A case of haemophagocytic syndrome presenting with oculogyric crises

Detalhes bibliográficos
Autor(a) principal: Taipa, R.
Data de Publicação: 2010
Outros Autores: Moreira, B., França, M., Maia, L.
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10400.16/717
Resumo: Haemophagocytic lymphohistiocytosis (HLH), also called haemophagocytic syndrome (HPS), is a rare disorder resulting in abnormal proliferation of histiocytes in tissues and organs, including the CNS. HLH can present as a primary disease or occur as a secondary reactive disease. Clinical features are high fever, splenomegaly, cytopenia of two or more cell lines, hypertriglyceridaemia and haemophagocytosis. CNS involvement varies between 10% and 73%, and clinical manifestations include seizures, decreased sensorium, brainstem symptoms, ataxia or demyelinating peripheral neuropathy.
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spelling A case of haemophagocytic syndrome presenting with oculogyric crisesHaemophagocytic lymphohistiocytosis (HLH), also called haemophagocytic syndrome (HPS), is a rare disorder resulting in abnormal proliferation of histiocytes in tissues and organs, including the CNS. HLH can present as a primary disease or occur as a secondary reactive disease. Clinical features are high fever, splenomegaly, cytopenia of two or more cell lines, hypertriglyceridaemia and haemophagocytosis. CNS involvement varies between 10% and 73%, and clinical manifestations include seizures, decreased sensorium, brainstem symptoms, ataxia or demyelinating peripheral neuropathy.BMJ Publishing GroupRepositório Científico do Centro Hospitalar Universitário de Santo AntónioTaipa, R.Moreira, B.França, M.Maia, L.2011-07-04T13:24:17Z20102010-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.16/717engJ Neurol Neurosurg Psychiatry 2010 81: 469-4710022-3050info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-10-20T10:53:34Zoai:repositorio.chporto.pt:10400.16/717Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T20:37:04.980175Repositório 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 A case of haemophagocytic syndrome presenting with oculogyric crises
title A case of haemophagocytic syndrome presenting with oculogyric crises
spellingShingle A case of haemophagocytic syndrome presenting with oculogyric crises
Taipa, R.
title_short A case of haemophagocytic syndrome presenting with oculogyric crises
title_full A case of haemophagocytic syndrome presenting with oculogyric crises
title_fullStr A case of haemophagocytic syndrome presenting with oculogyric crises
title_full_unstemmed A case of haemophagocytic syndrome presenting with oculogyric crises
title_sort A case of haemophagocytic syndrome presenting with oculogyric crises
author Taipa, R.
author_facet Taipa, R.
Moreira, B.
França, M.
Maia, L.
author_role author
author2 Moreira, B.
França, M.
Maia, L.
author2_role author
author
author
dc.contributor.none.fl_str_mv Repositório Científico do Centro Hospitalar Universitário de Santo António
dc.contributor.author.fl_str_mv Taipa, R.
Moreira, B.
França, M.
Maia, L.
description Haemophagocytic lymphohistiocytosis (HLH), also called haemophagocytic syndrome (HPS), is a rare disorder resulting in abnormal proliferation of histiocytes in tissues and organs, including the CNS. HLH can present as a primary disease or occur as a secondary reactive disease. Clinical features are high fever, splenomegaly, cytopenia of two or more cell lines, hypertriglyceridaemia and haemophagocytosis. CNS involvement varies between 10% and 73%, and clinical manifestations include seizures, decreased sensorium, brainstem symptoms, ataxia or demyelinating peripheral neuropathy.
publishDate 2010
dc.date.none.fl_str_mv 2010
2010-01-01T00:00:00Z
2011-07-04T13:24:17Z
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://hdl.handle.net/10400.16/717
url http://hdl.handle.net/10400.16/717
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv J Neurol Neurosurg Psychiatry 2010 81: 469-471
0022-3050
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv BMJ Publishing Group
publisher.none.fl_str_mv BMJ Publishing Group
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
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