Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester Disease

Detalhes bibliográficos
Autor(a) principal: Martins,Nuno
Data de Publicação: 2019
Outros Autores: Achega,Miguel, Rodrigues,Alice, Aldomiro,Fernando
Tipo de documento: Relatório
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-671X2019000200008
Resumo: A 69-year-old woman presented with sudden left hemiparesis. Computed tomography (CT) scan excluded acute brain injuries. Patient was admitted with acute ischemic stroke of right hemisphere. There was full recovery within 24 hours and etiologic studies were normal. There was a concomitant history of ingestion of 6 litres of water per day over the previous 2 years. Water deprivation test followed by the administration of desmopressin confirmed central diabetes insipidus diagnosis. Brain magnetic resonance imaging (MRI) was normal. Full body CT scan showed arterial wall thickening suggestive of large and medium vessels vasculitis, retroperitoneal fibrosis, perinephric infiltration, pleural and pericardial thickening and diffuse densification of greater omentum. Angio-MRI showed occlusive micro-arterial injuries. Laparoscopic biopsy of greater omentum revealed multinucleated giant Touton CD68+, CD1A- cells. This finding is pathognomonic of Erdheim-Chester Disease, a rare non-Langerhans histiocytosis.
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spelling Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester DiseaseDiabetes InsipidusNeurogenicErdheim-Chester DiseasePolydipsiaRetroperitoneal FibrosisStrokeA 69-year-old woman presented with sudden left hemiparesis. Computed tomography (CT) scan excluded acute brain injuries. Patient was admitted with acute ischemic stroke of right hemisphere. There was full recovery within 24 hours and etiologic studies were normal. There was a concomitant history of ingestion of 6 litres of water per day over the previous 2 years. Water deprivation test followed by the administration of desmopressin confirmed central diabetes insipidus diagnosis. Brain magnetic resonance imaging (MRI) was normal. Full body CT scan showed arterial wall thickening suggestive of large and medium vessels vasculitis, retroperitoneal fibrosis, perinephric infiltration, pleural and pericardial thickening and diffuse densification of greater omentum. Angio-MRI showed occlusive micro-arterial injuries. Laparoscopic biopsy of greater omentum revealed multinucleated giant Touton CD68+, CD1A- cells. This finding is pathognomonic of Erdheim-Chester Disease, a rare non-Langerhans histiocytosis.Sociedade Portuguesa de Medicina Interna2019-06-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-671X2019000200008Medicina Interna v.26 n.2 2019reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-671X2019000200008Martins,NunoAchega,MiguelRodrigues,AliceAldomiro,Fernandoinfo:eu-repo/semantics/openAccess2024-02-06T17:08:16Zoai:scielo:S0872-671X2019000200008Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:20:49.646218Repositório 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 Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester Disease
title Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester Disease
spellingShingle Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester Disease
Martins,Nuno
Diabetes Insipidus
Neurogenic
Erdheim-Chester Disease
Polydipsia
Retroperitoneal Fibrosis
Stroke
title_short Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester Disease
title_full Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester Disease
title_fullStr Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester Disease
title_full_unstemmed Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester Disease
title_sort Unusual Manifestations of a Rare Clinical Entity: Erdheim-Chester Disease
author Martins,Nuno
author_facet Martins,Nuno
Achega,Miguel
Rodrigues,Alice
Aldomiro,Fernando
author_role author
author2 Achega,Miguel
Rodrigues,Alice
Aldomiro,Fernando
author2_role author
author
author
dc.contributor.author.fl_str_mv Martins,Nuno
Achega,Miguel
Rodrigues,Alice
Aldomiro,Fernando
dc.subject.por.fl_str_mv Diabetes Insipidus
Neurogenic
Erdheim-Chester Disease
Polydipsia
Retroperitoneal Fibrosis
Stroke
topic Diabetes Insipidus
Neurogenic
Erdheim-Chester Disease
Polydipsia
Retroperitoneal Fibrosis
Stroke
description A 69-year-old woman presented with sudden left hemiparesis. Computed tomography (CT) scan excluded acute brain injuries. Patient was admitted with acute ischemic stroke of right hemisphere. There was full recovery within 24 hours and etiologic studies were normal. There was a concomitant history of ingestion of 6 litres of water per day over the previous 2 years. Water deprivation test followed by the administration of desmopressin confirmed central diabetes insipidus diagnosis. Brain magnetic resonance imaging (MRI) was normal. Full body CT scan showed arterial wall thickening suggestive of large and medium vessels vasculitis, retroperitoneal fibrosis, perinephric infiltration, pleural and pericardial thickening and diffuse densification of greater omentum. Angio-MRI showed occlusive micro-arterial injuries. Laparoscopic biopsy of greater omentum revealed multinucleated giant Touton CD68+, CD1A- cells. This finding is pathognomonic of Erdheim-Chester Disease, a rare non-Langerhans histiocytosis.
publishDate 2019
dc.date.none.fl_str_mv 2019-06-01
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
format report
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-671X2019000200008
url http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-671X2019000200008
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-671X2019000200008
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eu_rights_str_mv openAccess
dc.format.none.fl_str_mv text/html
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 Medicina Interna v.26 n.2 2019
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
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reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
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