Nieman-Pick’s disease
Autor(a) principal: | |
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Data de Publicação: | 2022 |
Outros Autores: | , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Clinical and Biomedical Research |
Texto Completo: | https://seer.ufrgs.br/index.php/hcpa/article/view/124760 |
Resumo: | This article presents and discusses the case of a 2-month old boy who came to Hospital de Clínicas de Porto Alegre with a history of jaundice persisting for 45 days. He presented with regular general state, no fever, important jaundice, low weight/height ratio, abdominal distention and enlargement of the liver and spleen. Abdominal ultrasonography and hepatobiliary scintigraphy were normal. The pacient stayed at HCPA for 26 days, and presented a respiratory dysfunction which was interpreted as pneumonia. He was treated and discharged in good state, and was scheduled for follow-up at the outpatient clinic. One week after discharge, he developed fever, respiratory dysfunction, bleeding, ascite, failure of multiple systems, and died. |
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Clinical and Biomedical Research |
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Nieman-Pick’s diseaseDoença de Nieman-PickThis article presents and discusses the case of a 2-month old boy who came to Hospital de Clínicas de Porto Alegre with a history of jaundice persisting for 45 days. He presented with regular general state, no fever, important jaundice, low weight/height ratio, abdominal distention and enlargement of the liver and spleen. Abdominal ultrasonography and hepatobiliary scintigraphy were normal. The pacient stayed at HCPA for 26 days, and presented a respiratory dysfunction which was interpreted as pneumonia. He was treated and discharged in good state, and was scheduled for follow-up at the outpatient clinic. One week after discharge, he developed fever, respiratory dysfunction, bleeding, ascite, failure of multiple systems, and died.O presente artigo apresenta e discute o caso de um paciente masculino, de 2 meses de idade, que foi encaminhado para o Hospital de Clínicas de Porto Alegre com história de icterícia persistente há 45 dias, apresentando-se em regular estado geral, afebril, com icterícia importante, desproporção peso/altura, abdômen distendido e fígado e baço palpáveis. Ecografia abdominal e cintilografia das vias biliares eram normais. O paciente permaneceu 26 dias no hospital para investigação, onde apresentou quadro respiratório diagnosticado como pneumonia, recebendo alta hospitalar em bom estado. Foi encaminhado para o ambulatório para prosseguir a investigação. Uma semana após a alta, apresentou febre, disfunção respiratória, sangramento difuso, ascite e falência de múltiplos órgãos, evoluindo para o óbito.HCPA/FAMED/UFRGS2022-05-25info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://seer.ufrgs.br/index.php/hcpa/article/view/124760Clinical & Biomedical Research; Vol. 18 No. 2 (1998): Revista HCPAClinical and Biomedical Research; v. 18 n. 2 (1998): Revista HCPA2357-9730reponame:Clinical and Biomedical Researchinstname:Universidade Federal do Rio Grande do Sul (UFRGS)instacron:UFRGSporhttps://seer.ufrgs.br/index.php/hcpa/article/view/124760/84967http://creativecommons.org/licenses/by/4.0info:eu-repo/semantics/openAccessSilva de Athayde Bohrer, Mauro Meurer, Luise Varela Gonzaga, RafaelRasia de Mello, Letícia M. Gleisner, Maria Luiza 2022-09-16T16:32:21Zoai:seer.ufrgs.br:article/124760Revistahttps://www.seer.ufrgs.br/index.php/hcpaPUBhttps://seer.ufrgs.br/index.php/hcpa/oai||cbr@hcpa.edu.br2357-97302357-9730opendoar:2022-09-16T16:32:21Clinical and Biomedical Research - Universidade Federal do Rio Grande do Sul (UFRGS)false |
dc.title.none.fl_str_mv |
Nieman-Pick’s disease Doença de Nieman-Pick |
title |
Nieman-Pick’s disease |
spellingShingle |
Nieman-Pick’s disease Silva de Athayde Bohrer, Mauro |
title_short |
Nieman-Pick’s disease |
title_full |
Nieman-Pick’s disease |
title_fullStr |
Nieman-Pick’s disease |
title_full_unstemmed |
Nieman-Pick’s disease |
title_sort |
Nieman-Pick’s disease |
author |
Silva de Athayde Bohrer, Mauro |
author_facet |
Silva de Athayde Bohrer, Mauro Meurer, Luise Varela Gonzaga, Rafael Rasia de Mello, Letícia M. Gleisner, Maria Luiza |
author_role |
author |
author2 |
Meurer, Luise Varela Gonzaga, Rafael Rasia de Mello, Letícia M. Gleisner, Maria Luiza |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Silva de Athayde Bohrer, Mauro Meurer, Luise Varela Gonzaga, Rafael Rasia de Mello, Letícia M. Gleisner, Maria Luiza |
description |
This article presents and discusses the case of a 2-month old boy who came to Hospital de Clínicas de Porto Alegre with a history of jaundice persisting for 45 days. He presented with regular general state, no fever, important jaundice, low weight/height ratio, abdominal distention and enlargement of the liver and spleen. Abdominal ultrasonography and hepatobiliary scintigraphy were normal. The pacient stayed at HCPA for 26 days, and presented a respiratory dysfunction which was interpreted as pneumonia. He was treated and discharged in good state, and was scheduled for follow-up at the outpatient clinic. One week after discharge, he developed fever, respiratory dysfunction, bleeding, ascite, failure of multiple systems, and died. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-05-25 |
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://seer.ufrgs.br/index.php/hcpa/article/view/124760 |
url |
https://seer.ufrgs.br/index.php/hcpa/article/view/124760 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://seer.ufrgs.br/index.php/hcpa/article/view/124760/84967 |
dc.rights.driver.fl_str_mv |
http://creativecommons.org/licenses/by/4.0 info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
http://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 |
HCPA/FAMED/UFRGS |
publisher.none.fl_str_mv |
HCPA/FAMED/UFRGS |
dc.source.none.fl_str_mv |
Clinical & Biomedical Research; Vol. 18 No. 2 (1998): Revista HCPA Clinical and Biomedical Research; v. 18 n. 2 (1998): Revista HCPA 2357-9730 reponame:Clinical and Biomedical Research instname:Universidade Federal do Rio Grande do Sul (UFRGS) instacron:UFRGS |
instname_str |
Universidade Federal do Rio Grande do Sul (UFRGS) |
instacron_str |
UFRGS |
institution |
UFRGS |
reponame_str |
Clinical and Biomedical Research |
collection |
Clinical and Biomedical Research |
repository.name.fl_str_mv |
Clinical and Biomedical Research - Universidade Federal do Rio Grande do Sul (UFRGS) |
repository.mail.fl_str_mv |
||cbr@hcpa.edu.br |
_version_ |
1799767056790323200 |