Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age.
Autor(a) principal: | |
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Data de Publicação: | 2004 |
Outros Autores: | , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121 |
Resumo: | Peutz-Jeghers syndrome is a rare autosomal dominant condition, characterized by gastrointestinal polyposis, mucocutaneous pigmentation and high risk of neoplasia in multiple organs. At pediatric age, major clinical impact is related to complications associated to intestinal polyps, but neoplasic risk isn't negligible. Though clinical surveillance is recommended since the age of 10 years, relevant lesions may occur before that age. Conventional radiology and endoscopy have recognized limitations at this age group, and new diagnostic and intervention tools, such as video-capsule and per-operative enteroscopy, are not yet widely used. We present 5 pediatric cases (age under 10 years) with diversity of gastrointestinal expression (including one case with histologic evidence of dysplasia in a large colonic polyp), emphasizing the need of specific guidelines concerning young children. |
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Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age.Sindrome de Peutz-Jeghers. Diversidade de expressão gastrointestinal em idade pediátrica e considerações sobre a sua abordagem clínica.Peutz-Jeghers syndrome is a rare autosomal dominant condition, characterized by gastrointestinal polyposis, mucocutaneous pigmentation and high risk of neoplasia in multiple organs. At pediatric age, major clinical impact is related to complications associated to intestinal polyps, but neoplasic risk isn't negligible. Though clinical surveillance is recommended since the age of 10 years, relevant lesions may occur before that age. Conventional radiology and endoscopy have recognized limitations at this age group, and new diagnostic and intervention tools, such as video-capsule and per-operative enteroscopy, are not yet widely used. We present 5 pediatric cases (age under 10 years) with diversity of gastrointestinal expression (including one case with histologic evidence of dysplasia in a large colonic polyp), emphasizing the need of specific guidelines concerning young children.Peutz-Jeghers syndrome is a rare autosomal dominant condition, characterized by gastrointestinal polyposis, mucocutaneous pigmentation and high risk of neoplasia in multiple organs. At pediatric age, major clinical impact is related to complications associated to intestinal polyps, but neoplasic risk isn't negligible. Though clinical surveillance is recommended since the age of 10 years, relevant lesions may occur before that age. Conventional radiology and endoscopy have recognized limitations at this age group, and new diagnostic and intervention tools, such as video-capsule and per-operative enteroscopy, are not yet widely used. We present 5 pediatric cases (age under 10 years) with diversity of gastrointestinal expression (including one case with histologic evidence of dysplasia in a large colonic polyp), emphasizing the need of specific guidelines concerning young children.Ordem dos Médicos2004-12-31info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121oai:ojs.www.actamedicaportuguesa.com:article/1121Acta Médica Portuguesa; Vol. 17 No. 6 (2004): November-December; 445-450Acta Médica Portuguesa; Vol. 17 N.º 6 (2004): Novembro-Dezembro; 445-4501646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121/786Lopes, Ana IGonçalves, JuanPalha, Ana MFurtado, FátimaMarques, Antónioinfo:eu-repo/semantics/openAccess2022-12-20T10:57:29Zoai:ojs.www.actamedicaportuguesa.com:article/1121Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:16:58.891179Repositório 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 |
Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age. Sindrome de Peutz-Jeghers. Diversidade de expressão gastrointestinal em idade pediátrica e considerações sobre a sua abordagem clínica. |
title |
Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age. |
spellingShingle |
Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age. Lopes, Ana I |
title_short |
Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age. |
title_full |
Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age. |
title_fullStr |
Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age. |
title_full_unstemmed |
Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age. |
title_sort |
Peutz-Jeghers syndrome: variability of gastrointestinal expression at pediatric age. |
author |
Lopes, Ana I |
author_facet |
Lopes, Ana I Gonçalves, Juan Palha, Ana M Furtado, Fátima Marques, António |
author_role |
author |
author2 |
Gonçalves, Juan Palha, Ana M Furtado, Fátima Marques, António |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Lopes, Ana I Gonçalves, Juan Palha, Ana M Furtado, Fátima Marques, António |
description |
Peutz-Jeghers syndrome is a rare autosomal dominant condition, characterized by gastrointestinal polyposis, mucocutaneous pigmentation and high risk of neoplasia in multiple organs. At pediatric age, major clinical impact is related to complications associated to intestinal polyps, but neoplasic risk isn't negligible. Though clinical surveillance is recommended since the age of 10 years, relevant lesions may occur before that age. Conventional radiology and endoscopy have recognized limitations at this age group, and new diagnostic and intervention tools, such as video-capsule and per-operative enteroscopy, are not yet widely used. We present 5 pediatric cases (age under 10 years) with diversity of gastrointestinal expression (including one case with histologic evidence of dysplasia in a large colonic polyp), emphasizing the need of specific guidelines concerning young children. |
publishDate |
2004 |
dc.date.none.fl_str_mv |
2004-12-31 |
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 |
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121 oai:ojs.www.actamedicaportuguesa.com:article/1121 |
url |
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121 |
identifier_str_mv |
oai:ojs.www.actamedicaportuguesa.com:article/1121 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121 https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121/786 |
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info:eu-repo/semantics/openAccess |
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openAccess |
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application/pdf |
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Ordem dos Médicos |
publisher.none.fl_str_mv |
Ordem dos Médicos |
dc.source.none.fl_str_mv |
Acta Médica Portuguesa; Vol. 17 No. 6 (2004): November-December; 445-450 Acta Médica Portuguesa; Vol. 17 N.º 6 (2004): Novembro-Dezembro; 445-450 1646-0758 0870-399X reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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