Gross Motor Function Classification System in Patients with Mucopolysaccharidosis

IntroductionMucopolysaccharidosis (MPS) are a group of rare orthopedic disorders caused by heterogeneous genetic abnormalities in which lysosomal storage alteration lead to intracellular accumulation of glycosaminoglycans (GAGs) that injure and create dysfunction of varying degrees in multiple organ...

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Autores principales: Almeida Matos, Marcos, Barreto, Rosa, Quadros, Vitor, Penha, Carlos Eduardo, Xavier Acosta, Angelina
Formato: Artículo revista
Lenguaje:Español
Publicado: Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2013
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Acceso en línea:https://revistas.unc.edu.ar/index.php/med/article/view/7464
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Revista de la Facultad de Ciencias Médicas de Córdoba de Universidad Nacional de Córdoba
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record_format ojs
spelling I10-R327-article-74642024-08-27T18:19:22Z Gross Motor Function Classification System in Patients with Mucopolysaccharidosis Almeida Matos, Marcos Barreto, Rosa Quadros, Vitor Penha, Carlos Eduardo Xavier Acosta, Angelina . IntroductionMucopolysaccharidosis (MPS) are a group of rare orthopedic disorders caused by heterogeneous genetic abnormalities in which lysosomal storage alteration lead to intracellular accumulation of glycosaminoglycans (GAGs) that injure and create dysfunction of varying degrees in multiple organs and systems, in a progressive and lethal way. The type of MPS can be classified according to the enzyme defect by which it is determined or according to its clinical manifestations and the progression of the disease The impairment of the musculoskeletal system or “dysostosis multiplex” is a common feature in all MPSs. It is characterized by osteoarticular deformities (kyphosis, scoliosis, knee valgus, equinovarus), joint stiffness with a loss in range of motion (ROM), and upper motor neuron impairment (myelopathy, hypertonia, spasticity). There is no specific motor function classification system for individuals with MPS. However, the progression of motor function impairment such as walking, sitting, and functional independence in day to day activities is directly related to the severity of the disorder... Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2013-12-01 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion application/pdf https://revistas.unc.edu.ar/index.php/med/article/view/7464 10.31053/1853.0605.v70.n4.7464 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 70 No. 4 (2013) Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 70 Núm. 4 (2013) Revista da Faculdade de Ciências Médicas de Córdoba; v. 70 n. 4 (2013) 1853-0605 0014-6722 10.31053/1853.0605.v70.n4 spa https://revistas.unc.edu.ar/index.php/med/article/view/7464/8517 Derechos de autor 2013 Universidad Nacional de Córdoba https://creativecommons.org/licenses/by-nc/4.0
institution Universidad Nacional de Córdoba
institution_str I-10
repository_str R-327
container_title_str Revista de la Facultad de Ciencias Médicas de Córdoba
language Español
format Artículo revista
topic .
spellingShingle .
Almeida Matos, Marcos
Barreto, Rosa
Quadros, Vitor
Penha, Carlos Eduardo
Xavier Acosta, Angelina
Gross Motor Function Classification System in Patients with Mucopolysaccharidosis
topic_facet .
author Almeida Matos, Marcos
Barreto, Rosa
Quadros, Vitor
Penha, Carlos Eduardo
Xavier Acosta, Angelina
author_facet Almeida Matos, Marcos
Barreto, Rosa
Quadros, Vitor
Penha, Carlos Eduardo
Xavier Acosta, Angelina
author_sort Almeida Matos, Marcos
title Gross Motor Function Classification System in Patients with Mucopolysaccharidosis
title_short Gross Motor Function Classification System in Patients with Mucopolysaccharidosis
title_full Gross Motor Function Classification System in Patients with Mucopolysaccharidosis
title_fullStr Gross Motor Function Classification System in Patients with Mucopolysaccharidosis
title_full_unstemmed Gross Motor Function Classification System in Patients with Mucopolysaccharidosis
title_sort gross motor function classification system in patients with mucopolysaccharidosis
description IntroductionMucopolysaccharidosis (MPS) are a group of rare orthopedic disorders caused by heterogeneous genetic abnormalities in which lysosomal storage alteration lead to intracellular accumulation of glycosaminoglycans (GAGs) that injure and create dysfunction of varying degrees in multiple organs and systems, in a progressive and lethal way. The type of MPS can be classified according to the enzyme defect by which it is determined or according to its clinical manifestations and the progression of the disease The impairment of the musculoskeletal system or “dysostosis multiplex” is a common feature in all MPSs. It is characterized by osteoarticular deformities (kyphosis, scoliosis, knee valgus, equinovarus), joint stiffness with a loss in range of motion (ROM), and upper motor neuron impairment (myelopathy, hypertonia, spasticity). There is no specific motor function classification system for individuals with MPS. However, the progression of motor function impairment such as walking, sitting, and functional independence in day to day activities is directly related to the severity of the disorder...
publisher Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
publishDate 2013
url https://revistas.unc.edu.ar/index.php/med/article/view/7464
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first_indexed 2024-09-03T20:59:14Z
last_indexed 2024-09-03T20:59:14Z
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