dc.creator | De Sonati M.F. | |
dc.creator | Costa F.F. | |
dc.date | 2008 | |
dc.date | 2015-06-30T19:35:59Z | |
dc.date | 2015-11-26T14:46:02Z | |
dc.date | 2015-06-30T19:35:59Z | |
dc.date | 2015-11-26T14:46:02Z | |
dc.date.accessioned | 2018-03-28T21:55:29Z | |
dc.date.available | 2018-03-28T21:55:29Z | |
dc.identifier | | |
dc.identifier | Jornal De Pediatria. , v. 84, n. 4 SUPPL., p. S40 - S51, 2008. | |
dc.identifier | 217557 | |
dc.identifier | 10.2223/JPED.1802 | |
dc.identifier | http://www.scopus.com/inward/record.url?eid=2-s2.0-57349187681&partnerID=40&md5=b09dd9333bbb208c9322e24d259a7ecf | |
dc.identifier | http://www.repositorio.unicamp.br/handle/REPOSIP/106804 | |
dc.identifier | http://repositorio.unicamp.br/jspui/handle/REPOSIP/106804 | |
dc.identifier | 2-s2.0-57349187681 | |
dc.identifier.uri | http://repositorioslatinoamericanos.uchile.cl/handle/2250/1252676 | |
dc.description | Objective: 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.description | 84 | |
dc.description | 4 SUPPL. | |
dc.description | S40 | |
dc.description | S51 | |
dc.description | Steiner, L.A., Gallagher, P.G., Erythrocyte disorders in the perinatal period (2007) Semin Perinatol, 31, pp. 254-261 | |
dc.description | Tolentino, 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.description | Dhaliwal, G., Cornett, P.A., Tierney Jr., L.M., Hemolytic anemia (2004) Am Fam Physician, 69, pp. 2599-2606 | |
dc.description | Madigan, C., Malik, P., Pathophysiology and therapy for haemoglobinopathies. Part I: Sickle cell disease (2006) Expert Rev Mol Med, 8, pp. 1-23 | |
dc.description | Urbinati, F., Madigan, C., Malik, P., Pathophysiology and therapy for haemoglobinopathies. Part II: Thalassaemias (2006) Expert Rev Mol Med, 8, pp. 1-26 | |
dc.description | Old, J.M., Screening and genetic diagnosis of haemoglobinopathies (2007) Scand J Clin Lab Invest, 67, pp. 71-86 | |
dc.description | Khattab, 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.description | Theodorsson, 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.description | Morris, 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.description | Costa, 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.description | Wenning, M.R., Sonati, M.F., Hemoglobinopatias hereditárias (2007) Diagnóstico e tratamento, pp. 310-314. , Lopes AC, editor, São Paulo: Manole; | |
dc.description | Vekilov, 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.description | Tefferi, A., Clinical, genetic, and therapeutic insights into systemic mast cell disease (2004) Curr Opin Hematol, 11, pp. 58-64 | |
dc.description | Catlin, A.J., Thalassemia: The facts and the controversies (2003) Pediatr Nurs, 29, pp. 447-449 | |
dc.description | Thein, S.L., Genetic insights into the clinical diversity of beta thalassaemia (2004) Br J Haematol, 124, pp. 264-274 | |
dc.description | Shah, A., Thalassemia syndromes (2004) Indian J Med Sci, 58, pp. 445-449 | |
dc.description | Williams, T.N., Human red blood cell polymorphisms and malaria (2006) Curr Opin Microbiol, 9, pp. 388-394 | |
dc.description | Williams, T.N., Red blood cell defects and malaria (2006) Mol Biochem Parasitol, 149, pp. 121-127 | |
dc.description | Frenette, P.S., Atweh, G.F., Sickle cell disease: Old discoveries, new concepts, and future promise (2007) J Clin Invest, 117, pp. 850-858 | |
dc.description | Di Nuzzo, D.V., Fonseca, S.F., Sickle cell disease and infection] (2004) J Pediatr (Rio J), 80, pp. 347-354 | |
dc.description | Lyra, 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.description | Lima, 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.description | Adams, R.J., Big strokes in small persons (2007) Arch Neurol, 64, pp. 1567-1574 | |
dc.description | Brittain, J.E., Parise, L.V., Cytokines and plasma factors in sickle cell disease (2007) Curr Opin Hematol, 14, pp. 438-443 | |
dc.description | Seidman, C., Kirkham, F., Pavlakis, S., Pediatric stroke: Current developments (2007) Curr Opin Pediatr, 19, pp. 657-662 | |
dc.description | Traina, 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.description | Wong, 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.description | Creary, 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.description | Castro, O., Hoque, M., Brown, B.D., Pulmonary hypertension in sickle cell disease: Cardiac catheterization results and survival (2003) Blood, 101, pp. 1257-1261 | |
dc.description | Ataga, 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.description | Machado, 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.description | Morris, 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.description | Kato, 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.description | Barnett, 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.description | Claster, S., Vichinsky, E.P., Managing sickle cell disease (2003) BMJ, 327, pp. 1151-1155 | |
dc.description | Berry, 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.description | Adorno, 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.description | Chiang, E.Y., Frenette, P.S., Sickle cell vaso-occlusion (2005) Hematol Oncol Clin North Am, 19, pp. 771-784 | |
dc.description | Joiner, 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.description | Lew, V.L., Bookchin, R.M., Ion transport pathology in the mechanism of sickle cell dehydration (2005) Physiol Rev, 85, pp. 179-200 | |
dc.description | Zen 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.description | Canalli, 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.description | Conran, 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.description | Canalli, 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.description | Conran, 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.description | Canalli, 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.description | Belcher, 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.description | Perelman, 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.description | Solovey, 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.description | Aslan, M., Freeman, B.A., Redox-dependent impairment of vascular function in sickle cell disease (2007) Free Radic Biol Med, 43, pp. 1469-1483 | |
dc.description | Conran, 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.description | Kaul, 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.description | Steinberg, M.H., Predicting clinical severity in sickle cell anaemia (2005) Br J Haematol, 129, pp. 465-481 | |
dc.description | Fathallah, 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.description | Adorno, 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.description | Hoppe, 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.description | Sebastiani, 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.description | Castro, 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.description | Steinberg, M.H., Adewoye, A.H., Modifier genes and sickle cell anemia (2006) Curr Opin Hematol, 13, pp. 131-136 | |
dc.description | Kutlar, F., Diagnostic approach to hemoglobinopathies (2007) Hemoglobin, 31, pp. 243-250 | |
dc.description | Colah, 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.description | Cremonesi, 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.description | Kutlar, A., Sickle cell disease: A multigenic perspective of a single gene disorder (2007) Hemoglobin, 31, pp. 209-224 | |
dc.description | Steinberg, 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.description | Davies, S.C., Gilmore, A., The role of hydroxyurea in the management of sickle cell disease (2003) Blood Rev, 17, pp. 99-109 | |
dc.description | Conran, 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.description | De Franceschi, L., Corrocher, R., Established and experimental treatments for sickle cell disease (2004) Haematologica, 89, pp. 348-356 | |
dc.description | Gambero, 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.description | Haynes 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.description | Johnson, C., Telen, M.J., Adhesion molecules and hydroxyurea in the pathophysiology of sickle cell disease (2008) Haematologica, 93, pp. 481-485 | |
dc.description | Moreira, 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.description | Fattori, 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.description | Steinberg, M.H., Brugnara, C., Pathophysiological-based approaches to treatment of sickle cell disease (2003) Annu Rev Med, 54, pp. 89-112 | |
dc.description | Locatelli, 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.description | Cheung, 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.description | Makis, A.C., Hatzimichael, E.C., Stebbing, J., The genomics of new drugs in sickle cell disease (2006) Pharmacogenomics, 7, pp. 909-917 | |
dc.description | Stocker, 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.description | Mack, 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.description | Machado, 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.description | Canalli, 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.description | Bank, A., On the road to gene therapy for beta-thalassemia and sickle cell anemia (2008) Pediatr Hematol Oncol, 25, pp. 1-4 | |
dc.description | Shen, 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.description | Chang, 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.description | Wu, 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.description | Birgens, H., Ljung, R., The thalassaemia syndromes (2007) Scand J Clin Lab Invest, 67, pp. 11-25 | |
dc.description | Araújo, 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.description | Taher, A., Isma'eel, H., Cappellini, M.D., Thalassemia intermedia: Revisited (2006) Blood Cells Mol Dis, 37, pp. 12-20 | |
dc.description | Rund, D., Rachmilewitz, E., Beta-thalassemia (2005) N Engl J Med, 353, pp. 1135-1146 | |
dc.description | Kuypers, 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.description | Datta, 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.description | De 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.description | Ayi, 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.description | Weiss, 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.description | Toumba, M., Sergis, A., Kanaris, C., Skordis, N., Endocrine complications in patients with Thalassaemia Major (2007) Pediatr Endocrinol Rev, 5, pp. 642-648 | |
dc.description | Aessopos, 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.description | Weizer-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.description | Tanno, 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.description | Cohen, A.R., Galanello, R., Pennell, D.J., Cunningham, M.J., Vichinsky, E., (2004) Thalassemia, pp. 14-34. , Hematology Am Soc Hematol Educ Program | |
dc.description | Ataga, 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.description | Panigrahi, I., Agarwal, S., Thromboembolic complications in beta-thalassemia: Beyond the horizon (2007) Thromb Res, 120, pp. 783-789 | |
dc.description | Amer, 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.description | Pattanapanyasat, 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.description | Weinstein, 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.description | Singh, 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.description | Kimura, 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.description | Bailey, 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.description | dos Santos, C.O., Costa, F.F., AHSP and beta-thalassemia: A possible genetic modifier (2005) Hematology, 10, pp. 157-161 | |
dc.description | dos 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.description | Franchini, M., Veneri, D., Iron-chelation therapy: An update (2004) Hematol J, 5, pp. 287-292 | |
dc.description | Pootrakul, 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.description | Tsironi, 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.description | Neufeld, 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.description | Choudhry, V.P., Naithani, R., Current status of iron overload and chelation with deferasirox (2007) Indian J Pediatr, 74, pp. 759-764 | |
dc.description | Yang, 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.description | Rivera, 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.description | Higgs, D.R., (2004) Gene regulation in hematopoiesis: New lessons from thalassemia, pp. 1-13. , Hematology Am Soc Hematol Educ Program | |
dc.description | Weatherall, D.J., Thalassaemia: The long road from bedside to genome (2004) Nat Rev Genet, 5, pp. 625-631 | |
dc.description | Quek, L., Thein, S.L., Molecular therapies in beta-thalassaemia (2007) Br J Haematol, 136, pp. 353-365 | |
dc.description | Dissayabutra, 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.description | Tesoriere, 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.description | Ghavamzadeh, 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.description | Sadelain, 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.description | Xie, 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.description | Moi, P., Sadelain, M., Towards the genetic treatment of beta-thalassemia: New disease models, new vectors, new cells (2008) Haematologica, 93, pp. 325-330 | |
dc.language | en | |
dc.publisher | | |
dc.relation | Jornal de Pediatria | |
dc.rights | aberto | |
dc.source | Scopus | |
dc.title | The Genetics Of Blood Disorders: Hereditary Hemoglobinopathies | |
dc.type | Artículos de revistas | |