Systemic congenital lymphangiomatosis
Autor(a) principal: | |
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Data de Publicação: | 1996 |
Outros Autores: | , , |
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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São Paulo medical journal (Online) |
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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 |
_version_ |
1754209259542806528 |