Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia
Autor(a) principal: | |
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Data de Publicação: | 2005 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UNIFESP |
dARK ID: | ark:/48912/0013000007zk8 |
Texto Completo: | http://dx.doi.org/10.1002/ajh.20293 http://repositorio.unifesp.br/handle/11600/28158 |
Resumo: | Fetal hemoglobin (HbF) inhibits the polymerization of sickle hemoglobin, modulating the clinical features of sickle cell anemia (SCA). Hydroxyurea (HU) therapy can increase the HbF level, although its production can be influenced by genetic determinants. Twenty-two Brazilian SCA patients were evaluated over 5 years before and after HU use. We analyzed (1) beta(S) haplotype; (2) patient characteristics; and (3) toxicity. No differences between age, sex, and HU response were observed. We found 40.9% of homozygous for Bantu haplotype, and, in contrasting to other trials, we observed HbF level increase in this group (3.84-9.08 g/dL, P = 0.003). Adverse effects were rare. Labyrinthitis was observed in 2 (9.10%) patients after HU use, although this complication had not been described before. |
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Repositório Institucional da UNIFESP |
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Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemiafetal hemoglobinhydroxyureabeta(s) haplotypesickle cell anemiaFetal hemoglobin (HbF) inhibits the polymerization of sickle hemoglobin, modulating the clinical features of sickle cell anemia (SCA). Hydroxyurea (HU) therapy can increase the HbF level, although its production can be influenced by genetic determinants. Twenty-two Brazilian SCA patients were evaluated over 5 years before and after HU use. We analyzed (1) beta(S) haplotype; (2) patient characteristics; and (3) toxicity. No differences between age, sex, and HU response were observed. We found 40.9% of homozygous for Bantu haplotype, and, in contrasting to other trials, we observed HbF level increase in this group (3.84-9.08 g/dL, P = 0.003). Adverse effects were rare. Labyrinthitis was observed in 2 (9.10%) patients after HU use, although this complication had not been described before.Universidade Federal de São Paulo, UNIFESP, EPM, Disciplina Hematol & Hemoterapia, BR-04023000 São Paulo, BrazilUniversidade Federal de São Paulo, UNIFESP, EPM, Disciplina Hematol & Hemoterapia, BR-04023000 São Paulo, BrazilWeb of ScienceWiley-BlackwellUniversidade Federal de São Paulo (UNIFESP)Vicari, Perla [UNIFESP]Mello, Adriana Barretto de [UNIFESP]Figueiredo, Maria Stella [UNIFESP]2016-01-24T12:37:41Z2016-01-24T12:37:41Z2005-03-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion243-244http://dx.doi.org/10.1002/ajh.20293American Journal of Hematology. Hoboken: Wiley-liss, v. 78, n. 3, p. 243-244, 2005.10.1002/ajh.202930361-8609http://repositorio.unifesp.br/handle/11600/28158WOS:000227412900015ark:/48912/0013000007zk8engAmerican Journal of Hematologyinfo:eu-repo/semantics/openAccesshttp://olabout.wiley.com/WileyCDA/Section/id-406071.htmlreponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESP2022-09-27T10:00:03Zoai:repositorio.unifesp.br/:11600/28158Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestbiblioteca.csp@unifesp.bropendoar:34652024-12-11T20:03:59.312291Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false |
dc.title.none.fl_str_mv |
Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia |
title |
Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia |
spellingShingle |
Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia Vicari, Perla [UNIFESP] fetal hemoglobin hydroxyurea beta(s) haplotype sickle cell anemia |
title_short |
Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia |
title_full |
Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia |
title_fullStr |
Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia |
title_full_unstemmed |
Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia |
title_sort |
Effects of hydroxyurea in a population of Brazilian patients with sickle cell anemia |
author |
Vicari, Perla [UNIFESP] |
author_facet |
Vicari, Perla [UNIFESP] Mello, Adriana Barretto de [UNIFESP] Figueiredo, Maria Stella [UNIFESP] |
author_role |
author |
author2 |
Mello, Adriana Barretto de [UNIFESP] Figueiredo, Maria Stella [UNIFESP] |
author2_role |
author author |
dc.contributor.none.fl_str_mv |
Universidade Federal de São Paulo (UNIFESP) |
dc.contributor.author.fl_str_mv |
Vicari, Perla [UNIFESP] Mello, Adriana Barretto de [UNIFESP] Figueiredo, Maria Stella [UNIFESP] |
dc.subject.por.fl_str_mv |
fetal hemoglobin hydroxyurea beta(s) haplotype sickle cell anemia |
topic |
fetal hemoglobin hydroxyurea beta(s) haplotype sickle cell anemia |
description |
Fetal hemoglobin (HbF) inhibits the polymerization of sickle hemoglobin, modulating the clinical features of sickle cell anemia (SCA). Hydroxyurea (HU) therapy can increase the HbF level, although its production can be influenced by genetic determinants. Twenty-two Brazilian SCA patients were evaluated over 5 years before and after HU use. We analyzed (1) beta(S) haplotype; (2) patient characteristics; and (3) toxicity. No differences between age, sex, and HU response were observed. We found 40.9% of homozygous for Bantu haplotype, and, in contrasting to other trials, we observed HbF level increase in this group (3.84-9.08 g/dL, P = 0.003). Adverse effects were rare. Labyrinthitis was observed in 2 (9.10%) patients after HU use, although this complication had not been described before. |
publishDate |
2005 |
dc.date.none.fl_str_mv |
2005-03-01 2016-01-24T12:37:41Z 2016-01-24T12:37:41Z |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://dx.doi.org/10.1002/ajh.20293 American Journal of Hematology. Hoboken: Wiley-liss, v. 78, n. 3, p. 243-244, 2005. 10.1002/ajh.20293 0361-8609 http://repositorio.unifesp.br/handle/11600/28158 WOS:000227412900015 |
dc.identifier.dark.fl_str_mv |
ark:/48912/0013000007zk8 |
url |
http://dx.doi.org/10.1002/ajh.20293 http://repositorio.unifesp.br/handle/11600/28158 |
identifier_str_mv |
American Journal of Hematology. Hoboken: Wiley-liss, v. 78, n. 3, p. 243-244, 2005. 10.1002/ajh.20293 0361-8609 WOS:000227412900015 ark:/48912/0013000007zk8 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
American Journal of Hematology |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess http://olabout.wiley.com/WileyCDA/Section/id-406071.html |
eu_rights_str_mv |
openAccess |
rights_invalid_str_mv |
http://olabout.wiley.com/WileyCDA/Section/id-406071.html |
dc.format.none.fl_str_mv |
243-244 |
dc.publisher.none.fl_str_mv |
Wiley-Blackwell |
publisher.none.fl_str_mv |
Wiley-Blackwell |
dc.source.none.fl_str_mv |
reponame:Repositório Institucional da UNIFESP instname:Universidade Federal de São Paulo (UNIFESP) instacron:UNIFESP |
instname_str |
Universidade Federal de São Paulo (UNIFESP) |
instacron_str |
UNIFESP |
institution |
UNIFESP |
reponame_str |
Repositório Institucional da UNIFESP |
collection |
Repositório Institucional da UNIFESP |
repository.name.fl_str_mv |
Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP) |
repository.mail.fl_str_mv |
biblioteca.csp@unifesp.br |
_version_ |
1818602423100375040 |