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CC BY 4.0 · Rev Bras Ortop (Sao Paulo) 2024; 59(05): e737-e744
DOI: 10.1055/s-0044-1786200
Artigo Original
Ortopedia Pediátrica

Avaliação tridimensional da marcha: Cinética, cinemática e eletromiográfica em pacientes com mucopolissacaridose tipos IV e VI

Article in several languages: português | English
Francisco Robson Queiroz Rego 
1   Universidade Federal de Pernambuco, Recife, PE, Brasil
,
Herison Franklin Viana de Oliveira 
1   Universidade Federal de Pernambuco, Recife, PE, Brasil
,
Epitácio Leite Rolim Filho 
1   Universidade Federal de Pernambuco, Recife, PE, Brasil
› Author Affiliations Suporte Financeiro Os autores declaram que não houve suporte financeiro de fontes públicas, comerciais ou sem fins lucrativos.
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Resumo

Objetivo Avaliar e determinar, através de análise tridimensional (3D) instrumentada da marcha, o perfil cinético, cinemático e eletromiográfico de pacientes com mucopolissacaridose (MPS) IV e VI.

Métodos Foi realizado um estudo transversal com 11 pacientes atendidos em um serviço de referência em doenças raras, avaliados em laboratório de análise do movimento. Foram coletados dados clínicos, do exame físico e dados cinéticos, cinemáticos e eletromiográficos através de sistema 3D de movimento, entre junho de 2020 e janeiro de 2021.

Resultados Havia 5 pacientes (45,5%) do gênero feminino e 6 (54,5%) do masculino. Adicionalmente, 9 (81,8%) tinham MPS VI e 2 (18,2%) IV. A idade média foi de 14,6 anos. A velocidade média (m/s) foi de 0,68 (±0,21) e o comprimento da passada de 0,66 (±0,15). Os ângulos estáticos mais alterados foram a abdução-adução dos quadris, o arco de movimento dos joelhos e o ângulo de progressão do pé. Um padrão de marcha em flexão e adução dos quadris e flexão dos joelhos foi identificado na maioria dos casos. O Gait Profile Score (GPS) foi de 14,58 (±6,72) à direita e 11,71(±3,39) à esquerda. O Gait Deviation Index (GDI) foi de 73,23 (±14,50) à direita e de 80,45 (±17,05) à esquerda. A atividade muscular seguiu aproximadamente o modelo corrente.

Conclusão Os pacientes apresentaram uma velocidade média e comprimento da passada diminuídos. Um padrão de marcha em flexão e adução dos quadris e flexão dos joelhos foi identificado na maioria dos casos. Tanto GPS quanto GDI mostraram desvio importante da normalidade.

Palavras-chave

avaliação tridimensional da marcha - cinemática - cinética - eletromiografia - mucopolissacaridoses

Trabalho desenvolvido na Universidade Federal de Pernambuco, Hospital Maria Lucinda e no Laboratório de Análise do Movimento do Instituto Rolim, Recife, PE, Brasil.




Publication History

Received: 19 April 2023

Accepted: 21 July 2023

Article published online:
22 July 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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  • Referências

  • 1 Gaffke L, Pierzynowska K, Podlacha M, Brokowska J, Węgrzyn G. Changes in cellular processes occurring in mucopolysaccharidoses as underestimated pathomechanisms of these diseases. Cell Biol Int 2021; 45 (03) 498-506
    CrossrefPubMedSearch in Google Scholar
  • 2 Herring JA, Tachdjian MO. Texas Scottish Rite Hospital for Children. 5th ed.. Philadelphia: Saunders; 2013: 455
    Search in Google Scholar
  • 3 Çelik B, Tomatsu SC, Tomatsu S, Khan SA. Epidemiology of Mucopolysaccharidoses Update. Diagnostics (Basel) 2021; 11 (02) 273
    CrossrefPubMedSearch in Google Scholar
  • 4 Federhen A, Pasqualim G, de Freitas TF. et al. Estimated birth prevalence of mucopolysaccharidoses in Brazil. Am J Med Genet A 2020; 182 (03) 469-483
    CrossrefPubMedSearch in Google Scholar
  • 5 Pinto LL, Schwartz IV, Puga AC, Vieira TA, Munoz MV, Giugliani R. Prospective study of 11 Brazilian patients with mucopolysaccharidosis II. J Pediatr (Rio J) 2006; 82 (04) 273-278
    CrossrefPubMedSearch in Google Scholar
  • 6 Del Longo A, Piozzi E, Schweizer F. Ocular features in mucopolysaccharidosis: diagnosis and treatment. Ital J Pediatr 2018; 44 (Suppl. 02) 125
    CrossrefPubMedSearch in Google Scholar
  • 7 Barone R, Pellico A, Pittalà A, Gasperini S. Neurobehavioral phenotypes of neuronopathic mucopolysaccharidoses. Ital J Pediatr 2018; 44 (Suppl. 02) 121
    CrossrefPubMedSearch in Google Scholar
  • 8 Kubaski F, de Oliveira Poswar F, Michelin-Tirelli K. et al. Diagnosis of Mucopolysaccharidoses. Diagnostics (Basel) 2020; 10 (03) 172
    CrossrefPubMedSearch in Google Scholar
  • 9 Cardoso-Santos A, Azevedo AC, Fagondes S, Burin MG, Giugliani R, Schwartz IV. Mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): assessment of joint mobility and grip and pinch strength. J Pediatr (Rio J) 2008; 84 (02) 130-135
    PubMedSearch in Google Scholar
  • 10 Davis RB, Ounpuu S, Tyburski D, Gage JR. A gait analysis data collection and reduction technique. Hum Mov Sci 1991; 10 (05) 575-587
    CrossrefSearch in Google Scholar
  • 11 Souza MA, Cezarani A, Lizzi EADS. et al. The use of the gait profile score and gait variable score in individuals with Duchenne Muscular Dystrophy. J Biomech 2020; 98: 109485
    CrossrefPubMedSearch in Google Scholar
  • 12 Schwartz IV, Ribeiro MG, Mota JG. et al. A clinical study of 77 patients with mucopolysaccharidosis type II. Acta Paediatr 2007; 96 (455) 63-70
    CrossrefPubMedSearch in Google Scholar
  • 13 Sutherland DH. The evolution of clinical gait analysis part l: kinesiological EMG. Gait Posture 2001; 14 (01) 61-70
    CrossrefPubMedSearch in Google Scholar
  • 14 Dhawale AA, Church C, Henley J. et al. Gait pattern and lower extremity alignment in children with Morquio syndrome. J Pediatr Orthop B 2013; 22 (01) 59-62
    CrossrefPubMedSearch in Google Scholar
  • 15 Akyol MU, Alden TD, Amartino H. et al; MPS Consensus Programme Steering Committee, MPS Consensus Programme Co-Chairs. Recommendations for the management of MPS VI: systematic evidence- and consensus-based guidance. Orphanet J Rare Dis 2019; 14 (01) 118
    CrossrefPubMedSearch in Google Scholar
  • 16 Matos MA, Prado A, Schenkel G, Barreto R, Acosta AX. Energy expenditure during gait in patients with mucopolysaccharidosis. Acta Ortop Bras 2013; 21 (02) 116-119
    PubMedSearch in Google Scholar
  • 17 Schmid S, Schweizer K, Romkes J, Lorenzetti S, Brunner R. Secondary gait deviations in patients with and without neurological involvement: a systematic review. Gait Posture 2013; 37 (04) 480-493
    CrossrefPubMedSearch in Google Scholar
  • 18 Patterson KK, Nadkarni NK, Black SE, McIlroy WE. Gait symmetry and velocity differ in their relationship to age. Gait Posture 2012; 35 (04) 590-594
    CrossrefPubMedSearch in Google Scholar
  • 19 Baker R, Esquenazi A, Benedetti MG, Desloovere K. Gait analysis: clinical facts. Eur J Phys Rehabil Med 2016; 52 (04) 560-574
    PubMedSearch in Google Scholar
  • 20 Lin HY, Lee CL, Lo YT. et al. The relationships between urinary glycosaminoglycan levels and phenotypes of mucopolysaccharidoses. Mol Genet Genomic Med 2018; 6 (06) 982-992
    CrossrefPubMedSearch in Google Scholar
  • 21 Salazar-Torres JJ, Church C, Shields T. et al. Evaluation of Gait Pattern and Lower Extremity Kinematics of Children with Morquio Syndrome (MPS IV). Diagnostics (Basel) 2021; 11 (08) 1350
    CrossrefPubMedSearch in Google Scholar
  • 22 Kimoto M, Okada K, Sakamoto H, Kondou T, Kawanobe U. Relationship between walking efficiency and muscular strength of the lower limbs in children with cerebral palsy. J Phys Ther Sci 2019; 31 (03) 232-235
    CrossrefPubMedSearch in Google Scholar
  • 23 Hahn ME, Chou LS. Can motion of individual body segments identify dynamic instability in the elderly?. Clin Biomech (Bristol, Avon) 2003; 18 (08) 737-744
    CrossrefPubMedSearch in Google Scholar
  • 24 Ellis RG, Howard KC, Kram R. The metabolic and mechanical costs of step time asymmetry in walking. Proc Biol Sci 2013; 280 (1756) 20122784
    PubMedSearch in Google Scholar
  • 25 Neufeld EF, Muenzer J. The mucoplysaccharidoses. In: Scriver CR. et al. The metabolic and molecular bases of inherited disease. 8 th ed.. New York: McGraw-Hill; 2001: 3421-3452
    Search in Google Scholar
  • 26 Massaad A, Assi A, Skalli W, Ghanem I. Repeatability and validation of gait deviation index in children: typically developing and cerebral palsy. Gait Posture 2014; 39 (01) 354-358
    CrossrefPubMedSearch in Google Scholar
  • 27 Agostini V, Nascimbeni A, Gaffuri A, Imazio P, Benedetti MG, Knaflitz M. Normative EMG activation patterns of school-age children during gait. Gait Posture 2010; 32 (03) 285-289
    CrossrefPubMedSearch in Google Scholar
  • 28 Granata KP, Padua DA, Abel MF. Repeatability of surface EMG during gait in children. Gait Posture 2005; 22 (04) 346-350
    CrossrefPubMedSearch in Google Scholar
  • 29 Fleming AM, Ribeiro CT, Horovitz DG, Ribeiro LC, Correa AP. Description of functioning in children and adolescents with mucopolysaccharidosis-case series. J Inborn Errors Metab Screen 2020; 8: e20190010
    CrossrefSearch in Google Scholar