ISSN: 1300-7777 E-ISSN: 1308-5263
Glomerular and Tubular Functions in Children and Adults with Transfusion-Dependent Thalassemia [Turk J Hematol]
Turk J Hematol. 2018; 35(1): 66-70 | DOI: 10.4274/tjh.2017.0266  

Glomerular and Tubular Functions in Children and Adults with Transfusion-Dependent Thalassemia

Agageldi Annayev1, Zeynep Karakas1, Serap Karaman1, Altan Yalciner2, Alev Yilmaz3, Sevinc Emre3
1İstanbul University İstanbul Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey
2Düzen Laboratories, İstanbul, Turkey
3İstanbul University İstanbul Faculty of Medicine, Department of Pediatric Nephrology, İstanbul, Turkey

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 β2-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 β2-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 β2-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, β, 2 Microglobulin, Cystatin


Transfüzyona Bağımlı Çocuk ve Erişkin Talasemi Hastalarında Glomerüler ve Tubuler Böbrek Fonksiyonları

Agageldi Annayev1, Zeynep Karakas1, Serap Karaman1, Altan Yalciner2, Alev Yilmaz3, Sevinc Emre3
1İstanbul University İstanbul Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey
2Düzen Laboratories, İstanbul, Turkey
3İstanbul University İstanbul Faculty of Medicine, Department of Pediatric Nephrology, İstanbul, Turkey

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 β2-mikroglobülin düzeyleri nefelometrik yöntemle ölçüldü. Otuz sekiz hasta deferasiroks, 8 hasta deferipron alıyordu. Hastaların serum elektrolitleri ve albumin düzeyleri normal sınırlardaydı. İdrar β2mikroglobulin ve serum sistatin-C düzeyleri hasta grubunda kontrol grubundakilere göre anlamlı derecede yüksekti. Serum Cys-C ve idrar β2-mikroglobulin düzeyleri, deferipron kullananlar ve kontrol grubu arasında anlamlı farklılık göstermezken, deferasiroks kullananlarda kontrol grubuna göre daha yüksek bulundu. İdrar β2 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 Kelimeler: Talasemi, Tubulopati, Glomerulopati, β, 2 Mikroglobulin, Sistatin C


Agageldi Annayev, Zeynep Karakas, Serap Karaman, Altan Yalciner, Alev Yilmaz, Sevinc Emre. Glomerular and Tubular Functions in Children and Adults with Transfusion-Dependent Thalassemia. Turk J Hematol. 2018; 35(1): 66-70

Corresponding Author: Serap Karaman, Türkiye


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