Avaliação antropométrica e musculoesquelética de pacientes com síndrome de Marfan

Detalhes bibliográficos
Autor(a) principal: Cipriano, Graziella França Bernardelli [UNIFESP]
Data de Publicação: 2011
Outros Autores: Brech, Guilherme Carlos, Peres, Paulo Alberto Tayar [UNIFESP], Mendes, Cássia Claudino [UNIFESP], Cipriano Junior, Gerson [UNIFESP], Carvalho, Antonio Carlos [UNIFESP]
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.
id UFSP_39f26639387c0789bbd6f54811a6df54
oai_identifier_str oai:repositorio.unifesp.br/:11600/6546
network_acronym_str UFSP
network_name_str Repositório Institucional da UNIFESP
repository_id_str 3465
spelling 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