Renal Hemosiderosis among Iranian Transfusion Dependent β-Thalassemia Major Patients
Background:In recent years, the success in management of thalassemic patients, has allowed for some previously unrecognized complications including renal abnormalities to emerge. This prospective study aimed to investigate kidney iron overload by means of MRI T2* and also renal function based on laboratory tests for early markers of glomerular and tubular dysfunction among adult Iranian transfusion-dependent thalassemia major patients.
Subjects and Methods: Two-hundred and two patients with transfusion-dependent β-thalassemia major were included in this study in Zafar Adult Thalassemia Center, Tehran, Iran. For all patients, kidney MRI T2* as well as evaluation of BUN, creatinine, uric acid, calcium, phosphorus, sodium (Na), potassium (K), total protein, albumin, cystatin C, serum ferritin β2-microglobulin, NAG (N-acetyl-beta-D-Glucosaminidase), and urine protein were performed.
Results: One-hundred and fourteen female and 88 male transfusion-dependent β-thalassemia major patients with mean age of 30.1 ± 9.4 participated in the present study. We found that 77.7% of our patients had kidney hemosiderosis based on MRI T2*. Also, 67 patients (33.2%) had elevation of serum cystatin C, and 104 patients (51.5%) had reduced estimated glomerular filtration rate (e-GFR). Increased urinary excretion of NAG and hypercalciuria were found in 50% and 79.2% of participants, respectively.
Conclusion: Renal hemosiderosis and asymptomatic renal dysfunction are prevalent among transfusion- dependent β-thalassemia major patients which necessitate regular screening with early markers of glomerular and tubular dysfunction. Further studies in order to investigate the correlation between renal hemosiderosis and early markers of kidney dysfunction among these patients are recommended.
Weatherall DJ. The inherited diseases of hemoglobin are an emerging global health burden. Blood. 2010; 115 (22):4331-6.
Cunningham MJ, Sankaran VG, Nathan DG, et al. The thalassemia syndromes. In: Orkin SH, Nathan DG, Ginsburg D, Fisher DE, Look AT, Lux SE, eds. Nathan and Oski’s Hematology of Infancy and Childhood. 7th ed. Philadelphia: Saunders; 2009: 1015-76.
Bakr A, Al-Tonbary Y, Osman G, et al. Renal complications of beta-thalassemia major in children. Am J Blood Res. 2014; 4(1):1-6.
Azarkeivan A, Hashemieh M, Akhlaghpoor S, et al. Relation between serum ferritin and liver and heart MRI T2* in beta thalassaemia major patients. East Mediterr Health J. 2013; 19(8):727-32.
Hashemieh M, Azarkeivan A, Akhlaghpoor S, et al. T2-star (T2*) magnetic resonance imaging for assessment of kidney iron overload in thalassemic patients. Arch Iran Med. 2012; 15(2):91-4.
Azarkeivan A, Hashemieh M, Shirkavand A, et al. Correlation between Heart, Liver and Pancreas Hemosiderosis Measured by MRI T2* among Thalassemia Major Patients from Iran. Arch Iran Med. 2016; 19(2):96-100.
Tantawy AA, El Bablawy N, Adly AA, et al. Early Predictors of Renal Dysfunction in Egyptian Patients with β-Thalassemia Major and Intermedia .Mediterr J Hematol Infect Dis. 2014; 6(1):e2014057.
Musallam KM, Taher AT. Mechanisms of renal disease in β-thalassemia. J Am Soc Nephrol. 2012; 23(8):1299-302.
Ponticelli C, Musallam KM, Cianciulli P, et al. Renal complications in transfusion-dependent beta thalassaemia. Blood Rev. 2010; 24(6):239-44.
Quinn CT, Johnson VL, Kim HY, et al. Renal dysfunction in patients with thalassaemia. Br J Haematol. 2011; 153(1):111-7
Ali BA, Mahmoud AM. Frequency of glomerular dysfunction in children with Beta thalassaemia major. Sultan Qaboos Univ Med J. 2014; 14(1):e88-94.
Papassotiriou I, Margeli A, Hantzi E, et al .Cystatin C levels in patients with beta-thalassemia during deferasirox treatment. Blood Cells Mol Dis. 2010; 44(3):152-5.
Voskaridou E, Terpos E, Michail S, et al. Early markers of renal dysfunction in patients with sickle cell/beta-thalassemia. Kidney Int. 2006; 69(11):2037-42.
Nagababu E, Gulyani S, Earley CJ, et al. Iron-deficiency anaemia enhances red blood cell oxidative stress. Free Radic Res. 2008; 42(9):824-9.
Zhou XJ, Laszik Z, Wang XQ, et al. Association of renal injury with increased oxygen free radical activity and altered nitric oxide metabolism in chronic experimental hemosiderosis. Lab Invest. 2000; 80(12):1905-14.
Weatherall DJ, Clegg JB, eds. The Thalassemia Syndromes. 4th ed. Oxford: England: Blackwell Science, 2001:133-91
Schein A, Enriquez C, Coates TD, et al. Magnetic resonance detection of kidney iron deposition in sickle cell disease: a marker of chronic hemolysis. J Magn Reson Imaging. 2008; 28(3):698-704.
Koliakos G, Papachristou F, Koussi A, et al. Urine biochemical markers of early renal dysfunction are associated with iron overload in beta-thalassemia. Clin Lab Haematol. 2003; 25(2):105-109.
Naderi M, Sadeghi-Bojd S, Valeshabad AK, et al. A prospective study of tubular dysfunction in pediatric patients with Beta thalassemia major receiving deferasirox. Pediatr Hematol Oncol. 2013; 30(8):748-54.
Stevens LA, Levey. AS: Measurement of kidney function. Med Clin North Am. 2005, 89(3):457-73.
Mohkam M, Shamsian BS, Gharib A, et al. Early markers of renal dysfunction in patients with beta-thalassemia major. Pediatr Nephrol. 2008; 23(6):971-6.
Jalali A, Khalilian H, Ahmadzadeh A, et al. Renal function in transfusion-dependent pediatric beta-thalassemia major patients. Hematology. 2011; 16(4):249-54.
Milo G, Feige Gross Nevo R, Pazgal I, et al. GFR in Patients with β-Thalassemia Major. Clin J Am Soc Nephrol. 2015; 10(8):1350-6.
Mallat NS, Mallat SG, Musallam KM, et al. Potential mechanisms for renal damage in beta-thalassemia. J Nephrol. 2013; 26(5):821-8.
Hamed EA, ElMelegy NT. Renal functions in pediatric patients with beta-thalassemia major: relation to chelation therapy: original prospective study. Ital J Pediatr. 2010; 36: 39.
Shlipak MG, Matsushita K, Ärnlöv J, et al. Cystatin C versus creatinine in determining risk based on kidney function. N Engl J Med. 20135; 369(10):932-43.
Economou M, Printza N, Teli A, et al. Renal dysfunction in patients with beta-thalassemia major receiving iron chelation therapy either with deferoxamine and deferiprone or with deferasirox. Acta Haematol. 2010; 123(3):148-52.
Skalova S, Chladek J. Urinary N-acetyl-beta-D-glucosaminidase activity in healthy children. Nephrology (Carlton). 2004; 9(1):19-21.
Şen V, Ece A, Uluca Ü, et al. Urinary early kidney injury molecules in children with beta-thalassemia major. Ren Fail. 2015; 37(4):607-13.
Ahmadzadeh A, Jalali A, Assar S, et al. Renal tubular dysfunction in pediatric patients with beta-thalassemia major. Saudi J Kidney Dis Transpl. 2011; 22(3):497-500.
Sadeghi-Bojd S, Hashemi M, Karimi M. Renal tubular function in patients with beta-thalassaemia major in Zahedan, southeast Iran. Singapore Med J. 2008; 49(5):410-2.
|Issue||Vol 11, No 2 (2017)|
|β-Thalassemia major Renal involvement Transfusion MRI T2* Iran|
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