The Kidney in Beta-Thalassaemia major

ÖzetAmaç: Talaseminin dünyadaki en yaygın genetik hastalık olduğu düşünülmek-tedir. Gerek hastalık, gerekse tekrarlayan transfüzyonlar nedeniyle birçok sistemde komplikasyon ortaya çıkmaktadır. Bu çalışma; üzerinde daha az durulan böbrek glomerüler hasarının beta talasemi majorlü hastalarda mevcut olup olmadığının saptanması ve glomerüler hasarı saptarken üre, kreatinin, kreatinin klirensinin erken böbrek hasarı göstergeçlerinden olan sistatin c, B2 mikroglobulin ile kıyaslanması amacıyla yapıldı. Gereç ve Yöntem: Bu çalışma beta talasemi major tanısıyla çocuk hematoloji polikliniğimizce düzenli takip edilen hastalarda prospektif olarak yapılmıştır. Bulgular: Üre ile kreatinin klirensi ve sistain C arasında istatistiksel olarak anlamlı bir iliş-ki bulunmamaktadır(p>0.05). Kreatinin ile kreatinin klirensi arasında negatif yönde, %53.7 düzeyinde ve istatistiksel olarak ileri düzeyde anlamlı bir iliş-ki bulunmaktadır (p:0.002; p<0.01). Kreatinin ile sistain C ve Beta 2 mikroglobulin düzeyleri arasında istatistiksel olarak anlamlı bir ilişki bulunmamaktadır (p>0.05). Sistain C ile β-2 mikroglobulin arasında pozitif yönde, %86.1 düzeyinde ve istatistiksel olarak ileri düzeyde anlamlı bir ilişki bulunmaktadır (p:0.001; p<0.01). Tartışma: Literatürde GFR>40 ml/dk/1.73 m² olduğun-da kreatinin ile β-2 mikroglobulin ve sistatin c arası ilişkinin azaldığı belirtilmiştir. Bizim çalışmamız da bu literatür sonuçlarıyla benzerlik göstermekte-dir. Ancak renal parametrelerin birbiriyle korelasyonu hakkında kesin sonuç-lara varmak için daha geniş populasyonda yapılacak daha fazla sayıda çalış-malara ihtiyaç vardır. Anahtar KelimelerSistatin C; Böbrek; Talasemi; Beta 2-Mikroglobulin Abstract Aim: Thalassemia is accepted to be the most common genetic disease in the world. This study was performed to establish whether there was a glomerular renal damage, which was usually a less mentioned subject in patients with Beta Thalassemia Major, and to compare urea, creatinine and creatinine clearance with early indicators of kidney damage as Cystatin-C and β-2 microglobulin as on determining the glomerular damage. Material and Method:This study was prospectively performed in patients, who were regularly followed in the children hematology outpatient clinic with a diagnosis of Beta Thalassemia Major. Results: There was no statistically significant difference between urea and levels of creatinine clearance and Cystatin-C. There was a statistically negative relationship between creatinine and creatinine clearance at an advanced level as 53.7% (p: 0.002, p<0.01). There was not any significant relationship between Cystatin-C and levels of creatinine and B-2 microglobulin. There was a significant high positive relationship between Cystatin-C and B-2 microglobulin at level of 86.1% (p: 0.001; p<0.01). Discussion: The results of our study were also similar to the literature. However, new studies are required to carry out in wider populations in order to reach definite conclusions about correlation of renal parameters with each other.

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“…Increased [11,12]. In recent years, the research studies about effects of thalassemia over kidneys were started to perform.…”

Section: Discussionmentioning

confidence: 99%

Levels of Beta-2 Microglobulin and Cystatin C in Beta Thalassemia Major Patients

Kacar¹,

Şehitoğlu²,

Kaçar³

et al. 2015

Ann Clin Anal Med

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“…Patients with β-thalassemia major receive blood transfusions chronically, and each 1 ml of packed red cells increases the body's iron load by 1 mg. Increased iron deposition coming from multiple life-long transfusions and enhanced iron absorption results in secondary hemosiderosis [2]. Removal of excess iron with iron chelators, the most common of which is deferoxamine, produce iron excretion in urine and stool.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2008

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In beta-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 (U(NAG)) and beta2 microglobulin, in patients with thalassemia. The object of this study was to analyze renal tubular and glomerular function in pediatric patients with beta-thalassemia and to correlate the renal findings to iron overload. Thirty-seven patients with beta-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. U(NAG) and U(NAG) to creatinine ratio (U(NAG/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 beta- 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.

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“…The frequency of βthalassemia major in Turkey as a whole is 1.5-2.5%, and 0.9-3.9% in the southern part of the country known as the Çukurova region [1]. These patients receive blood transfusions chronically and each 1 ml of packed red blood cells increases the body's iron load by 1 mg [2]. With increased duration of survival of children with βthalassemia major, the effects of iron overload in the liver, pancreas, and heart become more severe, but renal involvement has received little attention [3].…”

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confidence: 99%

Renal function in pediatric patients with β-thalassemia major

Aldudak

Bayazıt

Noyan

et al. 2000

Pediatric Nephrology

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In patients with beta-thalassemia major, the most important cause of mortality and morbidity is organ failure due to deposits of iron. In this study, the nature of the kidney injury and possible pathogenetic factors were investigated. Seventy children with beta-thalassemia major and 14 age and sex-matched healthy children were involved in the study. Blood and timed urine samples were obtained for hematological and biochemical tests. The mean values of blood urea nitrogen (BUN), serum creatinine, creatinine clearance, serum sodium, urine osmolality, fractional excretion of sodium, potassium, and uric acid were not statistically different between the groups. Serum levels of potassium, phosphorus, and uric acid and the urine volume, high urinary protein to creatinine (UP/Cr), urinary N-acetyl-beta-D-glucosaminidase to creatinine (UNAG/Cr), and urinary malondialdehyde to creatinine, (UMDA/Cr) and the tubular phosphate reabsorption (TRP) values were statistically different between two groups (P<0.05). Increased serum levels of potassium, phosphorus, and uric acid in the patient group were attributed to the rapid erythrocyte turnover. The presence of high UP/cr, UNAG/Cr and UMDA/Cr ratios shows that in these patients with proximal renal tubular damage may be secondary to oxidative lipid peroxidation mediated by the iron overload.

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The Kidney in Beta-Thalassaemia major

Cited by 8 publications

References 1 publication

Levels of Beta-2 Microglobulin and Cystatin C in Beta Thalassemia Major Patients

Levels of Beta-2 Microglobulin and Cystatin C in Beta Thalassemia Major Patients

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

Renal function in pediatric patients with β-thalassemia major

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