Abstract
Advances in hematopoietic stem cell transplantation (HSCT) for beta-thalassemia major make the long-term outcome of these patients very important. Few data on long-term renal function of thalassemia patients are available. We evaluated the renal function in children after successful allogeneic HSCT for beta-thalassemia. Twenty-nine patients were included; the mean age at HSCT was 4.9 years. Mean follow-up time was 7.6 years. After HSCT, two patients developed acute renal failure and two had graft versus host disease. At last follow up, height standard deviation score (SDS) remained the same, but weight SDS had improved. Mean hemoglobin was 12.5 g/dl, and serum ferritin level was 545 ng/ml. All children had normal estimated glomerular filtration rate (GFR). One patient had hypertension and proteinuria, 10 years after HSCT. When comparing 39 children of the same age with beta-thalassemia of similar disease severity but who had not experienced HSCT, we found that the parameters of renal tubule function were better in patients that had undergone HSCT, as demonstrated by urine protein level (0.36 mg/mg creatinine vs 3.03 mg/mg creatinine, P < 0.001), osmolality (712 mosmol/kg vs 573 mosmol/kg, P = 0.006), N-acetyl-beta-D-glucosaminidase (17.7 U/g creatinine vs 42.9 U/g creatinine, P = 0.045), and beta 2 microglobulin (0.09 μg/mg creatinine vs 0.13 μg/mg creatinine, P = 0.029). This study showed a low incidence of long-term renal impairment after HSCT and indicated that renal tubule function may be better in beta-thalassemia patients after HSCT.
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References
Wasi P (1983) Hemoglobinopathies in Southeast Asia. In: Bowman JF (ed) Distribution and evolution of hemoglobin and globin loci. Elsevier, New York, pp 179–208
Fuchareon S, Winichagoon P (1997) Hemoglobinopathies in Southeast Asia: molecular biology and clinical medicine. Hemoglobin 21:299–319
Modell B, Khan M, Darlison M (2000) Survival in beta-thalassaemia major in the UK: data from the UK Thalassemia Register. Lancet 355:2051–2052
Lawson SE, Roberts IA, Amrolia P, Dokal I, Szydlo R, Darbyshire PJ (2003) Bone marrow transplantation for beta-thalassaemia major: the UK experience in two paediatric centres. Br J Haematol 120:289–295
Lo L, Singer ST (2002) Thalassemia: current approach to an old disease. Pediatr Clin North Am 49:1165–1191
Lucarelli G, Andreani M, Angelucci E (2002) The cure of thalassemia by bone marrow transplantation. Blood Rev 16:81–85
Kist-van Holthe JE, Goedvolk CA, Brand R, van Weel MH, Bredius RG, van Oostayen JA, Vossen JM, van der Heijden BJ (2002) Prospective study of renal insufficiency after bone marrow transplantation. Pediatr Nephrol 17:1032–1037
Kist-van Holthe JE, Bresters D, Ahmed-Ousenkova YM, Goedvolk CA, Abbink FC, Wolterbeek R, Bredius RG, Pauwels EK, van der Heijden AJ (2005) Long-term renal function after hemopoietic stem cell transplantation in children. Bone Marrow Transplant 36:605–610
Patzer L, Kentouche K, Ringelmann F, Misselwitz J (2003) Renal function following hematological stem cell transplantation in childhood. Pediatr Nephrol 18:623–625
Hingorani S, Guthrie KA, Schoch G, Weiss NS, McDonald GB (2007) Chronic kidney disease in long-term survivors of hematopoietic cell transplant. Bone Marrow Transplant 39:223–229
Grönroos MH, Bolme P, Winiarski J, Berg UB (2007) Long-term renal function following bone marrow transplantation. Bone Marrow Transplant 39:717–723
Sumboonnanonda A, Malasit P, Tanphaichitr VS, Ong-ajyooth S, Sunthornchart S, Pattanakitsakul S, Petrarat S, Assateerawatt A, Vongjirad A (1998) Renal tubular function in beta-thalassemia. Pediatr Nephrol 12:280–283
Ong-ajyooth L, Malasit P, Ong-ajyooth S, Fucharoen S, Pootrakul P, Vasuvattakul S, Siritanaratkul N, Nilwarangkur S (1998) Renal function in adult beta-thalassemia/Hb E disease. Nephron 78:156–161
Aldudak B, Karabay Bayazit A, Noyan A, Ozel A, Anarat A, Sasmaz I, Kilinc Y, Gali E, Anarat R, Dikmen N (2000) Renal function in pediatric patients with beta-thalassemia major. Pediatr Nephrol 15:109–112
Koliakos G, Papachristou F, Koussi A, Perifanis V, Tsatra I, Souliou E, Athanasiou M (2003) Urine biochemical markers of early renal dysfunction are associated with iron overload in beta-thalassemia. Clin Lab Haematol 25:105–109
Tanner JM, Whitehouse RH, Cameron N, Marshall WA, Healy MJ, Goldstein H (1990) Assessment of skeletal maturity and prediction of adult height (TW2 method), 2nd edn. Alden, Oxford
Schwartz GJ, Haycock GB, Edelmann CM Jr, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 58:259–263
Michelakakis H, Dimitriou E, Georgakis H, Karabatsos F, Fragodimitri C, Saraphidou J, Premetis E, Karagiorga-Lagana M (1997) Iron overload and urinary lysosomal enzyme levels in beta-thalassaemia major. Eur J Pediatr 156:602–604
Leblond V, Sutton L, Jacguiaud C, Item C, Sadoun R, Jaudon MC, Raymond F, Jacobs C, Deray G (1995) Evaluation of renal function in 60 long-term survivors of bone marrow transplantation. J Am Soc Nephrol 6:1661–1665
Van Why SK, Friedman AL, Wei LJ, Hong R (1991) Renal insufficiency after bone marrow transplantation in children. Bone Marrow Transplant 7:383–388
Miralbell R, Bieri S, Mermillod B, Helg C, Sancho G, Pastoors B, Keller A, Kurtz JM, Chapuis B (1996) Renal toxicity after allogeneic bone marrow transplantation: the combined effects of total-body irradiation and graft-versus-host disease. J Clin Oncol 14:579–585
Patzer L, Hempel L, Ringelmann F, Misselwitz J, Fuchs D, Zintl F, Brandis M, deBroe ME, Zimmerhackl LB (1997) Renal function after conditioning therapy for bone marrow transplantation in childhood. Med Pediatr Oncol 28:274–283
Patzer L, Ringelmann F, Kentouche K, Fuchs D, Zintl F, Brandis M, Zimmerhackl LB, Misselwitz J (2001) Renal function in long-term survivors of stem cell transplantation in childhood. A prospective trial. Bone Marrow Transplant 27:319–327
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This study was funded by the Siriraj Grant for Research Development and Medical Education, Faculty of Medicine Siriraj Hospital, Thailand (grant no.004 (III)/50.
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Sumboonnanonda, A., Sanpakit, K. & Piyaphanee, N. Renal tubule function in beta-thalassemia after hematopoietic stem cell transplantation. Pediatr Nephrol 24, 183–187 (2009). https://doi.org/10.1007/s00467-008-0949-0
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DOI: https://doi.org/10.1007/s00467-008-0949-0