Evolução motora de paciente com mucopolissacaridose tipo 1

Iwabe, Cristina; Frezzato, Renata Camargo; Nogueira, Ana Lívia

doi:10.1590/s0103-05822010000300018

Cited by 3 publications

(1 citation statement)

References 11 publications

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“…In this study, 13 patients (68.42%) were diagnosed before the age of three. The diagnosis in this population is usually given between one and three years, due to the fact that it is the period of onset of the first signs of the disease [18][19][20][21] .…”

Section: Discussionmentioning

confidence: 99%

Knee and ankle disorders during functional gait in mucopolysaccharidosis type VI

et al. 2021

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To evaluate knee and ankle disorders during functional gait assessment in individuals with Mucopolysaccharidosis type VI (MPS VI). 19 subjects were included in this cross-sectional study and allocated in three groups according to age: Children Group (n=11); Adolescent Group (n=4); and Adult Group (n=4). Subphases of one gait cycle were analyzed: Initial contact; Medium support, and Pre-Balance. All volunteers with MPS VI presented greater knee and ankle flexion angles, in all gait subphases, when compared to the normal values defined by literature (p<0.05). Initial contact subphase: knee flexion angle ranging from 8.5º to 15º; Ankle = Child Group −23.73º ± 8.53º; Adolescent Group = −25º ± 11.22º; Adult Group = −27.75º ± 3.3º. Medium support subphase: Knee = Child Group 19.64º ± 10.47º; Adolescent Group 16.75º ± 10.34º; Adult Group = 21.25º ± 12.84º. Ankle = Child Group −18.82º ± 8.91º ± 8.53º; Adolescent Group = −16.5º ± 9.33º; Adult Group = −22.25º ± 4.19º. Pre-Balance subphase: Knee = Child Group 22.72º ± 13.49º; Adolescent Group 21.25º ± 7.97º; Adult Group = 27º ± 16.27º. Ankle = Child Group -15º ± 9.76º; Adolescent Group = −15.75º ± 5.31º; Adult Group = −14.75º ± 3.86º. In this study, MPS VI individuals presented hyperflexion of knee and ankle as the main joint disorders during functional gait, regardless of age.

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Section: Discussionmentioning

confidence: 99%

Knee and ankle disorders during functional gait in mucopolysaccharidosis type VI

et al. 2021

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Mucopolysaccharidosis patients have reduced functional capacity

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Santos

et al. 2021

Pediatric Pulmonology

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Introduction Mucopolysaccharidoses (MPSs) are a group of rare diseases caused by an intralysosomal accumulation of glycosaminoglycans, resulting in a multisystemic clinical condition characterized by variable degrees of physical‐functional impairment. Objective To evaluate the functional capacity (FC) of MPS patients and compare with a healthy control group. Methods This is a cross‐sectional study of 6‐ to 39‐year‐old patients followed at a medical reference center and compared with their control peers, matched by age and sex. FC was assessed using the Sit‐to‐Stand Test (SST) and Incremental Shuttle Walk Test (ISWT). Heart rate (HR) and Borg rating of perceived exertion were measured before and after ISWT. HR recovery (HRR) was defined as the HR at the end of the test minus the HR in the second minute after ISWT. Results Nineteen (19) MPS patients, 69% with type II MPS and mean age 17 ± 11 years were evaluated. Every patient was under enzyme replacement therapy. The time to perform the SST was longer in the MPS group (10.6 ± 2.5 s vs. 6.7 ± 1.2 s; p < .01). The MPS group achieved lower values of distance covered on the ISWT (407.6 ± 329.8 m vs. 1131.9 ± 183.3 m; p < .01), with a significantly higher Borg (6 [5–8] vs. 2 [1–4]; p = .02). The MPS group's HRR was slower than the controls (32.9 ± 20.2 beats per minute [bpm] vs. 69.1 ± 25.9 bpm; p < .001). Discussion We observed a pronounced reduction in the MPS group's FC compared to their healthy peers and a worse HRR after completing the test.

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Produção científica em avaliação motora: análise bibliométrica sobre a utilização da Escala de Desenvolvimento Motor

et al. 2020

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A avaliação motora é essencial para acompanhamento do desenvolvimento infantil. No Brasil, a Escala de Desenvolvimento Motor (EDM) destaca-se como um instrumento de avaliação de crianças de 2 a 11 anos. O estudo teve como objetivo analisar a produção científica relacionada a utilização dessa escala através de uma análise bibliométrica, explorando dados nas publicações, autores e periódicos, com foco em indicadores e impactos científicos. Um total de 53 artigos foram considerados, selecionados nas bases de dados SciELO, SCOPUS, MEDLINE, SPORTDiscus, Web of Science e Portal de Periódicos CAPES. Como resultados, a produção científica aumentou a partir de 2010 e têm alcançado revistas nacionais e internacionais e publicações em três idiomas. As revistas com maior fator de impacto estão na área de medicina e as publicações contemplam profissionais da saúde e da educação. A rede de pesquisas envolve 32 Universidades com maior concentração em SC e SP. O autor da EDM e seus colaboradores institucionais são os que mais vezes publicaram com a escala. Por fim, os estudos têm focado na avaliação de crianças com transtornos do desenvolvimento ou dificuldades de aprendizagem.

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Evolução motora de paciente com mucopolissacaridose tipo 1

Cited by 3 publications

References 11 publications

Knee and ankle disorders during functional gait in mucopolysaccharidosis type VI

Knee and ankle disorders during functional gait in mucopolysaccharidosis type VI

Mucopolysaccharidosis patients have reduced functional capacity

Produção científica em avaliação motora: análise bibliométrica sobre a utilização da Escala de Desenvolvimento Motor

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