Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan
Autor(a) principal: | |
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Data de Publicação: | 2011 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Institucional da UNIFESP |
Texto Completo: | http://dx.doi.org/10.1590/S1413-35552011000400006 http://repositorio.unifesp.br/handle/11600/6546 |
Resumo: | BACKGROUND: Marfan syndrome (MS) is an autosomic dominant condition of the connective tissue that involves the ocular, cardiovascular and musculoskeletal systems. MS is caused by mutations in the fibrillin-1 gene, leading to joint ligaments flaccidity, joint hypermobility and an overgrowth of the long bones. OBJECTIVES: The aim of the present study was to assess anthropometry, musculoskeletal alterations and the prevalence of physical therapy treatments among patients with MS. METHODS: Twenty-six patients were included in this study [17 females (age: 13.23±2.77 years; body mass 51.5±24-68 Kg; height 1.70±1.40-1.81 m; arm span: 1.73±0.12 m) and 9 males (age: 14.44±2.18; body mass: 61.0±42-72 Kg; height: 1.83±1.66-1.97 m; arm span: 1.93±0.13 m)]. Anthropometric measurements and musculoskeletal abnormalities were determined in a standardized fashion: pectus and scoliosis were assessed through radiography and angulation (â) of the scoliosis curve using the Cobb method; arachnodactyly was assessed through the thumb sign and Walker-Murdoch test and dolichostenomelia was assessed by arm span in relation to height. Patients also responded to a questionnaire addressing participation in physical therapy. RESULTS: In comparison to values estimated for the Brazilian population, mass and height were greater among the patients with MS (females: p=0.001 e p<0.0005 e males p=0.019 e p=0.0001, respectively). The following musculoskeletal abnormalities were found: pectus in 3 patients (11%), pectus and scoliosis in 19 (73%), dolichostenomelia in 11 (42%) and arachnodactyly in 21 (80%). Eleven patients (42%) with MS had previously undergone physical therapy. CONCLUSIONS: Patients with MS exhibit altered musculoskeleto and anthropometry and have infrequent physical therapy treatment. |
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Avaliação antropométrica e musculoesquelética de pacientes com síndrome de MarfanAnthropometric and musculoskeletal assessment of patients with Marfan syndromeMarfan Syndromeanthropometrymusculoskeletal systemconnective tissuephysical therapySíndrome de Marfanantropometriasistema musculoesqueléticotecido conjuntivofisioterapiaBACKGROUND: Marfan syndrome (MS) is an autosomic dominant condition of the connective tissue that involves the ocular, cardiovascular and musculoskeletal systems. MS is caused by mutations in the fibrillin-1 gene, leading to joint ligaments flaccidity, joint hypermobility and an overgrowth of the long bones. OBJECTIVES: The aim of the present study was to assess anthropometry, musculoskeletal alterations and the prevalence of physical therapy treatments among patients with MS. METHODS: Twenty-six patients were included in this study [17 females (age: 13.23±2.77 years; body mass 51.5±24-68 Kg; height 1.70±1.40-1.81 m; arm span: 1.73±0.12 m) and 9 males (age: 14.44±2.18; body mass: 61.0±42-72 Kg; height: 1.83±1.66-1.97 m; arm span: 1.93±0.13 m)]. Anthropometric measurements and musculoskeletal abnormalities were determined in a standardized fashion: pectus and scoliosis were assessed through radiography and angulation (â) of the scoliosis curve using the Cobb method; arachnodactyly was assessed through the thumb sign and Walker-Murdoch test and dolichostenomelia was assessed by arm span in relation to height. Patients also responded to a questionnaire addressing participation in physical therapy. RESULTS: In comparison to values estimated for the Brazilian population, mass and height were greater among the patients with MS (females: p=0.001 e p<0.0005 e males p=0.019 e p=0.0001, respectively). The following musculoskeletal abnormalities were found: pectus in 3 patients (11%), pectus and scoliosis in 19 (73%), dolichostenomelia in 11 (42%) and arachnodactyly in 21 (80%). Eleven patients (42%) with MS had previously undergone physical therapy. CONCLUSIONS: Patients with MS exhibit altered musculoskeleto and anthropometry and have infrequent physical therapy treatment.CONTEXTUALIZAÇÃO: A Síndrome de Marfan (SM) é uma doença autossômica dominante do tecido conjuntivo que envolve os sistemas ocular, cardiovascular e musculoesquelético, causada por mutações no gene da fibrilina1, gerando flacidez nos ligamentos articulares, favorecendo a hipermobilidade articular e redução na contenção do crescimento ósseo. OBJETIVOS: Avaliar as medidas antropométricas, alterações musculoesqueléticas e a frequência do tratamento fisioterapêutico nos pacientes com SM. MÉTODOS: Participaram deste estudo 26 pacientes, sendo 17 do gênero feminino, com idade de 13,23±2,77 anos, massa corpórea de 51,5±24-68 Kg, altura de 1,70±1,40-1,81 m e envergadura de 1,73±0,12 cm, e nove do gênero masculino, com idade de 14,44±2,18, massa corpórea de 61,0±42-72 Kg, altura de 1,83±1,66-1,97 m e envergadura de 1,93±0,13. Foram obtidas medidas antropométricas, alterações ME de forma padronizada, sendo o pectus e a escoliose, por avaliação radiológica, e a angulação (â) da curva escoliótica, pelo método de Cobb; a aracnodactilia, pelo sinal do polegar e teste de Walker-Murdoch, e a dolicostenomelia, pela envergadura em relação à altura. Os pacientes responderam a um questionário quanto à participação em tratamento de fisioterapia. RESULTADOS: Quando comparados com a estimativa brasileira, a massa corpórea e a altura apresentaram valores maiores no gênero feminino (p=0,001 e p<0,0005) e masculino (p=0,019 e p=0,0001). Das alterações musculoesqueléticas, encontrou-se pectus em 3 (11%), pectus e escoliose em 19 (73%), dolicostenomelia em 11 (42%) e aracnodactilia em 21(80%). Onze (42%) pacientes com SM já haviam realizado tratamento de fisioterapia. CONCLUSÕES: As alterações antropométricas e musculoesqueléticas estão presentes na SM, e o tratamento fisioterapêutico é pouco frequente.Universidade Federal de São Paulo (UNIFESP) Departamento de MedicinaUniversidade de São Paulo Faculdade de Medicina Hospital das ClínicasUniversidade de Brasília Faculdade Ceilândia Curso de FisioterapiaUNIFESP, Depto. de MedicinaSciELOCoordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Associação Brasileira de Pesquisa e Pós-Graduação em FisioterapiaUniversidade Federal de São Paulo (UNIFESP)Universidade de São Paulo (USP)Universidade de Brasília Faculdade Ceilândia Curso de FisioterapiaCipriano, Graziella França Bernardelli [UNIFESP]Brech, Guilherme CarlosPeres, Paulo Alberto Tayar [UNIFESP]Mendes, Cássia Claudino [UNIFESP]Cipriano Junior, Gerson [UNIFESP]Carvalho, Antonio Carlos [UNIFESP]2015-06-14T13:43:11Z2015-06-14T13:43:11Z2011-08-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion291-296application/pdfapplication/pdfhttp://dx.doi.org/10.1590/S1413-35552011000400006CIPRIANO, Graziella França Bernardelli et al . Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan. Rev. bras. fisioter., São Carlos , v. 15, n. 4, p. 291-296, ago. 201110.1590/S1413-35552011000400006S1413-35552011000400006.pdfS1413-35552011000400006-pt.pdf1413-3555S1413-35552011000400006http://repositorio.unifesp.br/handle/11600/6546WOS:000295237800006porBrazilian Journal of Physical Therapyinfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UNIFESPinstname:Universidade Federal de São Paulo (UNIFESP)instacron:UNIFESP2024-08-06T04:55:18Zoai:repositorio.unifesp.br/:11600/6546Repositório InstitucionalPUBhttp://www.repositorio.unifesp.br/oai/requestbiblioteca.csp@unifesp.bropendoar:34652024-08-06T04:55:18Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP)false |
dc.title.none.fl_str_mv |
Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan Anthropometric and musculoskeletal assessment of patients with Marfan syndrome |
title |
Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan |
spellingShingle |
Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan Cipriano, Graziella França Bernardelli [UNIFESP] Marfan Syndrome anthropometry musculoskeletal system connective tissue physical therapy Síndrome de Marfan antropometria sistema musculoesquelético tecido conjuntivo fisioterapia |
title_short |
Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan |
title_full |
Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan |
title_fullStr |
Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan |
title_full_unstemmed |
Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan |
title_sort |
Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan |
author |
Cipriano, Graziella França Bernardelli [UNIFESP] |
author_facet |
Cipriano, Graziella França Bernardelli [UNIFESP] Brech, Guilherme Carlos Peres, Paulo Alberto Tayar [UNIFESP] Mendes, Cássia Claudino [UNIFESP] Cipriano Junior, Gerson [UNIFESP] Carvalho, Antonio Carlos [UNIFESP] |
author_role |
author |
author2 |
Brech, Guilherme Carlos Peres, Paulo Alberto Tayar [UNIFESP] Mendes, Cássia Claudino [UNIFESP] Cipriano Junior, Gerson [UNIFESP] Carvalho, Antonio Carlos [UNIFESP] |
author2_role |
author author author author author |
dc.contributor.none.fl_str_mv |
Universidade Federal de São Paulo (UNIFESP) Universidade de São Paulo (USP) Universidade de Brasília Faculdade Ceilândia Curso de Fisioterapia |
dc.contributor.author.fl_str_mv |
Cipriano, Graziella França Bernardelli [UNIFESP] Brech, Guilherme Carlos Peres, Paulo Alberto Tayar [UNIFESP] Mendes, Cássia Claudino [UNIFESP] Cipriano Junior, Gerson [UNIFESP] Carvalho, Antonio Carlos [UNIFESP] |
dc.subject.por.fl_str_mv |
Marfan Syndrome anthropometry musculoskeletal system connective tissue physical therapy Síndrome de Marfan antropometria sistema musculoesquelético tecido conjuntivo fisioterapia |
topic |
Marfan Syndrome anthropometry musculoskeletal system connective tissue physical therapy Síndrome de Marfan antropometria sistema musculoesquelético tecido conjuntivo fisioterapia |
description |
BACKGROUND: Marfan syndrome (MS) is an autosomic dominant condition of the connective tissue that involves the ocular, cardiovascular and musculoskeletal systems. MS is caused by mutations in the fibrillin-1 gene, leading to joint ligaments flaccidity, joint hypermobility and an overgrowth of the long bones. OBJECTIVES: The aim of the present study was to assess anthropometry, musculoskeletal alterations and the prevalence of physical therapy treatments among patients with MS. METHODS: Twenty-six patients were included in this study [17 females (age: 13.23±2.77 years; body mass 51.5±24-68 Kg; height 1.70±1.40-1.81 m; arm span: 1.73±0.12 m) and 9 males (age: 14.44±2.18; body mass: 61.0±42-72 Kg; height: 1.83±1.66-1.97 m; arm span: 1.93±0.13 m)]. Anthropometric measurements and musculoskeletal abnormalities were determined in a standardized fashion: pectus and scoliosis were assessed through radiography and angulation (â) of the scoliosis curve using the Cobb method; arachnodactyly was assessed through the thumb sign and Walker-Murdoch test and dolichostenomelia was assessed by arm span in relation to height. Patients also responded to a questionnaire addressing participation in physical therapy. RESULTS: In comparison to values estimated for the Brazilian population, mass and height were greater among the patients with MS (females: p=0.001 e p<0.0005 e males p=0.019 e p=0.0001, respectively). The following musculoskeletal abnormalities were found: pectus in 3 patients (11%), pectus and scoliosis in 19 (73%), dolichostenomelia in 11 (42%) and arachnodactyly in 21 (80%). Eleven patients (42%) with MS had previously undergone physical therapy. CONCLUSIONS: Patients with MS exhibit altered musculoskeleto and anthropometry and have infrequent physical therapy treatment. |
publishDate |
2011 |
dc.date.none.fl_str_mv |
2011-08-01 2015-06-14T13:43:11Z 2015-06-14T13:43:11Z |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://dx.doi.org/10.1590/S1413-35552011000400006 CIPRIANO, Graziella França Bernardelli et al . Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan. Rev. bras. fisioter., São Carlos , v. 15, n. 4, p. 291-296, ago. 2011 10.1590/S1413-35552011000400006 S1413-35552011000400006.pdf S1413-35552011000400006-pt.pdf 1413-3555 S1413-35552011000400006 http://repositorio.unifesp.br/handle/11600/6546 WOS:000295237800006 |
url |
http://dx.doi.org/10.1590/S1413-35552011000400006 http://repositorio.unifesp.br/handle/11600/6546 |
identifier_str_mv |
CIPRIANO, Graziella França Bernardelli et al . Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan. Rev. bras. fisioter., São Carlos , v. 15, n. 4, p. 291-296, ago. 2011 10.1590/S1413-35552011000400006 S1413-35552011000400006.pdf S1413-35552011000400006-pt.pdf 1413-3555 S1413-35552011000400006 WOS:000295237800006 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
Brazilian Journal of Physical Therapy |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
291-296 application/pdf application/pdf |
dc.publisher.none.fl_str_mv |
Associação Brasileira de Pesquisa e Pós-Graduação em Fisioterapia |
publisher.none.fl_str_mv |
Associação Brasileira de Pesquisa e Pós-Graduação em Fisioterapia |
dc.source.none.fl_str_mv |
reponame:Repositório Institucional da UNIFESP instname:Universidade Federal de São Paulo (UNIFESP) instacron:UNIFESP |
instname_str |
Universidade Federal de São Paulo (UNIFESP) |
instacron_str |
UNIFESP |
institution |
UNIFESP |
reponame_str |
Repositório Institucional da UNIFESP |
collection |
Repositório Institucional da UNIFESP |
repository.name.fl_str_mv |
Repositório Institucional da UNIFESP - Universidade Federal de São Paulo (UNIFESP) |
repository.mail.fl_str_mv |
biblioteca.csp@unifesp.br |
_version_ |
1814268342571106304 |