dc.creatorDe Sonati M.F.
dc.creatorCosta F.F.
dc.date2008
dc.date2015-06-30T19:35:59Z
dc.date2015-11-26T14:46:02Z
dc.date2015-06-30T19:35:59Z
dc.date2015-11-26T14:46:02Z
dc.date.accessioned2018-03-28T21:55:29Z
dc.date.available2018-03-28T21:55:29Z
dc.identifier
dc.identifierJornal De Pediatria. , v. 84, n. 4 SUPPL., p. S40 - S51, 2008.
dc.identifier217557
dc.identifier10.2223/JPED.1802
dc.identifierhttp://www.scopus.com/inward/record.url?eid=2-s2.0-57349187681&partnerID=40&md5=b09dd9333bbb208c9322e24d259a7ecf
dc.identifierhttp://www.repositorio.unicamp.br/handle/REPOSIP/106804
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/106804
dc.identifier2-s2.0-57349187681
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1252676
dc.descriptionObjective: To summarize recently published data on the pathophysiology, diagnosis and treatment of sickle cell diseases and β-Thalassemias, the most relevant hereditary hemoglobinopathies in the global population. Sources: Searches were run on the MEDLINE and SCIELO databases, limited to the period from 2003 to May 2008, using the terms hereditary hemoglobinopathies, sickle cell diseases and β-thalassemia. Two books and two chapters were also included. Summary of the findings: More than 2,000 articles were identified; those providing the most important information and broadest views were selected. Conclusions: Morbidity and mortality rates from sickle cell diseases and β-thalassemia are still very high and represent an important challenge. Increased understanding of pathophysiological aspects has lead to significant improvements in treatment and prevention of these diseases. Copyright © 2008 by Sociedade Brasileira de Pediatria.
dc.description84
dc.description4 SUPPL.
dc.descriptionS40
dc.descriptionS51
dc.descriptionSteiner, L.A., Gallagher, P.G., Erythrocyte disorders in the perinatal period (2007) Semin Perinatol, 31, pp. 254-261
dc.descriptionTolentino, K., Friedman, J.F., An update on anemia in less developed countries (2007) Am J Trop Med Hyg, 77, pp. 44-51
dc.description(2004) Wintrobe's clinical hematology, , Wintrobe MM, Foerster J, editors, 11th ed. Philadelphia: Lippincott Williams & Wilkins;
dc.descriptionDhaliwal, G., Cornett, P.A., Tierney Jr., L.M., Hemolytic anemia (2004) Am Fam Physician, 69, pp. 2599-2606
dc.descriptionMadigan, C., Malik, P., Pathophysiology and therapy for haemoglobinopathies. Part I: Sickle cell disease (2006) Expert Rev Mol Med, 8, pp. 1-23
dc.descriptionUrbinati, F., Madigan, C., Malik, P., Pathophysiology and therapy for haemoglobinopathies. Part II: Thalassaemias (2006) Expert Rev Mol Med, 8, pp. 1-26
dc.descriptionOld, J.M., Screening and genetic diagnosis of haemoglobinopathies (2007) Scand J Clin Lab Invest, 67, pp. 71-86
dc.descriptionKhattab, A.D., Rawlings, B., Ali, I.S., Care of patients with haemoglobin abnormalities: History and biology (2006) Br J Nurs, 15, pp. 994-998
dc.descriptionTheodorsson, E., Birgens, H., Hagve, T.A., Haemoglobinopathies and glucose-6-phosphate dehydrogenase deficiency in a Scandinavian perspective (2007) Scand J Clin Lab Invest, 67, pp. 3-10
dc.descriptionMorris, C.R., Singer, S.T., Walters, M.C., Clinical hemoglobinopathies: Iron, lungs and new blood (2006) Curr Opin Hematol, 13, pp. 407-418
dc.description(2004) Hematologia: Fundamentos e prática, , Zago MA, Falcão RP, Pasquini R, editors, São Paulo: Atheneu;
dc.descriptionCosta, F.F., Sonati, M.F., Hemoglobina: Estrutura, síntese e transporte de oxigênio (2007) Hemoterapia: Fundamentos e prática, pp. 27-33. , Covas DT, Langhi Júnior DM, Bordin JO, editors, São Paulo: Atheneu;
dc.descriptionWenning, M.R., Sonati, M.F., Hemoglobinopatias hereditárias (2007) Diagnóstico e tratamento, pp. 310-314. , Lopes AC, editor, São Paulo: Manole;
dc.descriptionVekilov, P.G., Sickle-cell haemoglobin polymerization: Is it the primary pathogenic event of sickle-cell anaemia? (2007) Br J Haematol, 139, pp. 173-184
dc.descriptionTefferi, A., Clinical, genetic, and therapeutic insights into systemic mast cell disease (2004) Curr Opin Hematol, 11, pp. 58-64
dc.descriptionCatlin, A.J., Thalassemia: The facts and the controversies (2003) Pediatr Nurs, 29, pp. 447-449
dc.descriptionThein, S.L., Genetic insights into the clinical diversity of beta thalassaemia (2004) Br J Haematol, 124, pp. 264-274
dc.descriptionShah, A., Thalassemia syndromes (2004) Indian J Med Sci, 58, pp. 445-449
dc.descriptionWilliams, T.N., Human red blood cell polymorphisms and malaria (2006) Curr Opin Microbiol, 9, pp. 388-394
dc.descriptionWilliams, T.N., Red blood cell defects and malaria (2006) Mol Biochem Parasitol, 149, pp. 121-127
dc.descriptionFrenette, P.S., Atweh, G.F., Sickle cell disease: Old discoveries, new concepts, and future promise (2007) J Clin Invest, 117, pp. 850-858
dc.descriptionDi Nuzzo, D.V., Fonseca, S.F., Sickle cell disease and infection] (2004) J Pediatr (Rio J), 80, pp. 347-354
dc.descriptionLyra, I.M., Gonçalves, M.S., Braga, J.A., MdeF, G., Carvalho, M.H., Saad, S.T., Clinical, hematological, and molecular characterization of sickle cell anemia pediatric patients from two different cities in Brazil (2005) Cad Saude Publica, 21, pp. 1287-1290
dc.descriptionLima, C.S., Rocha, E.M., Silva, N.M., Sonatti, M.F., Costa, F.F., Saad, S.T., Risk factors for conjunctival and retinal vessel alterations in sickle cell disease (2006) Acta Ophthalmol Scand, 84, pp. 234-241
dc.descriptionAdams, R.J., Big strokes in small persons (2007) Arch Neurol, 64, pp. 1567-1574
dc.descriptionBrittain, J.E., Parise, L.V., Cytokines and plasma factors in sickle cell disease (2007) Curr Opin Hematol, 14, pp. 438-443
dc.descriptionSeidman, C., Kirkham, F., Pavlakis, S., Pediatric stroke: Current developments (2007) Curr Opin Pediatr, 19, pp. 657-662
dc.descriptionTraina, F., Jorge, S.G., Yamanaka, A., de Meirelles, L.R., Costa, F.F., Saad, S.T., Chronic liver abnormalities in sickle cell disease: A clinicopathological study in 70 living patients (2007) Acta Haematol, 118, pp. 129-135
dc.descriptionWong, W.Y., Powars, D.R., Overt and incomplete (silent) cerebral infarction in sickle cell anemia: Diagnosis and management (2007) Neuroimaging Clin N Am, 17, pp. 269-280
dc.descriptionCreary, M., Williamson, D., Kulkarni, R., Sickle cell disease: Current activities, public health implications, and future directions (2007) J Womens Health (Larchmt), 16, pp. 575-582
dc.descriptionCastro, O., Hoque, M., Brown, B.D., Pulmonary hypertension in sickle cell disease: Cardiac catheterization results and survival (2003) Blood, 101, pp. 1257-1261
dc.descriptionAtaga, K.I., Sood, N., De Gent, G., Kelly, E., Henderson, A.G., Jones, S., Pulmonary hypertension in sickle cell disease (2004) Am J Med, 117, pp. 665-669
dc.descriptionMachado, R.F., Gladwin, M.T., Chronic sickle cell lung disease: New insights into the diagnosis, pathogenesis and treatment of pulmonary hypertension (2005) Br J Haematol, 129, pp. 449-464
dc.descriptionMorris, C.R., Kato, G.J., Poljakovic, M., Wang, X., Blackwelder, W.C., Sachdev, V., Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease (2005) JAMA, 294, pp. 81-90
dc.descriptionKato, G.J., Onyekwere, O.C., Gladwin, M.T., Pulmonary hypertension in sickle cell disease: Relevance to children (2007) Pediatr Hematol Oncol, 24, pp. 159-170
dc.descriptionBarnett, C.F., Hsue, P.Y., Machado, R.F., Pulmonary hypertension: An increasingly recognized complication of hereditary hemolytic anemias and HIV infection (2008) JAMA, 299, pp. 324-331
dc.descriptionClaster, S., Vichinsky, E.P., Managing sickle cell disease (2003) BMJ, 327, pp. 1151-1155
dc.descriptionBerry, P.A., Cross, T.J., Thein, S.L., Portmann, B.C., Wendon, J.A., Karani, J.B., Hepatic dysfunction in sickle cell disease: A new system of classification based on global assessment (2007) Clin Gastroenterol Hepatol, 5, pp. 1469-1476
dc.descriptionAdorno, E.V., Couto, F.D., Moura Neto, J.P., Menezes, J.F., Rêgo, M., Reis, M.G., Gonçalves, M.S., Hemoglobinopathies in newborns from Salvador, Bahia, Northeast Brazil (2005) Cad Saude Publica, 21, pp. 292-298
dc.descriptionChiang, E.Y., Frenette, P.S., Sickle cell vaso-occlusion (2005) Hematol Oncol Clin North Am, 19, pp. 771-784
dc.descriptionJoiner, C.H., Rettig, R.K., Jiang, M., Franco, R.S., KCl cotransport mediates abnormal sulfhydryl-dependent volume regulation in sickle reticulocytes (2004) Blood, 104, pp. 2954-2960
dc.descriptionLew, V.L., Bookchin, R.M., Ion transport pathology in the mechanism of sickle cell dehydration (2005) Physiol Rev, 85, pp. 179-200
dc.descriptionZen Q, Batchvarova M, Twyman CA, Eyler CE, Qiu H, De Castro LM, Telen MJ. B-CAM/LU expression and the role of B-CAM/LU activation in binding of low- and high-density red cells to laminin in sickle cell disease. Am J Hematol. 2004;75:63-7Assis, A., Conran, N., Canalli, A.A., Lorand-Metze, I., Saad, S.T., Costa, F.F., Effect of cytokines and chemokines on sickle neutrophil adhesion to fibronectin (2005) Acta Haematol, 113, pp. 130-136
dc.descriptionCanalli, A.A., Costa, F.F., Saad, S.T., Conran, N., Granulocytic adhesive interactions and their role in sickle cell vaso-occlusion (2005) Hematology, 10, pp. 419-425
dc.descriptionConran, N., Saad, S.T., Costa, F.F., Ikuta, T., Leukocyte numbers correlate with plasma levels of granulocyte-macrophage colony-stimulating factor in sickle cell disease (2007) Ann Hematol, 86, pp. 255-261
dc.descriptionCanalli, A.A., Franco-Penteado, C.F., Traina, F., Saad, S.T., Costa, F.F., Conran, N., Role for cAMP-protein kinase A signalling in augmented neutrophil adhesion and chemotaxis in sickle cell disease (2007) Eur J Haematol, 79, pp. 330-337
dc.descriptionConran, N., Almeida, C.B., Lanaro, C., Ferreira, R.P., Traina, F., Saad, S.T., Inhibition of caspase-dependent spontaneous apoptosis via a cAMP-protein kinase A dependent pathway in neutrophils from sickle cell disease patients (2007) Br J Haematol, 139, pp. 148-158
dc.descriptionCanalli, A.A., Conran, N., Fattori, A., Saad, S.T., Costa, F.F., Increased adhesive properties of eosinophils in sickle cell disease (2004) Exp Hematol, 32, pp. 728-734
dc.descriptionBelcher, J.D., Bryant, C.J., Nguyen, J., Bowlin, P.R., Kielbik, M.C., Bischof, J.C., Transgenic sickle mice have vascular inflammation (2003) Blood, 101, pp. 3953-3959
dc.descriptionPerelman, N., Selvaraj, S.K., Batra, S., Luck, L.R., Erdreich-Epstein, A., Coates, T.D., Placenta growth factor activates monocytes and correlates with sickle cell disease severity (2003) Blood, 102, pp. 1506-1514
dc.descriptionSolovey, A., Kollander, R., Shet, A., Milbauer, L.C., Choong, S., Panoskaltsis-Mortari, A., Endothelial cell expression of tissue factor in sickle mice is augmented by hypoxia/reoxygenation and inhibited by lovastatin (2004) Blood, 104, pp. 840-846
dc.descriptionAslan, M., Freeman, B.A., Redox-dependent impairment of vascular function in sickle cell disease (2007) Free Radic Biol Med, 43, pp. 1469-1483
dc.descriptionConran, N., Gambero, A., Ferreira, H.H., Antunes, E., de Nucci, G., Nitric oxide has a role in regulating VLA-4-integrin expression on the human neutrophil cell surface (2003) Biochem Pharmacol, 66, pp. 43-50
dc.descriptionKaul, D.K., Liu, X.D., Chang, H.Y., Nagel, R.L., Fabry, M.E., Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice (2004) J Clin Invest, 114, pp. 1136-1145
dc.descriptionSteinberg, M.H., Predicting clinical severity in sickle cell anaemia (2005) Br J Haematol, 129, pp. 465-481
dc.descriptionFathallah, H., Atweh, G.F., (2006) Induction of fetal hemoglobin in the treatment of sickle cell disease, pp. 58-62. , Hematology Am Soc Hematol Educ Program
dc.descriptionAdorno, E.V., Zanette, A., Lyra, I., Souza, C.C., Santos, L.F., Menezes, J.F., The beta-globin gene cluster haplotypes in sickle cell anemia patients from Northeast Brazil: A clinical and molecular view (2004) Hemoglobin, 28, pp. 267-271
dc.descriptionHoppe, C., Klitz, W., Noble, J., Vigil, L., Vichinsky, E., Styles, L., Distinct HLA associations by stroke subtype in children with sickle cell anemia (2003) Blood, 101, pp. 2865-2869
dc.descriptionSebastiani, P., Ramoni, M.F., Nolan, V., Baldwin, C.T., Steinberg, M.H., Genetic dissection and prognostic modeling of overt stroke in sickle cell anemia (2005) Nat Genet, 37, pp. 435-440
dc.descriptionCastro, V., Alberto, F.L., Costa, R.N., Lepikson-Neto, J., Gualandro, S.F., Figueiredo, M.S., Polymorphism of the human platelet antigen-5 system is a risk factor for occlusive vascular complications in patients with sickle cell anemia (2004) Vox Sang, 87, pp. 118-123
dc.descriptionSteinberg, M.H., Adewoye, A.H., Modifier genes and sickle cell anemia (2006) Curr Opin Hematol, 13, pp. 131-136
dc.descriptionKutlar, F., Diagnostic approach to hemoglobinopathies (2007) Hemoglobin, 31, pp. 243-250
dc.descriptionColah, R.B., Surve, R., Sawant, P., D'Souza, E., Italia, K., Phanasgaonkar, S., HPLC studies in hemoglobinopathies (2007) Indian J Pediatr, 74, pp. 657-662
dc.descriptionCremonesi, L., Ferrari, M., Giordano, P.C., Harteveld, C.L., Kleanthous, M., Papasavva, T., An overview of current microarray-based human globin gene mutation detection methods (2007) Hemoglobin, 31, pp. 289-311
dc.descriptionKutlar, A., Sickle cell disease: A multigenic perspective of a single gene disorder (2007) Hemoglobin, 31, pp. 209-224
dc.descriptionSteinberg, M.H., Barton, F., Castro, O., Pegelow, C.H., Ballas, S.K., Kutlar, A., Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: Risks and benefits up to 9 years of treatment (2003) JAMA, 289, pp. 1645-1651
dc.descriptionDavies, S.C., Gilmore, A., The role of hydroxyurea in the management of sickle cell disease (2003) Blood Rev, 17, pp. 99-109
dc.descriptionConran, N., Fattori, A., Saad, S.T., Costa, F.F., Increased levels of soluble ICAM-1 in the plasma of sickle cell patients are reversed by hydroxyurea (2004) Am J Hematol, 76, pp. 343-347
dc.descriptionDe Franceschi, L., Corrocher, R., Established and experimental treatments for sickle cell disease (2004) Haematologica, 89, pp. 348-356
dc.descriptionGambero, S., Canalli, A.A., Traina, F., Albuquerque, D.M., Saad, S.T., Costa, F.F., Conran, N., Therapy with hydroxyurea is associated with reduced adhesion molecule gene and protein expression in sickle red cells with a concomitant reduction in adhesive properties (2007) Eur J Haematol, 78, pp. 144-151
dc.descriptionHaynes Jr, J., Obiako, B., Hester, R.B., Baliga, B.S., Stevens, T., Hydroxyurea attenuates activated neutrophil-mediated sickle erythrocyte membrane phosphatidylserine exposure and adhesion to pulmonary vascular endothelium (2008) Am J Physiol Heart Circ Physiol, 294, pp. H379-H385
dc.descriptionJohnson, C., Telen, M.J., Adhesion molecules and hydroxyurea in the pathophysiology of sickle cell disease (2008) Haematologica, 93, pp. 481-485
dc.descriptionMoreira, L.S., de Andrade, T.G., Albuquerque, D.M., Cunha, A.F., Fattori, A., Saad, S.T., Costa, F.F., Identification of differentially expressed genes induced by hydroxyurea in reticulocytes from sickle cell anaemia patients (2008) Clin Exp Pharmacol Physiol, 35, pp. 651-655
dc.descriptionFattori, A., de Souza, R.A., Saad, S.T., Costa, F.F., Acute myocardial infarction in sickle cell disease: A possible complication of hydroxyurea treatment (2005) Hematol J, 5, pp. 589-590
dc.descriptionSteinberg, M.H., Brugnara, C., Pathophysiological-based approaches to treatment of sickle cell disease (2003) Annu Rev Med, 54, pp. 89-112
dc.descriptionLocatelli, F., Rocha, V., Reed, W., Bernaudin, F., Ertem, M., Grafakos, S., Related umbilical cord blood transplantation in patients with thalassemia and sickle cell disease (2003) Blood, 101, pp. 2137-2143
dc.descriptionCheung, A.T., Chan, M.S., Ramanujam, S., Rangaswami, A., Curl, K., Franklin, P., Effects of poloxamer 188 treatment on sickle cell vaso-occlusive crisis: Computer-assisted intravital microscopy study (2004) J Investig Med, 52, pp. 402-406
dc.descriptionMakis, A.C., Hatzimichael, E.C., Stebbing, J., The genomics of new drugs in sickle cell disease (2006) Pharmacogenomics, 7, pp. 909-917
dc.descriptionStocker, J.W., De Franceschi, L., McNaughton-Smith, G.A., Corrocher, R., Beuzard, Y., Brugnara, C., ICA-17043, a novel Gardos channel blocker, prevents sickled red blood cell dehydration in vitro and in vivo in SAD mice (2003) Blood, 101, pp. 2412-2418
dc.descriptionMack, A.K., Kato, G.J., Sickle cell disease and nitric oxide: A paradigm shift? (2006) Int J Biochem Cell Biol, 38, pp. 1237-1243
dc.descriptionMachado, R.F., Martyr, S., Kato, G.J., Barst, R.J., Anthi, A., Robinson, M.R., Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension (2005) Br J Haematol, 130, pp. 445-453
dc.descriptionCanalli, A.A., Franco-Penteado, C.F., Saad, S.T., Conran, N., Costa, F.F., Increased adhesive properties of neutrophils in sickle cell disease may be reversed by pharmacological nitric oxide donation (2008) Haematologica, 93, pp. 605-609
dc.descriptionBank, A., On the road to gene therapy for beta-thalassemia and sickle cell anemia (2008) Pediatr Hematol Oncol, 25, pp. 1-4
dc.descriptionShen, T.J., Rogers, H., Yu, X., Lin, F., Noguchi, C.T., Ho, C., Modification of globin gene expression by RNA targeting strategies (2007) Exp Hematol, 35, pp. 1209-1218
dc.descriptionChang, J.C., Ye, L., Kan, Y.W., Correction of the sickle cell mutation in embryonic stem cells (2006) Proc Natl Acad Sci U S A, 103, pp. 1036-1040
dc.descriptionWu, L.C., Sun, C.W., Ryan, T.M., Pawlik, K.M., Ren, J., Townes, T.M., Correction of sickle cell disease by homologous recombination in embryonic stem cells (2006) Blood, 108, pp. 1183-1188
dc.descriptionBirgens, H., Ljung, R., The thalassaemia syndromes (2007) Scand J Clin Lab Invest, 67, pp. 11-25
dc.descriptionAraújo, A.S., Silva, W.A., Leão, S.A., Bandeira, F.C., Petrou, M., Modell, B., A different molecular pattern of beta-thalassemia mutations in northeast (2003) Brazil. Hemoglobin, 27, pp. 211-217
dc.descriptionTaher, A., Isma'eel, H., Cappellini, M.D., Thalassemia intermedia: Revisited (2006) Blood Cells Mol Dis, 37, pp. 12-20
dc.descriptionRund, D., Rachmilewitz, E., Beta-thalassemia (2005) N Engl J Med, 353, pp. 1135-1146
dc.descriptionKuypers, F.A., de Jong, K., The role of phosphatidylserine in recognition and removal of erythrocytes (2004) Cell Mol Biol (Noisy-le-grand), 50, pp. 147-158
dc.descriptionDatta, P., Basu, S., Chakravarty, S.B., Chakravarty, A., Banerjee, D., Chandra, S., Enhanced oxidative cross-linking of hemoglobin E with spectrin and loss of erythrocyte membrane asymmetry in hemoglobin E beta-thalassemia (2006) Blood Cells Mol Dis, 37, pp. 77-81
dc.descriptionDe Franceschi, L., Ronzoni, L., Cappellini, M.D., Cimmino, F., Siciliano, A., Alper, S.L., K-CL co-transport plays an important role in normal and beta thalassemic erythropoiesis (2007) Haematologica, 92, pp. 1319-1326
dc.descriptionAyi, K., Turrini, F., Piga, A., Arese, P., Enhanced phagocytosis of ring-parasitized mutant erythrocytes: A common mechanism that may explain protection against falciparum malaria in sickle trait and beta-thalassemia trait (2004) Blood, 104, pp. 3364-3371
dc.descriptionWeiss, M.J., Zhou, S., Feng, L., Gell, D.A., Mackay, J.P., Shi, Y., Role of alpha-hemoglobin-stabilizing protein in normal erythropoiesis and beta-thalassemia (2005) Ann N Y Acad Sci, 1054, pp. 103-117
dc.descriptionToumba, M., Sergis, A., Kanaris, C., Skordis, N., Endocrine complications in patients with Thalassaemia Major (2007) Pediatr Endocrinol Rev, 5, pp. 642-648
dc.descriptionAessopos, A., Berdoukas, V., Tsironi, M., The heart in transfusion dependent homozygous thalassaemia today - prediction, prevention and management (2008) Eur J Haematol, 80, pp. 93-106
dc.descriptionWeizer-Stern, O., Adamsky, K., Amariglio, N., Levin, C., Koren, A., Breuer, W., Downregulation of hepcidin and haemojuvelin expression in the hepatocyte cell-line HepG2 induced by thalassaemic sera (2006) Br J Haematol, 135, pp. 129-138
dc.descriptionTanno, T., Bhanu, N.V., Oneal, P.A., Goh, S.H., Staker, P., Lee, Y.T., High levels of GDF15 in thalassemia suppress expression of the iron regulatory protein hepcidin (2007) Nat Med, 13, pp. 1096-1101
dc.descriptionCohen, A.R., Galanello, R., Pennell, D.J., Cunningham, M.J., Vichinsky, E., (2004) Thalassemia, pp. 14-34. , Hematology Am Soc Hematol Educ Program
dc.descriptionAtaga, K.I., Cappellini, M.D., Rachmilewitz, E.A., Beta-thalassaemia and sickle cell anaemia as paradigms of hypercoagulability (2007) Br J Haematol, 139, pp. 3-13
dc.descriptionPanigrahi, I., Agarwal, S., Thromboembolic complications in beta-thalassemia: Beyond the horizon (2007) Thromb Res, 120, pp. 783-789
dc.descriptionAmer, J., Fibach, E., N-acetylcysteine amide, a novel cell-permeating thiol, restores cellular glutathione and protects human red blood cells from oxidative stress (2005) Free Radic Biol Med, 38, pp. 136-145
dc.descriptionPattanapanyasat, K., Gonwong, S., Chaichompoo, P., Noulsri, E., Lerdwana, S., Sukapirom, K., Activated platelet-derived microparticles in thalassaemia (2007) Br J Haematol, 136, pp. 462-471
dc.descriptionWeinstein, B.I., Erramouspe, B., Albuquerque, D.M., Oliveira, D.M., Kimura, E.M., Costa, F.F., Hb Florida: A novel elongated C-terminal beta-globin variant causing dominant beta-thalassemia phenotype (2006) Am J Hematol, 81, pp. 358-360
dc.descriptionSingh, S.P., Gupta, S., Molecular pathogenesis and clinical variability of homozygous beta 0-thalassemia in populations of Jammu region of J&K state (India) (2006) Hematology, 11, pp. 271-275
dc.descriptionKimura, E.M., Grignoli, C.R., Pinheiro, V.R., Costa, F.F., Sonati, M.F., Thalassemia intermedia as a result of heterozygosis for beta 0-thalassemia and alpha alpha alpha anti-3,7 genotype in a Brazilian patient (2003) Braz J Med Biol Res, 36, pp. 699-701
dc.descriptionBailey, L., Kuroyanagi, Y., Franco-Penteado, C.F., Conran, N., Costa, F.F., Ausenda, S., Expression of the gamma-globin gene is sustained by the cAMP-dependent pathway in beta-thalassaemia (2007) Br J Haematol, 138, pp. 382-395
dc.descriptiondos Santos, C.O., Costa, F.F., AHSP and beta-thalassemia: A possible genetic modifier (2005) Hematology, 10, pp. 157-161
dc.descriptiondos Santos, C.O., Zhou, S., Secolin, R., Wang, X., Cunha, A.F., Higgs, D.R., Population analysis of the alpha hemoglobin stabilizing protein (AHSP) gene identifies sequence variants that alter expression and function (2008) Am J Hematol, 83, pp. 103-108
dc.descriptionFranchini, M., Veneri, D., Iron-chelation therapy: An update (2004) Hematol J, 5, pp. 287-292
dc.descriptionPootrakul, P., Sirankapracha, P., Sankote, J., Kachintorn, U., Maungsub, W., Sriphen, K., Clinical trial of deferiprone iron chelation therapy in beta-thalassaemia/haemoglobin E patients in Thailand (2003) Br J Haematol, 122, pp. 305-310
dc.descriptionTsironi, M., Deftereos, S., Andriopoulos, P., Farmakis, D., Meletis, J., Aessopos, A., Reversal of heart failure in thalassemia major by combined chelation therapy: A case report (2005) Eur J Haematol, 74, pp. 84-85
dc.descriptionNeufeld, E.J., Oral chelators deferasirox and deferiprone for transfusional iron overload in thalassemia major: New data, new questions (2006) Blood, 107, pp. 3436-3441
dc.descriptionChoudhry, V.P., Naithani, R., Current status of iron overload and chelation with deferasirox (2007) Indian J Pediatr, 74, pp. 759-764
dc.descriptionYang, L.P., Keam, S.J., Keating, G.M., Deferasirox: A review of its use in the management of transfusional chronic iron overload (2007) Drugs, 67, pp. 2211-2230
dc.descriptionRivera, S., Nemeth, E., Gabayan, V., Lopez, M.A., Farshidi, D., Ganz, T., Synthetic hepcidin causes rapid dose-dependent hypoferremia and is concentrated in ferroportin-containing organs (2005) Blood, 106, pp. 2196-2199
dc.descriptionHiggs, D.R., (2004) Gene regulation in hematopoiesis: New lessons from thalassemia, pp. 1-13. , Hematology Am Soc Hematol Educ Program
dc.descriptionWeatherall, D.J., Thalassaemia: The long road from bedside to genome (2004) Nat Rev Genet, 5, pp. 625-631
dc.descriptionQuek, L., Thein, S.L., Molecular therapies in beta-thalassaemia (2007) Br J Haematol, 136, pp. 353-365
dc.descriptionDissayabutra, T., Tosukhowong, P., Seksan, P., The benefits of vitamin C and vitamin E in children with beta-thalassemia with high oxidative stress (2005) J Med Assoc Thai, 88 (SUPPL. 4), pp. S317-S321
dc.descriptionTesoriere, L., Allegra, M., Butera, D., Gentile, C., Livrea, M.A., Cytoprotective effects of the antioxidant phytochemical indicaxanthin in beta-thalassemia red blood cells (2006) Free Radic Res, 40, pp. 753-761
dc.descriptionGhavamzadeh, A., Iravani, M., Ashouri, A., Mousavi, S.A., Mahdavi, N., Shamshiri, A., Peripheral blood versus bone marrow as a source of hematopoietic stem cells for allogeneic transplantation in children with class I and II beta thalassemia major (2008) Biol Blood Marrow Transplant, 14, pp. 301-308
dc.descriptionSadelain, M., Recent advances in globin gene transfer for the treatment of beta-thalassemia and sickle cell anemia (2006) Curr Opin Hematol, 13, pp. 142-148
dc.descriptionXie, S.Y., Ren, Z.R., Zhang, J.Z., Guo, X.B., Wang, Q.X., Wang, S., Restoration of the balanced alpha/beta-globin gene expression in beta654-thalassemia mice using combined RNAi and antisense RNA approach (2007) Hum Mol Genet, 16, pp. 2616-2625
dc.descriptionMoi, P., Sadelain, M., Towards the genetic treatment of beta-thalassemia: New disease models, new vectors, new cells (2008) Haematologica, 93, pp. 325-330
dc.languageen
dc.publisher
dc.relationJornal de Pediatria
dc.rightsaberto
dc.sourceScopus
dc.titleThe Genetics Of Blood Disorders: Hereditary Hemoglobinopathies
dc.typeArtículos de revistas


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