatinine, uric acid, calcium, phosphorus, urinalysis, and ultrasonographical findings were evaluated. Mean age was 18 ± 3.0 in group 1 and 17 ± 3.5 years in group 2. The mean of serum ferritin levels was 871 ± 81.8 ng/ml in group 1 vs. 3503 ± 201 ng/ml in thalassemia major (p < 0.05). Ninety-two percent of the patients in group 1 were on hydroxyurea at the time of evaluation. Serum uric acid was significantly higher in group 1 than group 2 patients (5.74 ± 2.95 vs. 4.12 ± 0.9 mg/dl, p < 0.05). Microscopic hematuria (red blood cell in high power field of urine microscopy > 5) was observed among 19 children (17.6%); 17 of them were in group 1. In contrast, children with thalassemia major had significantly higher serum creatinine (0.89 ± 0.18 vs. 0.59 ± 0.37 mg/dl, p < 0.05) and blood urea nitrogen values (12.14 ± 5.58 vs. 13.85 ± 3.54 mg/dl, p < 0.05). We conclude that significant renal involvement is not a frequent complication in children and young adults suffering from thalassemia. Hyperuricemia and microscopic hematuria are more common in thalassemia intermedia than thalassemia major. Microscopic hematuria in thalassemia intermedia might be related to either hypercalciuria or hyperuricosuria.

Keywords: Thalassemia, Renal, Hyperuricosuria, Hypercalciuria, Hematuria, Hyperuricemia

How to cite this article:
Ali D, Mehran K, Moghaddam AG. Comparative Evaluation of Renal Findings in Beta-Thalassemia Major and Intermedia. Saudi J Kidney Dis Transpl 2008;19:206-9

How to cite this URL:
Ali D, Mehran K, Moghaddam AG. Comparative Evaluation of Renal Findings in Beta-Thalassemia Major and Intermedia. Saudi J Kidney Dis Transpl [serial online] 2008 [cited 2019 Nov 17];19:206-9. Available from: http://www.sjkdt.org/text.asp?2008/19/2/206/39031

   Introduction Top


Thalassemia was first described by Dr. Cooley in 1925. [1] It is more prevalent in the Mediterranean Region, North and West Africa, the Middle East and some other parts of the world. [2] About 150 million people worldwide carry beta-thalassemia gene. High frequency of thalassemia gene is encountered in Sardinia (11-34%), Greece (5-15%), and Iran (4-10%). [3],[4],[5]

Thalassemia is the most common type of genetic abnormality in the world. According to our health ministry data, there are 20,000 registered thalassemia patients in Iran, of whom health care has a large burden on health budget and family economics.

Despite advances in chelating therapy, some major complications of thalassemia are inevitable. A large number of studies have been performed on different complications of thalassemia, however, there are a few studies on the renal involvement in this disease. [6],[7],[8]

We aim in this study to evaluate the renal manifestations in patients with thalassemia major and intermedia.


   Materials and Methods Top


We randomly selected 50 thalassemia inter­media children, who were under regular follow-up of a hematologist and did not receive regular transfusions (group 1), and compared their renal findings with 58 thalassemia major children who were on regular blood transfusion and chelation therapy (group 2). Thalassemia was diag­nosed in the study patients by complete blood count and hemoglobin electrophoresis. Children with diabetes and major cardiac complications were excluded from the study. The study protocol and laboratory evalua­tions were discussed with the patients and their parents and a written consent was obtained.

The medical records were reviewed meti­culously, and a complete physical exami­nation was performed. Repeated investigations included hemoglobin, urinalysis, serum calcium, phosphate, blood urea nitrogen (BUN), creatinine, serum ferritin, and abdo­minal ultrasonography. All the laboratory investigations were performed in the same reference laboratory, and all ultrasono­graphical evaluations were performed by the same expert ultrasonographist.


   Statistical analysis Top


Statistical analysis was performed by SPSS 11.5 software and T-test. P <0.05 was considered significant.


   Results Top


Mean age was 18 ± 3 years (range, 4-24) in group 1 and 17 ± 3.5 years (range, 3-24) in group 2. Age at 1 st blood transfusion or diagnosis was 3.24 ± 3.38 and 1.6 ± 1.31 years in group1 and 2, respectively. Children in thalassemia major group received more regular infusions of desferrioxamine than thalassemia intermedia group (72.5% vs. 10%, p < 0.05). Hemoglobin level was 9.81 ± 1.13 and 9.11 ± 0.83 g/dL in group 1 and 2, respectively. Serum ferritin level was significantly higher in thalassemia major children (3503 ± 201 vs. 871 ± 818 ng/ml; p < 0.05). Ninety-two percent of thalassemia intermedia children were on hydroxyurea at the time of evaluation.

Serum uric acid was significantly higher in group 1 patients than group 2 (5.74 ± 2.95 vs. 4.12 ± 0.9 mg/dL, p < 0.05). Calcium and phosphorus levels were higher in group 1 than group 2 (9.08 ± 1.03 and 4 ± 0.53 mg/dL vs. 8.69 ± 0.99 and 3.46 ± 0.51 mg/dl, respectively, p < 0.05). In contrast, group 2 patients revealed significantly higher serum creatinine and BUN levels than group 1 (0.89 ± 0.18 vs. 0.59 ± 0.37 mg/dL, and 12.1 ± 5.58 vs. 13.9 ± 3.54 mg/dL, respectively, p < 0.05).

Microscopic hematuria (red blood cell in high power field of urine microscopy > 5) was observed among 19 (17.6%) children, of whom 17 patients had thalassemia intermedia. Pyuria was also more common in group 1 than group 2 (12% vs. 5%, P: not significant). On the other hand, hemoglobinuria was observed in 10% of group 2 patients, but in none of the patients in group 1.

The mean renal size was slightly larger in group 1 than group 2, but was not statis­tically significant. A small renal stone of nonclinical importance was noticed in 2 children in group 1 and 1 child in group 2.


   Discussion Top


Blood transfusion is life saving for patients with thalassemia major, but loads body with excess iron that may result in hemosiderosis and its complications, inclu­ding multiple endocrinopathies, cardiomyo­pathy, and hepatic failure. Survival of patients has greatly improved following the intro­duction of desferrioxamine and regular iron chelation when serum ferritin level is maintained below 2,000 µg/L. [9] High serum ferritin level in thalassemia major group in this study was related to higher frequencies of blood transfusion and suboptimal use of desferrioxamine mostly due to poor compliance.

Although renal involvement could occur at tubular level in thalassemia major [6],[7],[8] and minor, [10] significant renal findings are not common. Renal failure is a terminal event in thalassemia major and is usually secondary to heart failure and/or hepatic failure. Acute renal failure following deferoxamine over­ dose or hemolysis has been reported. [11],[12],[13] There are numerous reports on complications of β-thalassemia in different organs, [14],[15],[16],[17],[18] but a few on renal complications. [6],[7],[8],[10] The results of this study compared the renal findings in children with thalassemia major and thalassemia intermedia. Children with thalassemia major had higher BUN and creatinine levels possibly due to higher iron deposition in their kidneys. Calcium and phosphate levels was lower in thalassemia major, which could be related either to liver or parathyroid involvement. However, the simultaneous low phosphorus level in our patients could not be explained by hypo­parathyroidism. We found significantly higher levels of uric acid in thalassemia intermedia group, which was predictable due to the higher cellular turnover secondary to the use of hydroxyurea. [19]

An important finding in our study was the significant number of children in thalassemia intermedia group who revealed microscopic hematuria. This finding might be related to either hyperuricosuria and hypercalciuria. However, further studies are required to confirm this speculation.

We conclude that significant renal involve­ment is not a frequent complication in children and young adults suffering from thalassemia. Hyperuricemia and microscopic hematuria are more common in thalassemia intermedia than thalassemia major. Micros­copic hematuria in thalassemia intermedia might be related to either.


   Acknowledgement Top


The authors wish to thank the vice chancellor for research for his financial support and also Dr. Hamed Jalaeian for his editorial assistance.

 
   References Top

1.Cooley TB, Lee P. Series of cases of splenomegaly in children with anemia and peculiar bone change. Trans Am Pediatr Soc 1925;37:29.  Back to cited text no. 1    
2.Weatheall DJ, Clegg JB. The Thalassemia Syndrome, 3 rd ed. Boston, Blackwell Scientific Publication, 1981.  Back to cited text no. 2    
3.Guiso L, Frogheri L, Pistidda P, et al. Frequency of delta+ 27-thalassaemia in Sardinians. Clin Lab Haematol 1996;18(4): 241-4.  Back to cited text no. 3    
4.Lukens JN. The thalassemias and related disorders, quantitative disorders of hemoglobin synthesis. In: Lee GR, Bithell TC, Foerster J, et al, eds. Wintrobe's Clinical Hematology. 9th ed. Philadelphia: Lea & Febiger; 1993: 1102-45.  Back to cited text no. 4    
5.Haghshenas M, Zamani J. [Thalassemia]. 1st ed. Shiraz: Shiraz University of Medical Sciences Publishing Center, 1997. [Book in Persian]  Back to cited text no. 5    
6.Koliakos G, Papachristou F, Koussi A, et al. Urine biochemical markers of early renal dysfunction are associated with iron overload in beta-thalassaemia. Clin Lab Haematol 2003;25(2):105-9.  Back to cited text no. 6    
7.Michelakakis H, Dimitriou E, Georgakis H, et al. Iron overload and urinary lysosomal enzyme levels in beta-thalassaemia major. Eur J Pediatr 1997;156(8):602-4.  Back to cited text no. 7    
8.Aldudak B, Karabay Bayazit A, Noyan A, et al. Renal function in pediatric patients with beta-thalassemia major. Pediatr Nephrol 2000;15(1-2):109-12.  Back to cited text no. 8    
9.Low LC. Growth, puberty and endocrine function in beta-thalassaemia major. J Pediatr Endocrinol Metab 1997;10(2):175-84.  Back to cited text no. 9    
10.Cetin T, Oktenli C, Ozgurtas T, et al. Renal tubular dysfunction in beta-thalassemia minor. Am J Kidney Dis 2003;42(6):1164-8.  Back to cited text no. 10    
11.Cianciulli P, Sorrentino F, Forte L, et al. Acute renal failure occurring during intra­venous desferrioxamine therapy: Recovery after haemodialysis. Haematologica 1992; 77(6):514-5.  Back to cited text no. 11    
12.Prasannan L, Flynn JT, Levine JE. Acute renal failure following desferrioxamine overdose. Pediatr Nephrol 2003;18(3):283-5.  Back to cited text no. 12    
13.Niwa T, Imoto M, Okubo M, Sassa H, Matsui E. Acute renal failure due to rhabdomyohaemolysis in beta-thalassaemic trait. Lancet 1979;2(8140):476-7.  Back to cited text no. 13    
14.Low LC. Growth of children with [3­thalassemia major. Indian J Pediatr 2005; 72(2):159-64.  Back to cited text no. 14    
15.Al-Rimawi HS, Jallad MF, Amarin ZO, Obeidat BR. Hypothalamic-pituitary-gonadal function in adolescent females with beta­thalassemia major. Int J Gynaecol Obstet 2005;90(1):44-7.  Back to cited text no. 15    
16.Gamberini MR, Fortini M, De Sanctis V, Gilli G, Testa MR. Diabetes mellitus and impaired glucose tolerance in thalassaemia major: incidence, prevalence, risk factors and survival in patients followed in the Ferrara Center. Pediatr Endocrinol Rev 2004;2(Suppl 2):285-91.  Back to cited text no. 16  [PUBMED]  
17.Angelopoulos NG, Goula A, Rombopoulos G, Kaltzidou V, Katounda E, Kaltsas D,et al. Hypoparathyroidism in transfusion­dependent patients with beta-thalassemia. J Bone Miner Metab 2006;24(2):138-45.  Back to cited text no. 17    
18.Grundy RG, Woods KA, Savage MO, Evans JP. Relationship of endocrinopathy to iron chelation status in young patients with thalassaemia major. Arch Dis Child 1994;71(2):128-32.  Back to cited text no. 18    
19.Samuels ML, Howe CD. Renal abnormalities induced by hydroxyurea. Cancer Chemother Rep 1964;40:9-13.  Back to cited text no. 19  [PUBMED]  

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Correspondence Address:
Derakhshan Ali
Associate Professor of Pediatrics and Pediatric Nephrology, Shiraz Nephro-Urology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz
Iran
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PMID: 18310868

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    Abstract
    Introduction
    Materials and Me...
    Statistical analysis
    Results
    Discussion
    Acknowledgement
    References
 

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