Methemoglobinemia

Detalhes bibliográficos
Autor(a) principal: Murinello, António
Data de Publicação: 2023
Outros Autores: Bicho, Manuel, Estrela Inácio, Rosa, Loureiro, Maria Conceição
Tipo de documento: Artigo
Idioma: por
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: https://revista.spmi.pt/index.php/rpmi/article/view/2023
Resumo: Methemoglobinemia refers to the existence of high blood concentrations of methemoglobin, resulting from the oxidation offerrous iron to ferric iron within the hemoglobin molecule. An extensive review of methemoglobinemia is done, initiaJJy focusing on physiological enzymatic mechanisms of oxidation/ reduction of hemoglobin, referring its efficacy in pathologic methemoglobinemia. Methe­ moglobinemia causes tissue hypoxia, being responsible for chocolate colour type of cyanosis, and other progressive symptoms, including abnormalities of respiratory and cardiac function as the concentration of methemoglobin grow up. Etiology can be genetic or acquired. Acquired form is much more common, and there are a great number of oxidizing agents causing it, namely dn,gs. The most common genetic cause is deficiency of enzymatic system of cytochrome-b5 reductase, from which we know three differentgenetic variations with weak activity. Laboratory confirmation of methemoglobinemia requires co-oxymetry, but clinicai diagnosis is essential. Oxidizing  agents  besides  causing  methe­ moglobinemia can be responsible for some degree of hemolysis, mostly if there is concomitant deficiency of glucose-6-phosphate dehydrogenase, in which case it is better not to use methylene blue in the treatment of methemoglobinemia. ln high doses methylene blue is an oxidizing agent, causing induction of methemoglobin. If there is deficiency of the referred enzyme, the haemolytic action of methylene blue will happen with lower doses of the drug. Exchange transfusions are alternative therapy in serious cases. Recently N-acetylcysteine is being considered as alternative therapy to methylene blue. 
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spelling MethemoglobinemiaMeta-hemoglobinemiaMeta-hemoglobinemiaazul de metilenoredutase do citocrómio-bMethemoglobinemiamethylene bluecytochrome-bMethemoglobinemia refers to the existence of high blood concentrations of methemoglobin, resulting from the oxidation offerrous iron to ferric iron within the hemoglobin molecule. An extensive review of methemoglobinemia is done, initiaJJy focusing on physiological enzymatic mechanisms of oxidation/ reduction of hemoglobin, referring its efficacy in pathologic methemoglobinemia. Methe­ moglobinemia causes tissue hypoxia, being responsible for chocolate colour type of cyanosis, and other progressive symptoms, including abnormalities of respiratory and cardiac function as the concentration of methemoglobin grow up. Etiology can be genetic or acquired. Acquired form is much more common, and there are a great number of oxidizing agents causing it, namely dn,gs. The most common genetic cause is deficiency of enzymatic system of cytochrome-b5 reductase, from which we know three differentgenetic variations with weak activity. Laboratory confirmation of methemoglobinemia requires co-oxymetry, but clinicai diagnosis is essential. Oxidizing  agents  besides  causing  methe­ moglobinemia can be responsible for some degree of hemolysis, mostly if there is concomitant deficiency of glucose-6-phosphate dehydrogenase, in which case it is better not to use methylene blue in the treatment of methemoglobinemia. ln high doses methylene blue is an oxidizing agent, causing induction of methemoglobin. If there is deficiency of the referred enzyme, the haemolytic action of methylene blue will happen with lower doses of the drug. Exchange transfusions are alternative therapy in serious cases. Recently N-acetylcysteine is being considered as alternative therapy to methylene blue. O termo meta-hemoglobinemia refere-se à existência de uma concentração excessiva de meta­ hemoglobina no sangue, resultante da oxidação do ferro ferroso a ferro férrico na molécula de hemoglobina. Nesta revisão, começa-se por focar os mecanismos enzimáticos fisiológicos de oxidação/ redução da hemoglobina, e sua eficácia em caso de meta-hemoglobinemia patológica. A meta­ hemoglobinemia, ao causar hipóxia tissular, determina uma cianose típica cor de chocolate, agravando-se os sintomas acompanhantes e as funções cardíaca e respiratória, à medida que se atingem concentrações cada vez maiores de meta­ hemoglobina no sangue, quando o tratamento se torna uma emergência. A etiologia é genética ou adquirida, sendo esta a mais comum, determinada por um grande número de agentes oxidantes, químicos ou fármacos. A causa genética mais frequente é o deficit da redutase do citocrómio-b_;, enzima de que se conhecem três variantes genéticas. A confirmação laboratorial da meta­ hemoglobinemia faz-se por co-oximetria, mas a clínica é fundamental na indução do pensamento diagnóstico. Os agentes oxidantes, além de causarem meta­ hemoglobinemia, podem serresponsáveis por algum grau de hemólise, nomeadamente se houver deficiência associada de desidrogenase da glucose- 6-fosfato. Neste último caso, será preferível não medicar os doentes com azul de metileno, já que, se em doses elevadas, a substância é em si mesma um agente oxidante indutor da formação de meta­ hemoglobina, no caso do défice da referida enzima a sua acção hemolítica far-se-á sentir em doses mais baixas.Sociedade Portuguesa de Medicina Interna2023-06-06info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://revista.spmi.pt/index.php/rpmi/article/view/2023Internal Medicine; Vol. 8 No. 4 (2001): Outubro/ Dezembro; 210-216Medicina Interna; Vol. 8 N.º 4 (2001): Outubro/ Dezembro; 210-2162183-99800872-671Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://revista.spmi.pt/index.php/rpmi/article/view/2023https://revista.spmi.pt/index.php/rpmi/article/view/2023/1420Murinello, AntónioBicho, ManuelEstrela Inácio, RosaLoureiro, Maria Conceiçãoinfo:eu-repo/semantics/openAccess2023-06-10T06:11:12Zoai:oai.revista.spmi.pt:article/2023Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T18:00:08.704874Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Methemoglobinemia
Meta-hemoglobinemia
title Methemoglobinemia
spellingShingle Methemoglobinemia
Murinello, António
Meta-hemoglobinemia
azul de metileno
redutase do citocrómio-b
Methemoglobinemia
methylene blue
cytochrome-b
title_short Methemoglobinemia
title_full Methemoglobinemia
title_fullStr Methemoglobinemia
title_full_unstemmed Methemoglobinemia
title_sort Methemoglobinemia
author Murinello, António
author_facet Murinello, António
Bicho, Manuel
Estrela Inácio, Rosa
Loureiro, Maria Conceição
author_role author
author2 Bicho, Manuel
Estrela Inácio, Rosa
Loureiro, Maria Conceição
author2_role author
author
author
dc.contributor.author.fl_str_mv Murinello, António
Bicho, Manuel
Estrela Inácio, Rosa
Loureiro, Maria Conceição
dc.subject.por.fl_str_mv Meta-hemoglobinemia
azul de metileno
redutase do citocrómio-b
Methemoglobinemia
methylene blue
cytochrome-b
topic Meta-hemoglobinemia
azul de metileno
redutase do citocrómio-b
Methemoglobinemia
methylene blue
cytochrome-b
description Methemoglobinemia refers to the existence of high blood concentrations of methemoglobin, resulting from the oxidation offerrous iron to ferric iron within the hemoglobin molecule. An extensive review of methemoglobinemia is done, initiaJJy focusing on physiological enzymatic mechanisms of oxidation/ reduction of hemoglobin, referring its efficacy in pathologic methemoglobinemia. Methe­ moglobinemia causes tissue hypoxia, being responsible for chocolate colour type of cyanosis, and other progressive symptoms, including abnormalities of respiratory and cardiac function as the concentration of methemoglobin grow up. Etiology can be genetic or acquired. Acquired form is much more common, and there are a great number of oxidizing agents causing it, namely dn,gs. The most common genetic cause is deficiency of enzymatic system of cytochrome-b5 reductase, from which we know three differentgenetic variations with weak activity. Laboratory confirmation of methemoglobinemia requires co-oxymetry, but clinicai diagnosis is essential. Oxidizing  agents  besides  causing  methe­ moglobinemia can be responsible for some degree of hemolysis, mostly if there is concomitant deficiency of glucose-6-phosphate dehydrogenase, in which case it is better not to use methylene blue in the treatment of methemoglobinemia. ln high doses methylene blue is an oxidizing agent, causing induction of methemoglobin. If there is deficiency of the referred enzyme, the haemolytic action of methylene blue will happen with lower doses of the drug. Exchange transfusions are alternative therapy in serious cases. Recently N-acetylcysteine is being considered as alternative therapy to methylene blue. 
publishDate 2023
dc.date.none.fl_str_mv 2023-06-06
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
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dc.language.iso.fl_str_mv por
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dc.publisher.none.fl_str_mv Sociedade Portuguesa de Medicina Interna
publisher.none.fl_str_mv Sociedade Portuguesa de Medicina Interna
dc.source.none.fl_str_mv Internal Medicine; Vol. 8 No. 4 (2001): Outubro/ Dezembro; 210-216
Medicina Interna; Vol. 8 N.º 4 (2001): Outubro/ Dezembro; 210-216
2183-9980
0872-671X
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