Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil
Autor(a) principal: | |
---|---|
Data de Publicação: | 2018 |
Outros Autores: | , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Revista Brasileira de Saúde Materno Infantil (Online) |
Texto Completo: | http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1519-38292018000100083 |
Resumo: | Abstract Objectives: to assess the functional independence, functional capacity and respiratory muscle strength (RMS) in individuals with mucopolysaccharidosis (MPS) type VI. Methods: in this cross-sectional study, the Pediatric Evaluation of Disability Inventory (PEDI) and the Functional Independence Measure (FIM) scales were used to assess the functional independence. The functional capacity and the RMS were assessed by a 6-minute walk test (6MWT) and Manovacuometry, respectively. The associations between RMS and 6MWD were analyzed by using the adjusted simple linear regression models. And besides, the T-test was used to compare the differences among the groups. Results: twenty-four patients with MPS VI were included, the median age was 8 (ranged from 2-38 years old). The PEDI presented a functional performance below the expectations at the self-care and mobility domains of 33.3% when compared to groups of Brazilian children at the same age assessed with typical development. The decreased social function was found in only one single case. According to the FIM, of the 12 patients evaluated (age> 7.5 years), 58.3% were classified as modified dependence and 41.7% as modified independence. The mean distance in the 6MWT was significantly lower than predicted, 263m in G1 (children, n= 7) and 336m in G2 (adolescents and adults, n=6), p<0.001. The RMS was also lower than predicted in both groups, except for the RMS in G1. Conclusions: the functional capacity and the RMS were significantly reduced in individuals with MPS VI, with a decreased functional independence in one-third of the patients. However, a multidisciplinary follow-up in this population is essential to prevent, diagnose and treat early complications. |
id |
IMIPFF-1_070166317ee518d8c2bcbeb8f61ca2b8 |
---|---|
oai_identifier_str |
oai:scielo:S1519-38292018000100083 |
network_acronym_str |
IMIPFF-1 |
network_name_str |
Revista Brasileira de Saúde Materno Infantil (Online) |
repository_id_str |
|
spelling |
Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of BrazilPhysiotherapyMucopolysaccharidosis VIExercise toleranceRespiratory musclesAbstract Objectives: to assess the functional independence, functional capacity and respiratory muscle strength (RMS) in individuals with mucopolysaccharidosis (MPS) type VI. Methods: in this cross-sectional study, the Pediatric Evaluation of Disability Inventory (PEDI) and the Functional Independence Measure (FIM) scales were used to assess the functional independence. The functional capacity and the RMS were assessed by a 6-minute walk test (6MWT) and Manovacuometry, respectively. The associations between RMS and 6MWD were analyzed by using the adjusted simple linear regression models. And besides, the T-test was used to compare the differences among the groups. Results: twenty-four patients with MPS VI were included, the median age was 8 (ranged from 2-38 years old). The PEDI presented a functional performance below the expectations at the self-care and mobility domains of 33.3% when compared to groups of Brazilian children at the same age assessed with typical development. The decreased social function was found in only one single case. According to the FIM, of the 12 patients evaluated (age> 7.5 years), 58.3% were classified as modified dependence and 41.7% as modified independence. The mean distance in the 6MWT was significantly lower than predicted, 263m in G1 (children, n= 7) and 336m in G2 (adolescents and adults, n=6), p<0.001. The RMS was also lower than predicted in both groups, except for the RMS in G1. Conclusions: the functional capacity and the RMS were significantly reduced in individuals with MPS VI, with a decreased functional independence in one-third of the patients. However, a multidisciplinary follow-up in this population is essential to prevent, diagnose and treat early complications.Instituto de Medicina Integral Prof. Fernando Figueira2018-03-01info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersiontext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1519-38292018000100083Revista Brasileira de Saúde Materno Infantil v.18 n.1 2018reponame:Revista Brasileira de Saúde Materno Infantil (Online)instname:Instituto de Medicina Integral Prof. Fernando Figueira (IMIPFF)instacron:IMIPFF10.1590/1806-93042018000100004info:eu-repo/semantics/openAccessFigueirêdo,Bárbara Bernardo Rinaldo da SilvaMagalhães,Paulo André FreireAndrade,Liívia Barbosa deBezerra,PatríciaDuarte,Maria do Carmo Menezes Bezerraeng2018-10-16T00:00:00Zoai:scielo:S1519-38292018000100083Revistahttp://www.scielo.br/rbsmihttps://old.scielo.br/oai/scielo-oai.php||revista@imip.org.br1806-93041519-3829opendoar:2018-10-16T00:00Revista Brasileira de Saúde Materno Infantil (Online) - Instituto de Medicina Integral Prof. Fernando Figueira (IMIPFF)false |
dc.title.none.fl_str_mv |
Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil |
title |
Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil |
spellingShingle |
Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil Figueirêdo,Bárbara Bernardo Rinaldo da Silva Physiotherapy Mucopolysaccharidosis VI Exercise tolerance Respiratory muscles |
title_short |
Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil |
title_full |
Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil |
title_fullStr |
Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil |
title_full_unstemmed |
Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil |
title_sort |
Level of independence, functional capacity and respiratory muscle strength of patients with mucopolysaccharidosis type VI in the Northeast of Brazil |
author |
Figueirêdo,Bárbara Bernardo Rinaldo da Silva |
author_facet |
Figueirêdo,Bárbara Bernardo Rinaldo da Silva Magalhães,Paulo André Freire Andrade,Liívia Barbosa de Bezerra,Patrícia Duarte,Maria do Carmo Menezes Bezerra |
author_role |
author |
author2 |
Magalhães,Paulo André Freire Andrade,Liívia Barbosa de Bezerra,Patrícia Duarte,Maria do Carmo Menezes Bezerra |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Figueirêdo,Bárbara Bernardo Rinaldo da Silva Magalhães,Paulo André Freire Andrade,Liívia Barbosa de Bezerra,Patrícia Duarte,Maria do Carmo Menezes Bezerra |
dc.subject.por.fl_str_mv |
Physiotherapy Mucopolysaccharidosis VI Exercise tolerance Respiratory muscles |
topic |
Physiotherapy Mucopolysaccharidosis VI Exercise tolerance Respiratory muscles |
description |
Abstract Objectives: to assess the functional independence, functional capacity and respiratory muscle strength (RMS) in individuals with mucopolysaccharidosis (MPS) type VI. Methods: in this cross-sectional study, the Pediatric Evaluation of Disability Inventory (PEDI) and the Functional Independence Measure (FIM) scales were used to assess the functional independence. The functional capacity and the RMS were assessed by a 6-minute walk test (6MWT) and Manovacuometry, respectively. The associations between RMS and 6MWD were analyzed by using the adjusted simple linear regression models. And besides, the T-test was used to compare the differences among the groups. Results: twenty-four patients with MPS VI were included, the median age was 8 (ranged from 2-38 years old). The PEDI presented a functional performance below the expectations at the self-care and mobility domains of 33.3% when compared to groups of Brazilian children at the same age assessed with typical development. The decreased social function was found in only one single case. According to the FIM, of the 12 patients evaluated (age> 7.5 years), 58.3% were classified as modified dependence and 41.7% as modified independence. The mean distance in the 6MWT was significantly lower than predicted, 263m in G1 (children, n= 7) and 336m in G2 (adolescents and adults, n=6), p<0.001. The RMS was also lower than predicted in both groups, except for the RMS in G1. Conclusions: the functional capacity and the RMS were significantly reduced in individuals with MPS VI, with a decreased functional independence in one-third of the patients. However, a multidisciplinary follow-up in this population is essential to prevent, diagnose and treat early complications. |
publishDate |
2018 |
dc.date.none.fl_str_mv |
2018-03-01 |
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://old.scielo.br/scielo.php?script=sci_arttext&pid=S1519-38292018000100083 |
url |
http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1519-38292018000100083 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
10.1590/1806-93042018000100004 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Instituto de Medicina Integral Prof. Fernando Figueira |
publisher.none.fl_str_mv |
Instituto de Medicina Integral Prof. Fernando Figueira |
dc.source.none.fl_str_mv |
Revista Brasileira de Saúde Materno Infantil v.18 n.1 2018 reponame:Revista Brasileira de Saúde Materno Infantil (Online) instname:Instituto de Medicina Integral Prof. Fernando Figueira (IMIPFF) instacron:IMIPFF |
instname_str |
Instituto de Medicina Integral Prof. Fernando Figueira (IMIPFF) |
instacron_str |
IMIPFF |
institution |
IMIPFF |
reponame_str |
Revista Brasileira de Saúde Materno Infantil (Online) |
collection |
Revista Brasileira de Saúde Materno Infantil (Online) |
repository.name.fl_str_mv |
Revista Brasileira de Saúde Materno Infantil (Online) - Instituto de Medicina Integral Prof. Fernando Figueira (IMIPFF) |
repository.mail.fl_str_mv |
||revista@imip.org.br |
_version_ |
1752129929887088640 |