Susana Rocha *

Autora invitada por SIIC

Estudo realizado com doentes não-esplenectomizados

SEVERIDADE CLÍNICA DA ESFEROCITOSE HEREDITÁRIA – MARCADORES ANALÍTICOS

Dos possíveis parâmetros analíticos complementares estudados (MCHC, RDW, Hb/MCHC, Hb/RDW, MCHC/RDW, EPO, sTfR e CH), apenas o RDW e as razões Hb/MCHC, Hb/RDW e MCHC/RDW mostraram ser excelentes marcadores de severidade, e, portanto, marcadores complementares para a classificação clínica da EH.

*Susana Rocha
describe para SIIC los aspectos relevantes de su trabajo
COMPLEMENTARY MARKERS FOR THE CLINICAL SEVERITY CLASSIFICATION OF HEREDITARY SPHEROCYTOSIS IN UNSPLENECTOMIZED PATIENTS
Institución principal de la investigación
*Universidade do Porto, Porto, Portugal
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Referencias bibliográficas
[1]Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ. Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol 2004; 126:455-474. [2]Delaunay J. The molecular basis of hereditary red cell membrane disorders. Blood Rev 2007; 21:1-20. [3]Perrotta S, Gallagher PG, Mohandas N. Hereditary spherocytosis. Lancet 2008; 372:1411-1426. [4]del Giudice EM, Perrotta S, Nobili B, Specchia C, d'Urzo G, Iolascon A. Coinheritance of Gilbert syndrome increases the risk for developing gallstones in patients with hereditary spherocytosis. Blood 1999; 94:2259-2262. [5]Garg PK, Kumar A, Teckchandani N, Hadke NS. Hereditary spherocytosis coexisting with Gilbert's syndrome: a diagnostic dilemma. Singapore Med J 2008; 49:e308-309. [6]Rocha S, Costa E, Ferreira F, et al. Hereditary spherocytosis and the (TA)nTAA polymorphism of UGT1A1 gene promoter region--a comparison of the bilirubin plasmatic levels in the different clinical forms. Blood Cells Mol Dis 2010; 44:117-119. [7]Shiota M, Asada J, Nishida H, et al. Hereditary spherocytosis in 3 children coexisting with UDP-glucuronyl transferase 1A1 deficiency. J Pediatr Hematol Oncol 2009; 31:121-123. [8]Sugita K, Maruo Y, Kurosawa H, et al. Severe hyperbilirubinemia in a 10-year-old girl with a combined disorder of hereditary spherocytosis and Gilbert syndrome. Pediatr Int 2007; 49:540-542. [9]Fukagawa N, Friedman S, Gill FM, Schwartz E, Shaller C. Hereditary spherocytosis with normal osmotic fragility after incubation. Is the autohemolysis test really obsolete? Jama 1979; 242:63-64. [10]Godal HC, Gjonnes G, Ruyter R. Does preincubation of the red blood cells contribute to the capability of the osmotic fragility test to detect very mild forms of hereditary spherocytosis? Scand J Haematol 1982; 29:89-93. [11]Korones D, Pearson HA. Normal erythrocyte osmotic fragility in hereditary spherocytosis. J Pediatr 1989; 114:264-266. [12]Streichman S, Gescheidt Y. Cryohemolysis for the detection of hereditary spherocytosis: correlation studies with osmotic fragility and autohemolysis. Am J Hematol 1998; 58:206-212. [13]Rocha S, Costa E, Catarino C, et al. Erythropoietin levels in the different clinical forms of hereditary spherocytosis. Br J Haematol 2005; 131:534-542. [14]Rocha S, Costa E, Rocha-Pereira P, et al. Erythrocyte membrane protein destabilization versus clinical outcome in 160 Portuguese Hereditary Spherocytosis patients. Br J Haematol 2010; 149:785-794. [15]Rocha S, Rebelo I, Costa E, et al. Protein deficiency balance as a predictor of clinical outcome in hereditary spherocytosis. Eur J Haematol 2005; 74:374-380. [16]Rocha S, Vitorino RM, Lemos-Amado FM, et al. Presence of cytosolic peroxiredoxin 2 in the erythrocyte membrane of patients with hereditary spherocytosis. Blood Cells Mol Dis 2008; 41:5-9. [17]Iglauer A, Reinhardt D, Schroter W, Pekrun A. Cryohemolysis test as a diagnostic tool for hereditary spherocytosis. Ann Hematol 1999; 78:555-557. [18]Romero RR, Poo JL, Robles JA, Uriostegui A, Vargas F, Majluf-Cruz A. Usefulness of cryohemolysis test in the diagnosis of hereditary spherocytosis. Arch Med Res 1997; 28:247-251. [19]Streichman S, Gesheidt Y, Tatarsky I. Hypertonic cryohemolysis: a diagnostic test for hereditary spherocytosis. Am J Hematol 1990; 35:104-109. [20]Michaels LA, Cohen AR, Zhao H, Raphael RI, Manno CS. Screening for hereditary spherocytosis by use of automated erythrocyte indexes. J Pediatr 1997; 130:957-960. [21]Rocha S, Costa E, Ferreira F, et al. Erythropoiesis versus inflammation in Hereditary Spherocytosis clinical outcome. Clin Biochem 2011. In Press. [22]Kendall RG. Erythropoietin. Clin Lab Haematol 2001; 23:71-80. [23]Cooper AC, Mikhail A, Lethbridge MW, Kemeny DM, Macdougall IC. Increased expression of erythropoiesis inhibiting cytokines (IFN-gamma, TNF-alpha, IL-10, and IL-13) by T cells in patients exhibiting a poor response to erythropoietin therapy. J Am Soc Nephrol 2003; 14:1776-1784. [24]Jelkmann W. Erythropoietin after a century of research: younger than ever. Eur J Haematol 2007; 78:183-205. [25]Macdougall IC, Cooper AC. Erythropoietin resistance: the role of inflammation and pro-inflammatory cytokines. Nephrol Dial Transplant 2002; 17 Suppl 11:39-43. [26]Means RT, Jr., Krantz SB. Progress in understanding the pathogenesis of the anemia of chronic disease. Blood 1992; 80:1639-1647.

Otros artículos de Susana Rocha

Rocha S, Rocha-Pereira P, Ferreira F, Cleto E, Barbot J, Quintanilha A, Belo L, Santos-Silva A. Erythropoiesis versus inflammation in Hereditary Spherocytosis clinical outcome. Clinical Biochemistry 2011;In Press.

Rocha S, Costa E, Rocha-Pereira P, Ferreira F, Cleto E, Barbot J, Quintanilha A, Belo L, Santos-Silva A. Complementary markers for the clinical severity classification of Hereditary Spherocytosis in unsplenectomized patients. Blood Cells, Molecules, and Diseases 2011; 46:166-170.

Rocha S, Costa E, Rocha-Pereira P, Ferreira F, Cleto E, Barbot J, Quintanilha A, Belo L, Santos-Silva A. Erythrocyte membrane protein destabilization versus clinical outcome in 160 Portuguese Hereditary Spherocytosis patients. British Journal of Haematology2010;149:785-794.

Rocha S, Costa E, Ferreira F, Cleto E, Barbot J, Rocha-Pereira P, Quintanilha A, Belo L, Santos-Silva A. Hereditary Spherocytosis and the (TA)nTAA polymorphism of UGT1A1 gene promoter region – A comparison of the bilirubin plasmatic levels in the different clinical forms. Blood Cells, Molecules, and Diseases 2010;44:117-119.

Rocha S, Costa E, Coimbra S, Nascimento H, Catarino C, Rocha-Pereira P, Quintanilha A, Belo L, Santos-Silva A. Linkage of cytosolic peroxiredoxin 2 to the erythrocyte membrane imposed by hydrogen peroxide-induced oxidative stress. Blood Cells, Molecules, and Diseases 2009;43:68-73.

Rocha S, Vitorino RMP, Lemos-Amado FM, Castro EB, Rocha-Pereira P, Barbot J, Cleto E, Ferreira F, Quintanilha A, Belo L, Santos-Silva A. Presence of cytosolic peroxiredoxin 2 in the erythrocyte membrane of patients with Hereditary Spherocytosis. Blood Cells, Molecules, and Diseases 2008;41(1):5-9.

Rocha S, Costa E, Catarino C, Belo L, Castro EMB, Barbot J, Quintanilha A, Santos-Silva A. Erythropoietin levels in the different clinical forms of Hereditary Spherocytosis. British Journal of Haematology 2005;131(4):534-542.

Rocha S, Rebelo I, Costa E, Catarino C, Belo L, Castro EMB, Cabeda JM, Barbot J, Quintanilha A, Santos-Silva A. Protein deficiency balance asa predictor of clinical outcome in Hereditary Spherocytosis. European Journal of Haematology 2005;74(5):374-380.

Branca R, Costa E, Rocha S, Coelho H, Quintanilha A, Cabeda JM, Santos-Silva A, Barbot J. Coexistence of congenital red cell pyruvate kinase and band 3 deficiency. Clinical and Laboratory Haematology 2004;26(4):297-300.

Para comunicarse con Susana Rocha mencionar a SIIC como referencia:
susaroc@hotmail.com

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Acerca del trabajo completo

SEVERIDADE CLÍNICA DA ESFEROCITOSE HEREDITÁRIA – MARCADORES ANALÍTICOS

Título original en castellano
MARCADORES COMPLEMENTARES PARA A CLASSIFICAÇÃO DA SEVERIDADE CLINICA DA ESFEROCITOSE HEREDITARIA EM DOENTES NÃO-ESPLENECTOMIZADOS

Autores
Susana Rocha¹, Elísio Costa², Petronila Rocha-Pereira³, Fátima Ferreira⁴, Esmeralda Cleto⁵, José Barbot⁶, Alexandre Quintanilha⁷, Luís Belo⁸, Alice Santos-Silva⁹
1 Docente Universitária, Instituto de Biologia Molecular e Celular (IBMC), Universidade do Porto, Porto, Portugal, Investigadora
2 Professor Universitário, Instituto de Biologia Molecular e Celular (ibmc), Universidade Do Porto, Porto; Instituto de Ciências da Saúde, Universidade Católica Portuguesa, Porto, Professor Auxiliar
3 Professora Universitária, Instituto de Biologia Molecular e Celular (ibmc), Universidade Do Porto, Porto; Centro de Investigação Em Ciências da Saúde (cics), Universidade da Beira Interior, Covilhã, Professora Auxiliar
4 Médica, Serviço de Hematologia Clínica, Hospital S. João, Porto, Médica
5 Médica, Sector de Hematologia Pediátrica, Centro Hospitalar Do Porto – Hospital Santo António, Porto, Médica
6 Médico, Serviço de Hematologia, Centro Hospitalar Do Porto – Hospital Maria Pia, Porto;, Médico
7 Professor Universitário, Instituto de Biologia Molecular e Celular (ibmc), Universidade Do Porto, Porto; Instituto de Ciências Biomédicas Abel Salazar (icbas), Universidade Do Porto, Porto, Portugal, Professor Catedrático
8 Professor Universitário, Faculdade de Farmácia, Universidade Do Porto, Porto; Instituto de Biologia Molecular e Celular (ibmc), Universidade Do Porto, Porto, Professor Auxiliar
9 Professora Universitária, Faculdade de Farmácia, Universidade Do Porto, Porto; Instituto de Biologia Molecular e Celular (ibmc), Universidade Do Porto, Porto, Professora Associada

Acceso a la fuente original
Blood Cells, Molecules & Diseases
http://www.elsevier.com/wps/find/journaldescription.cws_home/622796/description#description

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