Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis
Autor(a) principal: | |
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Data de Publicação: | 2021 |
Outros Autores: | , , , , , , , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Research, Society and Development |
Texto Completo: | https://rsdjournal.org/index.php/rsd/article/view/18365 |
Resumo: | Atypical hemolytic uremic syndrome (aHUS) is characterized by a triad of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. The pathogenesis of HUS corresponds to thrombotic microangiopathy (THA). The work aims to expose through the analysis of scientific articles the main clinical manifestations and challenges in the diagnosis of Atypical Hemolytic-Uremic Syndrome. This is a literature review of a qualitative nature based on scientific production based on studies published between 2018 and 2021. Although atypical hemolytic uremic syndrome is rare, it is related to complications and important sequelae secondary to alterations of kidney function; 42% of children and 27% of adults may have severe thrombocytopenia (<50,000 platelets/mL). Similarly, related acute kidney injury is severe in 59% of children and 81% of adults, and may be accompanied by glomerular involvement, creatinine, hematuria, proteinuria, edema, and hypertension. The treatment that is most effective against aHUS is the use of the drug Eculizumab. This drug has a high cost, but studies show an increase in life expectancy and quality of life for these patients with the use of this medication. It is necessary that more studies about this disease are carried out for the development of new diagnostic measures and therapies against aHUS at a lower cost and with easy access for the population due to the seriousness of the disease and its high mortality rate. |
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Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosisSíndrome Hemolítico-Urémico Atípico: Manifestaciones clínicas y desafíos en el diagnósticoSíndrome Hemolítico-Urêmica Atípica: Manifestações clínicas e desafios no diagnósticoMicroangiopatiasAnemia HemolíticaSíndrome UrêmicaDiagnóstico.MicroangiopatíasAnemia hemolíticaSíndrome urémicoDiagnóstico.MicroangiopathiesHemolytic anemiaUremic SyndromeDiagnosis.Atypical hemolytic uremic syndrome (aHUS) is characterized by a triad of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. The pathogenesis of HUS corresponds to thrombotic microangiopathy (THA). The work aims to expose through the analysis of scientific articles the main clinical manifestations and challenges in the diagnosis of Atypical Hemolytic-Uremic Syndrome. This is a literature review of a qualitative nature based on scientific production based on studies published between 2018 and 2021. Although atypical hemolytic uremic syndrome is rare, it is related to complications and important sequelae secondary to alterations of kidney function; 42% of children and 27% of adults may have severe thrombocytopenia (<50,000 platelets/mL). Similarly, related acute kidney injury is severe in 59% of children and 81% of adults, and may be accompanied by glomerular involvement, creatinine, hematuria, proteinuria, edema, and hypertension. The treatment that is most effective against aHUS is the use of the drug Eculizumab. This drug has a high cost, but studies show an increase in life expectancy and quality of life for these patients with the use of this medication. It is necessary that more studies about this disease are carried out for the development of new diagnostic measures and therapies against aHUS at a lower cost and with easy access for the population due to the seriousness of the disease and its high mortality rate.El síndrome urémico hemolítico atípico (SHUa) se caracteriza por una tríada de trombocitopenia, anemia hemolítica microangiopática e insuficiencia renal aguda. La patogenia del SUH corresponde a la microangiopatía trombótica (ATC). El trabajo tiene como objetivo exponer a través del análisis de artículos científicos las principales manifestaciones clínicas y desafíos en el diagnóstico del Síndrome Hemolítico-Urémico Atípico. Se trata de una revisión de la literatura de carácter cualitativo basada en la producción científica basada en estudios publicados entre 2018 y 2021. Aunque el síndrome urémico hemolítico atípico es raro, se relaciona con complicaciones y secuelas importantes secundarias a alteraciones de la función renal; El 42% de los niños y el 27% de los adultos pueden tener trombocitopenia grave (<50 000 plaquetas / ml). De manera similar, la lesión renal aguda relacionada es grave en 59% de los niños y 81% de los adultos, y puede estar acompañada de afectación glomerular, creatinina, hematuria, proteinuria, edema e hipertensión. El tratamiento más eficaz contra el SHUa es el uso del fármaco Eculizumab. Este medicamento tiene un costo elevado, pero los estudios muestran un aumento en la esperanza de vida y la calidad de vida de estos pacientes con el uso de este medicamento. Es necesario que se realicen más estudios sobre esta enfermedad para el desarrollo de nuevas medidas diagnósticas y terapias contra el SHUa a menor costo y de fácil acceso para la población debido a la gravedad de la enfermedad y su alta tasa de mortalidad.A síndrome hemolítico-urêmica atípica (SHUa) é caracterizada por uma tríade de trombocitopenia, anemia hemolítica micro angiopática e insuficiência renal aguda. A patogênese da SHU corresponde à micro angiopatia trombótica (MAT). O trabalho tem por objetivo expor através da análise de artigos científicos as principais manifestações clínicas e os desafios no diagnóstico da Síndrome Hemolítico-Urêmica Atípica. Trata-se de uma revisão bibliográfica de caráter qualitativo que se baseia na produção científica a partir de estudos já publicados entre os anos de 2018 a 2021. Embora a síndrome hemolítico-urêmica atípica seja rara, está relacionada a complicações e sequelas importantes secundárias a alterações da função renal; 42% das crianças e 27% dos adultos podem apresentar trombocitopenia grave (<50.000 plaquetas / mL). Da mesma forma, a lesão renal aguda relacionada é grave em 59% das crianças e 81% dos adultos, e pode ser acompanhada por envolvimento glomerular, creatinina, hematúria, proteinúria, edema e hipertensão. O tratamento que possui mais eficácia contra a SHUa é com a utilização do fármaco Eculizumab. Este fármaco apresenta um alto custo, mas estudos mostram aumento da expectativa e qualidade de vida desses pacientes com o uso desta medicação. É necessário que sejam realizados mais estudos acerca desta doença para o desenvolvimento de novas medidas de diagnóstico e terapias contra a SHUa com menor custo e com fácil acesso para a população devido a gravidade da doença e da sua alta taxa de mortalidade.Research, Society and Development2021-08-03info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://rsdjournal.org/index.php/rsd/article/view/1836510.33448/rsd-v10i9.18365Research, Society and Development; Vol. 10 No. 9; e58210918365Research, Society and Development; Vol. 10 Núm. 9; e58210918365Research, Society and Development; v. 10 n. 9; e582109183652525-3409reponame:Research, Society and Developmentinstname:Universidade Federal de Itajubá (UNIFEI)instacron:UNIFEIporhttps://rsdjournal.org/index.php/rsd/article/view/18365/16494Copyright (c) 2021 Lennara Pereira Mota; Maria Vitalina Alves de Sousa; Ana Alinne Gomes da Penha; Alexandra Rodrigues Cardoso; Francisco de Assis da Silva Sousa; Francilene Vieira da Silva Freitas; Josana de Mello Dantas; Thaline Ravena Nunes Costa; Thaynara Alves Rodrigues Paulo da Silva; Eudes Rufino da Silveira Filho; Wilian Reis Rosário; Regina Célia da Silva; Lucas Costa de Gois; Jaqueline Dantas da Costa; Mayara Madeira de Carvalho; Stefânia Araújo Pereira; Joice Mara Ferreira dos Santoshttps://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessMota, Lennara PereiraSousa, Maria Vitalina Alves de Penha, Ana Alinne Gomes da Cardoso, Alexandra RodriguesSousa, Francisco de Assis da Silva Freitas, Francilene Vieira da Silva Dantas, Josana de MelloCosta, Thaline Ravena NunesSilva, Thaynara Alves Rodrigues Paulo da Silveira Filho, Eudes Rufino da Rosário, Wilian Reis Silva, Regina Célia da Gois, Lucas Costa de Costa, Jaqueline Dantas da Carvalho, Mayara Madeira de Pereira, Stefânia Araújo Santos, Joice Mara Ferreira dos 2021-09-12T14:28:06Zoai:ojs.pkp.sfu.ca:article/18365Revistahttps://rsdjournal.org/index.php/rsd/indexPUBhttps://rsdjournal.org/index.php/rsd/oairsd.articles@gmail.com2525-34092525-3409opendoar:2024-01-17T09:38:31.094173Research, Society and Development - Universidade Federal de Itajubá (UNIFEI)false |
dc.title.none.fl_str_mv |
Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis Síndrome Hemolítico-Urémico Atípico: Manifestaciones clínicas y desafíos en el diagnóstico Síndrome Hemolítico-Urêmica Atípica: Manifestações clínicas e desafios no diagnóstico |
title |
Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis |
spellingShingle |
Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis Mota, Lennara Pereira Microangiopatias Anemia Hemolítica Síndrome Urêmica Diagnóstico. Microangiopatías Anemia hemolítica Síndrome urémico Diagnóstico. Microangiopathies Hemolytic anemia Uremic Syndrome Diagnosis. |
title_short |
Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis |
title_full |
Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis |
title_fullStr |
Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis |
title_full_unstemmed |
Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis |
title_sort |
Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis |
author |
Mota, Lennara Pereira |
author_facet |
Mota, Lennara Pereira Sousa, Maria Vitalina Alves de Penha, Ana Alinne Gomes da Cardoso, Alexandra Rodrigues Sousa, Francisco de Assis da Silva Freitas, Francilene Vieira da Silva Dantas, Josana de Mello Costa, Thaline Ravena Nunes Silva, Thaynara Alves Rodrigues Paulo da Silveira Filho, Eudes Rufino da Rosário, Wilian Reis Silva, Regina Célia da Gois, Lucas Costa de Costa, Jaqueline Dantas da Carvalho, Mayara Madeira de Pereira, Stefânia Araújo Santos, Joice Mara Ferreira dos |
author_role |
author |
author2 |
Sousa, Maria Vitalina Alves de Penha, Ana Alinne Gomes da Cardoso, Alexandra Rodrigues Sousa, Francisco de Assis da Silva Freitas, Francilene Vieira da Silva Dantas, Josana de Mello Costa, Thaline Ravena Nunes Silva, Thaynara Alves Rodrigues Paulo da Silveira Filho, Eudes Rufino da Rosário, Wilian Reis Silva, Regina Célia da Gois, Lucas Costa de Costa, Jaqueline Dantas da Carvalho, Mayara Madeira de Pereira, Stefânia Araújo Santos, Joice Mara Ferreira dos |
author2_role |
author author author author author author author author author author author author author author author author |
dc.contributor.author.fl_str_mv |
Mota, Lennara Pereira Sousa, Maria Vitalina Alves de Penha, Ana Alinne Gomes da Cardoso, Alexandra Rodrigues Sousa, Francisco de Assis da Silva Freitas, Francilene Vieira da Silva Dantas, Josana de Mello Costa, Thaline Ravena Nunes Silva, Thaynara Alves Rodrigues Paulo da Silveira Filho, Eudes Rufino da Rosário, Wilian Reis Silva, Regina Célia da Gois, Lucas Costa de Costa, Jaqueline Dantas da Carvalho, Mayara Madeira de Pereira, Stefânia Araújo Santos, Joice Mara Ferreira dos |
dc.subject.por.fl_str_mv |
Microangiopatias Anemia Hemolítica Síndrome Urêmica Diagnóstico. Microangiopatías Anemia hemolítica Síndrome urémico Diagnóstico. Microangiopathies Hemolytic anemia Uremic Syndrome Diagnosis. |
topic |
Microangiopatias Anemia Hemolítica Síndrome Urêmica Diagnóstico. Microangiopatías Anemia hemolítica Síndrome urémico Diagnóstico. Microangiopathies Hemolytic anemia Uremic Syndrome Diagnosis. |
description |
Atypical hemolytic uremic syndrome (aHUS) is characterized by a triad of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. The pathogenesis of HUS corresponds to thrombotic microangiopathy (THA). The work aims to expose through the analysis of scientific articles the main clinical manifestations and challenges in the diagnosis of Atypical Hemolytic-Uremic Syndrome. This is a literature review of a qualitative nature based on scientific production based on studies published between 2018 and 2021. Although atypical hemolytic uremic syndrome is rare, it is related to complications and important sequelae secondary to alterations of kidney function; 42% of children and 27% of adults may have severe thrombocytopenia (<50,000 platelets/mL). Similarly, related acute kidney injury is severe in 59% of children and 81% of adults, and may be accompanied by glomerular involvement, creatinine, hematuria, proteinuria, edema, and hypertension. The treatment that is most effective against aHUS is the use of the drug Eculizumab. This drug has a high cost, but studies show an increase in life expectancy and quality of life for these patients with the use of this medication. It is necessary that more studies about this disease are carried out for the development of new diagnostic measures and therapies against aHUS at a lower cost and with easy access for the population due to the seriousness of the disease and its high mortality rate. |
publishDate |
2021 |
dc.date.none.fl_str_mv |
2021-08-03 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://rsdjournal.org/index.php/rsd/article/view/18365 10.33448/rsd-v10i9.18365 |
url |
https://rsdjournal.org/index.php/rsd/article/view/18365 |
identifier_str_mv |
10.33448/rsd-v10i9.18365 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://rsdjournal.org/index.php/rsd/article/view/18365/16494 |
dc.rights.driver.fl_str_mv |
https://creativecommons.org/licenses/by/4.0 info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
https://creativecommons.org/licenses/by/4.0 |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Research, Society and Development |
publisher.none.fl_str_mv |
Research, Society and Development |
dc.source.none.fl_str_mv |
Research, Society and Development; Vol. 10 No. 9; e58210918365 Research, Society and Development; Vol. 10 Núm. 9; e58210918365 Research, Society and Development; v. 10 n. 9; e58210918365 2525-3409 reponame:Research, Society and Development instname:Universidade Federal de Itajubá (UNIFEI) instacron:UNIFEI |
instname_str |
Universidade Federal de Itajubá (UNIFEI) |
instacron_str |
UNIFEI |
institution |
UNIFEI |
reponame_str |
Research, Society and Development |
collection |
Research, Society and Development |
repository.name.fl_str_mv |
Research, Society and Development - Universidade Federal de Itajubá (UNIFEI) |
repository.mail.fl_str_mv |
rsd.articles@gmail.com |
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1797052787643645952 |