Interferentes eritrocitários e ambientais na anemia falciforme
Autor(a) principal: | |
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Data de Publicação: | 2000 |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Institucional da UNESP |
DOI: | 10.1590/S1516-84842000000100003 |
Texto Completo: | http://dx.doi.org/10.1590/S1516-84842000000100003 http://hdl.handle.net/11449/66358 |
Resumo: | Erythrocytes and environmental interferences on sickle cell anaemia Sickle cell anaemia runs na extremely variable clinical course At one end of spectrum, it is characterized by a crippling haemolitic anaemia, interspersed with severe exacerbations, or crises, yet it may be an extremely mild disorder, which is found only by chance on rotine haematological examination. The reasons are only partly understood for these remarkable differences in phenotypic expression of what appears to be the same genetic defect: they include the level of Hb Fetal, coinheritance of the alpha thalassaemia and of other genetic variantsthat has influence as genetic modulation in sickle cell anaemia. However, other genetics abnormalities of erythrocytes: G-6PD deficiency, spherocytosis and deficiencies of anti-oxidant enzymes(SOD, GPx and Catalase) probably interfereon the clinical course of sickle cell anaemia. The haplotypes of the chromosome (Bantu, Benin, Camaroon and Arab-Indian) bearing the sickle gene is associated with assorted haematological and clinical features that are likely, at least in part, to be mediated throgh effects on Hb Fetal concentration. Beyond these factors characterizes as erythrocytes interferents, there are the environmental interferents. Between environmental interferents become detached the socio-economic and cultural situation of each patient. These aspects have influence on the life of affected individuals including social interactions, family relations, peer interaction, intimate relationships, education, enployment, violence, spiritual attitudes and navigating complexities of the health care system, providers and their ancillary functions. As a result of this article it is proposed a protocol of laboratorial management of sickle cell syndrome with detach to sickle cell anaemia. |
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Interferentes eritrocitários e ambientais na anemia falciformeEnvironmental interferencesErythrocytes interferencesSickle cell anaemiaErythrocytes and environmental interferences on sickle cell anaemia Sickle cell anaemia runs na extremely variable clinical course At one end of spectrum, it is characterized by a crippling haemolitic anaemia, interspersed with severe exacerbations, or crises, yet it may be an extremely mild disorder, which is found only by chance on rotine haematological examination. The reasons are only partly understood for these remarkable differences in phenotypic expression of what appears to be the same genetic defect: they include the level of Hb Fetal, coinheritance of the alpha thalassaemia and of other genetic variantsthat has influence as genetic modulation in sickle cell anaemia. However, other genetics abnormalities of erythrocytes: G-6PD deficiency, spherocytosis and deficiencies of anti-oxidant enzymes(SOD, GPx and Catalase) probably interfereon the clinical course of sickle cell anaemia. The haplotypes of the chromosome (Bantu, Benin, Camaroon and Arab-Indian) bearing the sickle gene is associated with assorted haematological and clinical features that are likely, at least in part, to be mediated throgh effects on Hb Fetal concentration. Beyond these factors characterizes as erythrocytes interferents, there are the environmental interferents. Between environmental interferents become detached the socio-economic and cultural situation of each patient. These aspects have influence on the life of affected individuals including social interactions, family relations, peer interaction, intimate relationships, education, enployment, violence, spiritual attitudes and navigating complexities of the health care system, providers and their ancillary functions. As a result of this article it is proposed a protocol of laboratorial management of sickle cell syndrome with detach to sickle cell anaemia.Departamento de Biologia Instituto de Biociências, Letras e Ciências Exatas Universidade Estadual Paulista, São José do Rio Preto, SPDepartamento de Biologia IBILCE-UNESP, Rua Cristovão Colombo, 2265. CEP: 15050-000. Sao Jose do Rio Preto. SPDepartamento de Biologia Instituto de Biociências, Letras e Ciências Exatas Universidade Estadual Paulista, São José do Rio Preto, SPDepartamento de Biologia IBILCE-UNESP, Rua Cristovão Colombo, 2265. CEP: 15050-000. Sao Jose do Rio Preto. SPUniversidade Estadual Paulista (Unesp)Naoum, Paulo C. [UNESP]2014-05-27T11:19:59Z2014-05-27T11:19:59Z2000-12-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article5-22application/pdfhttp://dx.doi.org/10.1590/S1516-84842000000100003Revista Brasileira de Hematologia e Hemoterapia, v. 22, n. 1, p. 5-22, 2000.1516-8484http://hdl.handle.net/11449/6635810.1590/S1516-84842000000100003S1516-848420000001000032-s2.0-337473752692-s2.0-33747375269.pdfScopusreponame:Repositório Institucional da UNESPinstname:Universidade Estadual Paulista (UNESP)instacron:UNESPporRevista Brasileira de Hematologia e Hemoterapia0,335info:eu-repo/semantics/openAccess2024-01-15T06:19:29Zoai:repositorio.unesp.br:11449/66358Repositório InstitucionalPUBhttp://repositorio.unesp.br/oai/requestopendoar:29462024-08-05T23:01:27.930709Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP)false |
dc.title.none.fl_str_mv |
Interferentes eritrocitários e ambientais na anemia falciforme |
title |
Interferentes eritrocitários e ambientais na anemia falciforme |
spellingShingle |
Interferentes eritrocitários e ambientais na anemia falciforme Interferentes eritrocitários e ambientais na anemia falciforme Naoum, Paulo C. [UNESP] Environmental interferences Erythrocytes interferences Sickle cell anaemia Naoum, Paulo C. [UNESP] Environmental interferences Erythrocytes interferences Sickle cell anaemia |
title_short |
Interferentes eritrocitários e ambientais na anemia falciforme |
title_full |
Interferentes eritrocitários e ambientais na anemia falciforme |
title_fullStr |
Interferentes eritrocitários e ambientais na anemia falciforme Interferentes eritrocitários e ambientais na anemia falciforme |
title_full_unstemmed |
Interferentes eritrocitários e ambientais na anemia falciforme Interferentes eritrocitários e ambientais na anemia falciforme |
title_sort |
Interferentes eritrocitários e ambientais na anemia falciforme |
author |
Naoum, Paulo C. [UNESP] |
author_facet |
Naoum, Paulo C. [UNESP] Naoum, Paulo C. [UNESP] |
author_role |
author |
dc.contributor.none.fl_str_mv |
Universidade Estadual Paulista (Unesp) |
dc.contributor.author.fl_str_mv |
Naoum, Paulo C. [UNESP] |
dc.subject.por.fl_str_mv |
Environmental interferences Erythrocytes interferences Sickle cell anaemia |
topic |
Environmental interferences Erythrocytes interferences Sickle cell anaemia |
description |
Erythrocytes and environmental interferences on sickle cell anaemia Sickle cell anaemia runs na extremely variable clinical course At one end of spectrum, it is characterized by a crippling haemolitic anaemia, interspersed with severe exacerbations, or crises, yet it may be an extremely mild disorder, which is found only by chance on rotine haematological examination. The reasons are only partly understood for these remarkable differences in phenotypic expression of what appears to be the same genetic defect: they include the level of Hb Fetal, coinheritance of the alpha thalassaemia and of other genetic variantsthat has influence as genetic modulation in sickle cell anaemia. However, other genetics abnormalities of erythrocytes: G-6PD deficiency, spherocytosis and deficiencies of anti-oxidant enzymes(SOD, GPx and Catalase) probably interfereon the clinical course of sickle cell anaemia. The haplotypes of the chromosome (Bantu, Benin, Camaroon and Arab-Indian) bearing the sickle gene is associated with assorted haematological and clinical features that are likely, at least in part, to be mediated throgh effects on Hb Fetal concentration. Beyond these factors characterizes as erythrocytes interferents, there are the environmental interferents. Between environmental interferents become detached the socio-economic and cultural situation of each patient. These aspects have influence on the life of affected individuals including social interactions, family relations, peer interaction, intimate relationships, education, enployment, violence, spiritual attitudes and navigating complexities of the health care system, providers and their ancillary functions. As a result of this article it is proposed a protocol of laboratorial management of sickle cell syndrome with detach to sickle cell anaemia. |
publishDate |
2000 |
dc.date.none.fl_str_mv |
2000-12-01 2014-05-27T11:19:59Z 2014-05-27T11:19:59Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://dx.doi.org/10.1590/S1516-84842000000100003 Revista Brasileira de Hematologia e Hemoterapia, v. 22, n. 1, p. 5-22, 2000. 1516-8484 http://hdl.handle.net/11449/66358 10.1590/S1516-84842000000100003 S1516-84842000000100003 2-s2.0-33747375269 2-s2.0-33747375269.pdf |
url |
http://dx.doi.org/10.1590/S1516-84842000000100003 http://hdl.handle.net/11449/66358 |
identifier_str_mv |
Revista Brasileira de Hematologia e Hemoterapia, v. 22, n. 1, p. 5-22, 2000. 1516-8484 10.1590/S1516-84842000000100003 S1516-84842000000100003 2-s2.0-33747375269 2-s2.0-33747375269.pdf |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
Revista Brasileira de Hematologia e Hemoterapia 0,335 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
5-22 application/pdf |
dc.source.none.fl_str_mv |
Scopus reponame:Repositório Institucional da UNESP instname:Universidade Estadual Paulista (UNESP) instacron:UNESP |
instname_str |
Universidade Estadual Paulista (UNESP) |
instacron_str |
UNESP |
institution |
UNESP |
reponame_str |
Repositório Institucional da UNESP |
collection |
Repositório Institucional da UNESP |
repository.name.fl_str_mv |
Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP) |
repository.mail.fl_str_mv |
|
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1822182564912168960 |
dc.identifier.doi.none.fl_str_mv |
10.1590/S1516-84842000000100003 |