Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus
Autor(a) principal: | |
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Data de Publicação: | 2015 |
Outros Autores: | , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UNESP |
Texto Completo: | http://dx.doi.org/10.1590/1677-5449.005615 http://hdl.handle.net/11449/172310 |
Resumo: | Proteus syndrome is a rare combination of malformations that can affect several tissues and organs. It is characterized by bilateral macrodactyly, cranial hypertrophy, bone anomalies, scoliosis, soft-tissue hamartomas, verrucous pigmented nevus, visceral abnormalities and other forms of hypertrophy. Just over 200 cases have been reported worldwide. This article reports on the clinical course of a pediatric patient with this syndrome. The child had severe malnutrition associated with extreme gigantism of the lower limbs and also psychosocial problems related to social exclusion. As the disease progressed it exacerbated and evolved into a wasting syndrome. After several years, the parents agreed to amputation of the hypertrophic lower limbs. One year after the amputations the child had been rehabilitated and had adapted to prostheses, with nutritional improvement and notable psychological recovery and social reintegration, which represented a significant improvement in his quality of life. |
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Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de ProteusClinical and psychosocial challenges in the treatment of a patient with proteus syndromeAmputationCongenital abnormalitiesMusculoskeletal abnormalitiesProteus syndrome is a rare combination of malformations that can affect several tissues and organs. It is characterized by bilateral macrodactyly, cranial hypertrophy, bone anomalies, scoliosis, soft-tissue hamartomas, verrucous pigmented nevus, visceral abnormalities and other forms of hypertrophy. Just over 200 cases have been reported worldwide. This article reports on the clinical course of a pediatric patient with this syndrome. The child had severe malnutrition associated with extreme gigantism of the lower limbs and also psychosocial problems related to social exclusion. As the disease progressed it exacerbated and evolved into a wasting syndrome. After several years, the parents agreed to amputation of the hypertrophic lower limbs. One year after the amputations the child had been rehabilitated and had adapted to prostheses, with nutritional improvement and notable psychological recovery and social reintegration, which represented a significant improvement in his quality of life.Universidade Estadual Paulista – UNESP Department of Surgery and OrthopedicsUniversidade Estadual Paulista – UNESP Department of PediatricsUniversidade Estadual Paulista – UNESP Department of Surgery and OrthopedicsUniversidade Estadual Paulista – UNESP Department of PediatricsUniversidade Estadual Paulista (Unesp)Bertanha, Matheus [UNESP]Moura, Regina [UNESP]Sobreira, Marcone Lima [UNESP]Pereira, Lied Martins Santiago [UNESP]Jaldin, Rodrigo Gibin [UNESP]Segredo, Manuella Pacífico De Freitas [UNESP]Rollo, Hamilton Almeida [UNESP]Yoshida, Winston Bonetti [UNESP]2018-12-11T16:59:38Z2018-12-11T16:59:38Z2015-10-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article346-350application/pdfhttp://dx.doi.org/10.1590/1677-5449.005615Jornal Vascular Brasileiro, v. 14, n. 4, p. 346-350, 2015.1677-73011677-5449http://hdl.handle.net/11449/17231010.1590/1677-5449.005615S1677-544920150004003462-s2.0-84949963308S1677-54492015000400346.pdf96093248325913824513014379461383Scopusreponame:Repositório Institucional da UNESPinstname:Universidade Estadual Paulista (UNESP)instacron:UNESPengJornal Vascular Brasileiro0,1360,136info:eu-repo/semantics/openAccess2024-08-14T14:19:43Zoai:repositorio.unesp.br:11449/172310Repositório InstitucionalPUBhttp://repositorio.unesp.br/oai/requestopendoar:29462024-08-14T14:19:43Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP)false |
dc.title.none.fl_str_mv |
Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus Clinical and psychosocial challenges in the treatment of a patient with proteus syndrome |
title |
Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus |
spellingShingle |
Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus Bertanha, Matheus [UNESP] Amputation Congenital abnormalities Musculoskeletal abnormalities |
title_short |
Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus |
title_full |
Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus |
title_fullStr |
Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus |
title_full_unstemmed |
Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus |
title_sort |
Desafios clínicos e psicossociais no tratamento de um paciente com síndrome de Proteus |
author |
Bertanha, Matheus [UNESP] |
author_facet |
Bertanha, Matheus [UNESP] Moura, Regina [UNESP] Sobreira, Marcone Lima [UNESP] Pereira, Lied Martins Santiago [UNESP] Jaldin, Rodrigo Gibin [UNESP] Segredo, Manuella Pacífico De Freitas [UNESP] Rollo, Hamilton Almeida [UNESP] Yoshida, Winston Bonetti [UNESP] |
author_role |
author |
author2 |
Moura, Regina [UNESP] Sobreira, Marcone Lima [UNESP] Pereira, Lied Martins Santiago [UNESP] Jaldin, Rodrigo Gibin [UNESP] Segredo, Manuella Pacífico De Freitas [UNESP] Rollo, Hamilton Almeida [UNESP] Yoshida, Winston Bonetti [UNESP] |
author2_role |
author author author author author author author |
dc.contributor.none.fl_str_mv |
Universidade Estadual Paulista (Unesp) |
dc.contributor.author.fl_str_mv |
Bertanha, Matheus [UNESP] Moura, Regina [UNESP] Sobreira, Marcone Lima [UNESP] Pereira, Lied Martins Santiago [UNESP] Jaldin, Rodrigo Gibin [UNESP] Segredo, Manuella Pacífico De Freitas [UNESP] Rollo, Hamilton Almeida [UNESP] Yoshida, Winston Bonetti [UNESP] |
dc.subject.por.fl_str_mv |
Amputation Congenital abnormalities Musculoskeletal abnormalities |
topic |
Amputation Congenital abnormalities Musculoskeletal abnormalities |
description |
Proteus syndrome is a rare combination of malformations that can affect several tissues and organs. It is characterized by bilateral macrodactyly, cranial hypertrophy, bone anomalies, scoliosis, soft-tissue hamartomas, verrucous pigmented nevus, visceral abnormalities and other forms of hypertrophy. Just over 200 cases have been reported worldwide. This article reports on the clinical course of a pediatric patient with this syndrome. The child had severe malnutrition associated with extreme gigantism of the lower limbs and also psychosocial problems related to social exclusion. As the disease progressed it exacerbated and evolved into a wasting syndrome. After several years, the parents agreed to amputation of the hypertrophic lower limbs. One year after the amputations the child had been rehabilitated and had adapted to prostheses, with nutritional improvement and notable psychological recovery and social reintegration, which represented a significant improvement in his quality of life. |
publishDate |
2015 |
dc.date.none.fl_str_mv |
2015-10-01 2018-12-11T16:59:38Z 2018-12-11T16:59:38Z |
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 |
http://dx.doi.org/10.1590/1677-5449.005615 Jornal Vascular Brasileiro, v. 14, n. 4, p. 346-350, 2015. 1677-7301 1677-5449 http://hdl.handle.net/11449/172310 10.1590/1677-5449.005615 S1677-54492015000400346 2-s2.0-84949963308 S1677-54492015000400346.pdf 9609324832591382 4513014379461383 |
url |
http://dx.doi.org/10.1590/1677-5449.005615 http://hdl.handle.net/11449/172310 |
identifier_str_mv |
Jornal Vascular Brasileiro, v. 14, n. 4, p. 346-350, 2015. 1677-7301 1677-5449 10.1590/1677-5449.005615 S1677-54492015000400346 2-s2.0-84949963308 S1677-54492015000400346.pdf 9609324832591382 4513014379461383 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Jornal Vascular Brasileiro 0,136 0,136 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
346-350 application/pdf |
dc.source.none.fl_str_mv |
Scopus reponame:Repositório Institucional da UNESP instname:Universidade Estadual Paulista (UNESP) instacron:UNESP |
instname_str |
Universidade Estadual Paulista (UNESP) |
instacron_str |
UNESP |
institution |
UNESP |
reponame_str |
Repositório Institucional da UNESP |
collection |
Repositório Institucional da UNESP |
repository.name.fl_str_mv |
Repositório Institucional da UNESP - Universidade Estadual Paulista (UNESP) |
repository.mail.fl_str_mv |
|
_version_ |
1808128199964491776 |