Systemic congenital lymphangiomatosis

Detalhes bibliográficos
Autor(a) principal: Souza,Ligia Maria Suppo de
Data de Publicação: 1996
Outros Autores: Bentlin,Maria Regina, Abreu,Eliana Souto de, Bacchi,Carlos Eduardo
Tipo de documento: Relatório
Idioma: eng
Título da fonte: São Paulo medical journal (Online)
Texto Completo: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801996000500008
Resumo: Systemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediately after delivery. Death occurred during the first hour of life. Autopsy findings showed numerous cysts in soft tissues of the cervical area, mediastinum and diaphragm, and several other organs including the liver, spleen, thyroid and kidneys. The severe and diffuse involvement with cysts in both lungs by lymphangiomatosis was associated with poor prognosis and death in our case.
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spelling Systemic congenital lymphangiomatosisLymphangiomatosisLymphangiomaCongenital lymphangiomatosisSystemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediately after delivery. Death occurred during the first hour of life. Autopsy findings showed numerous cysts in soft tissues of the cervical area, mediastinum and diaphragm, and several other organs including the liver, spleen, thyroid and kidneys. The severe and diffuse involvement with cysts in both lungs by lymphangiomatosis was associated with poor prognosis and death in our case.Associação Paulista de Medicina - APM1996-10-01info:eu-repo/semantics/reportinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801996000500008Sao Paulo Medical Journal v.114 n.5 1996reponame:São Paulo medical journal (Online)instname:Associação Paulista de Medicinainstacron:APM10.1590/S1516-31801996000500008info:eu-repo/semantics/openAccessSouza,Ligia Maria Suppo deBentlin,Maria ReginaAbreu,Eliana Souto deBacchi,Carlos Eduardoeng2008-11-07T00:00:00Zoai:scielo:S1516-31801996000500008Revistahttp://www.scielo.br/spmjhttps://old.scielo.br/oai/scielo-oai.phprevistas@apm.org.br1806-94601516-3180opendoar:2008-11-07T00:00São Paulo medical journal (Online) - Associação Paulista de Medicinafalse
dc.title.none.fl_str_mv Systemic congenital lymphangiomatosis
title Systemic congenital lymphangiomatosis
spellingShingle Systemic congenital lymphangiomatosis
Souza,Ligia Maria Suppo de
Lymphangiomatosis
Lymphangioma
Congenital lymphangiomatosis
title_short Systemic congenital lymphangiomatosis
title_full Systemic congenital lymphangiomatosis
title_fullStr Systemic congenital lymphangiomatosis
title_full_unstemmed Systemic congenital lymphangiomatosis
title_sort Systemic congenital lymphangiomatosis
author Souza,Ligia Maria Suppo de
author_facet Souza,Ligia Maria Suppo de
Bentlin,Maria Regina
Abreu,Eliana Souto de
Bacchi,Carlos Eduardo
author_role author
author2 Bentlin,Maria Regina
Abreu,Eliana Souto de
Bacchi,Carlos Eduardo
author2_role author
author
author
dc.contributor.author.fl_str_mv Souza,Ligia Maria Suppo de
Bentlin,Maria Regina
Abreu,Eliana Souto de
Bacchi,Carlos Eduardo
dc.subject.por.fl_str_mv Lymphangiomatosis
Lymphangioma
Congenital lymphangiomatosis
topic Lymphangiomatosis
Lymphangioma
Congenital lymphangiomatosis
description Systemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediately after delivery. Death occurred during the first hour of life. Autopsy findings showed numerous cysts in soft tissues of the cervical area, mediastinum and diaphragm, and several other organs including the liver, spleen, thyroid and kidneys. The severe and diffuse involvement with cysts in both lungs by lymphangiomatosis was associated with poor prognosis and death in our case.
publishDate 1996
dc.date.none.fl_str_mv 1996-10-01
dc.type.driver.fl_str_mv info:eu-repo/semantics/report
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
format report
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801996000500008
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1516-31801996000500008
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 10.1590/S1516-31801996000500008
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv text/html
dc.publisher.none.fl_str_mv Associação Paulista de Medicina - APM
publisher.none.fl_str_mv Associação Paulista de Medicina - APM
dc.source.none.fl_str_mv Sao Paulo Medical Journal v.114 n.5 1996
reponame:São Paulo medical journal (Online)
instname:Associação Paulista de Medicina
instacron:APM
instname_str Associação Paulista de Medicina
instacron_str APM
institution APM
reponame_str São Paulo medical journal (Online)
collection São Paulo medical journal (Online)
repository.name.fl_str_mv São Paulo medical journal (Online) - Associação Paulista de Medicina
repository.mail.fl_str_mv revistas@apm.org.br
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