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 ris...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Ordem dos Médicos
2004-12-01
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| Series: | Acta Médica Portuguesa |
| Online Access: | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121 |
| _version_ | 1798021062815907840 |
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| author | Ana I Lopes Juan Gonçalves Ana M Palha Fátima Furtado António Marques |
| author_facet | Ana I Lopes Juan Gonçalves Ana M Palha Fátima Furtado António Marques |
| author_sort | Ana I Lopes |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-04-11T17:07:56Z |
| format | Article |
| id | doaj.art-5b730e98ba4e418ab89907ab6f1a4f0a |
| institution | Directory Open Access Journal |
| issn | 0870-399X 1646-0758 |
| language | English |
| last_indexed | 2024-04-11T17:07:56Z |
| publishDate | 2004-12-01 |
| publisher | Ordem dos Médicos |
| record_format | Article |
| series | Acta Médica Portuguesa |
| spelling | doaj.art-5b730e98ba4e418ab89907ab6f1a4f0a2022-12-22T04:12:59ZengOrdem dos MédicosActa Médica Portuguesa0870-399X1646-07582004-12-0117610.20344/amp.1121Sindrome de Peutz-Jeghers. Diversidade de expressão gastrointestinal em idade pediátrica e considerações sobre a sua abordagem clínica.Ana I Lopes0Juan GonçalvesAna M PalhaFátima FurtadoAntónio MarquesServiço de Pediatria, Hospital de Santa Maria, Lisboa.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.https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121 |
| spellingShingle | Ana I Lopes Juan Gonçalves Ana M Palha Fátima Furtado António Marques Sindrome de Peutz-Jeghers. Diversidade de expressão gastrointestinal em idade pediátrica e considerações sobre a sua abordagem clínica. Acta Médica Portuguesa |
| title | Sindrome de Peutz-Jeghers. Diversidade de expressão gastrointestinal em idade pediátrica e considerações sobre a sua abordagem clínica. |
| title_full | Sindrome de Peutz-Jeghers. Diversidade de expressão gastrointestinal em idade pediátrica e considerações sobre a sua abordagem clínica. |
| title_fullStr | Sindrome de Peutz-Jeghers. Diversidade de expressão gastrointestinal em idade pediátrica e considerações sobre a sua abordagem clínica. |
| title_full_unstemmed | Sindrome de Peutz-Jeghers. Diversidade de expressão gastrointestinal em idade pediátrica e considerações sobre a sua abordagem clínica. |
| title_short | Sindrome de Peutz-Jeghers. Diversidade de expressão gastrointestinal em idade pediátrica e considerações sobre a sua abordagem clínica. |
| title_sort | sindrome de peutz jeghers diversidade de expressao gastrointestinal em idade pediatrica e consideracoes sobre a sua abordagem clinica |
| url | https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1121 |
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