Content area
Full Text
Abstract
This study aimed at assessing renal functions in patients with transfusion-dependent thalassemia (TDT). Fifty patients and 30 controls were enrolled in this prospective study. Serum levels of electrolytes and albumin were measured by a spectrophotometer. Serum levels of cystatin-C and urinary levels of ß2-microglobulin were measured by nephelometric method. Thirty-eight patients were receiving deferasirox and 8 were on deferiprone. Serum electrolytes and albumin levels of the patients were found to be within normal ranges. Urinary ß2-microglobulin and serum cystatin-C levels were significantly higher in patients than controls. They did not significantly differ between the subgroup of patients on deferiprone and the control group, whereas they were found to be higher in patients using deferasirox compared to controls. Urinary ß2-microglobulin levels significantly increased in patients who were receiving high-dose deferasirox compared to those who were receiving a daily dose of 15-20 mg/kg or controls. Subclinical renal injury may be present in TDT patients.
Keywords: Thalassemia, Tubulopathy, Glomerulopathy, ß2Microglobulin, Cystatin
Öz
Bu çalışmada transfüzyona bağımlı talasemi (TBT) hastalarında böbrek fonksiyonlarının değerlendirilmesi amaçlanmıştır. Prospektif çalışmaya, 50 TBT ve 30 kontrol grubu dahil edildi. Serum elektrolitleri ve albumin düzeyleri spektrofotometre ile ölçüldü. Serum sistatin-C ve idrar ß2-mikroglobülin düzeyleri nefelometrik yöntemle ölçüldü. Otuz sekiz hasta deferasiroks, 8 hasta deferipron alıyordu. Hastaların serum elektrolitleri ve albumin düzeyleri normal sınırlardaydı. İdrar ß2mikroglobulin ve serum sistatin-C düzeyleri hasta grubunda kontrol grubundakilere göre anlamlı derecede yüksekti. Serum Cys-C ve idrar ß2-mikroglobulin düzeyleri, deferipron kullananlar ve kontrol grubu arasında anlamlı farklılık göstermezken, deferasiroks kullananlarda kontrol grubuna göre daha yüksek bulundu. İdrar ß2 mikroglobulin düzeyleri, yüksek doz deferasiroks alan hastalarda, 15-20 mg/kg/ gün deferasiroks alanlara veya kontrol grubuna göre anlamlı şekilde artmıştı. Transfüzyona bağımlı talasemi hastalarında subklinik olarak renal hasar mevcut olabilir.
Anahtar Sözcükler: Talasemi, Tubulopati, Glomerulopati, ß2Mikroglobulin, Sistatin C
Introduction
Iron accumulation may lead to renal damage in transfusiondependent thalassemia (TDT) [1,2]. Cystatin C (Cys-C), a small molecular weight protein, is filtered from the glomeruli, reabsorbed from the tubular cells, and metabolized from the kidneys. It is a good marker for glomerular filtration rate (GFR). ß2-Microglobulin (ß2MG), a single-chain, low-molecular-weight polypeptide, is filtered by the glomeruli, then reabsorbed and catabolized in the proximal tubular cells. Increased urinary excretion of ß2MG may demonstrate tubular dysfunction. Our study assessed kidney function in patients with TDT...