Basic pulmonary function of Duchenne muscular dystrophy patients in a Brazilian sample
DOI:
https://doi.org/10.62827/fb.v26i1.1043Keywords:
Duchenne; Maximal Respiratory Pressures; Age Groups; Respiratory Function Tests; Child; Adolescent; Cross-Sectional Studies.Abstract
Introduction: Studies of lung and respiratory function favor the understanding of their variability and clinical applicability. Objective: Characterization and analysis of basic lung function (forced vital capacity – FVC, maximum respiratory pressures – MRP and peak cough flow – PFM) in a Brazilian sample of people with Duchenne muscular dystrophy (DMD) and development of predictive formulas. Methods: Sample of 231 medical records, divided into 2 groups: G1- 9 to 13 years and G2- 14 to 18 years. Descriptive analysis of the variables and Spearman correlation analysis were performed to verify association between them. Comparison of MRP by age used one-way ANOVA. Multiple regression analyses identified possible predictive variables for FVC and FVC (α=0.05). Results: Positive correlation between the variables studied and similarity with values in the literature. Analysis focusing on age showed Maximum Inspiratory Pressure (MIP) as a strong predictor of FVC. An equation was established to predict FVC from MRP and between MRP and PFM. Conclusion: The analysis of the age factor as an indicator of disease progression allowed the generation of predictive formulas for FVC from MIP and for PCF from MRP. It is noteworthy that MIP is a strong predictor of FVC.
References
Caromano FA, Gomes ALO, Pinto NA, Góes ER, Hirouse LN, Assis SMB, Carvalho EV. Correlação entre massa de gordura corporal, força muscular, pressões respiratórias máximas e função na distrofia muscular de Duchenne. ConScientiae Saúde. 2010;3(3):423-9. doi:10.5585/conssaude.v9i3.2320.
Garcia Júnior A, Caromano FA, Contesini AM, Escorcio R, Fernandes LA, João SMA. Thoracic cirtometry in children with Duchenne muscular dystrophy - expansion of the method. Braz J Phys Ther. 2013;17(1):1-8. doi:10.1590/S1413-35552012005000064.
Quanjer PH, et al. Lung volumes and forced ventilatory flows: report of working party, standardization of lung function tests. Eur Respir J. 1993;6:5-40. doi:10.1183/09041950.005s1693.
Wilson SH, Cook NT, Edwards RHS. Predicted normal values for maximal respiratory pressures in Caucasian adults and children. Thorax. 1984;39:535-8. doi:10.1136/thx.39.7.535.
Lyager S, Steffensen B, Juhl B. Indicators of need for mechanical ventilation in Duchenne muscular dystrophy and spinal muscular dystrophy. Chest. 1995;180(3):779-85. doi:10.1378/chest.108.3.779.
Zatz M, Pavanello RCM, Lazar M, Yamamoto GL, Lourenço NCV, Cerqueira A. Milder course in Duchenne patients with nonsense mutations and no muscle dystrophin. Neuromuscul Disord. 2014;24(11):1-6. doi:10.1016/j.nmd.2014.06.003.
Selestrin CC. Avaliação respiratória em pacientes com distrofia muscular de Duchenne [tese]. São Paulo: Faculdade de Saúde Pública da USP; 2014. doi:10.11606/T.6.2014.tde-11062014-155856.
Hubbard R, Bayarri MJ, Kenneth NB, Carlton MA. Confusion over measures of evidence (p´s) versus errors (α´s) in classical statistical testing. Am Stat. 2003;57(3):171-82. Disponível em: https://www.jstor.org/stable/30037265. Acesso em: 25 ago. 2021.
Nicot F, Hart N, Forin V, Boulè M, Clément A, Polkey MI, Lofaso F, et al. Respiratory muscle testing: a valuable tool for children with neuromuscular disorders. Am J Respir Crit Care Med. 2006;174:67-74. doi:10.1164/rccm.200512-1841OC.
Mayer OH, Finkel RS, Rummey C, Benton MJ, Glanzman AM, Flickinger J, et al. Characterization of pulmonary function in Duchenne muscular dystrophy. Pediatr Pulmonol. 2015;50:487-94. doi:10.1002/ppul.23172.
Meier T, Rummey C, Leinonen M, Spagnolo P, Mayer OH, Buyse GM, DELOS Study Group. Characterization of pulmonary function in 10-18 year old patients with Duchenne muscular dystrophy. Neuromuscul Disord. 2017;27:307-14. doi:10.1016/j.nmd.2016.12.014.
American Thoracic Society. Standardization of Spirometry, 1994 Update. Am J Respir Crit Care Med. 1995;152(3):1107-36. doi:10.1164/ajrccm.152.3.7663792.
Machado DL, Silva EC, Resende MBD, Carvalho CRF, Zanoteli E, Reed UC. Lung function monitoring in patients with Duchenne muscular dystrophy on steroid therapy. BMC Res Notes. 2012;5:435. doi:10.1186/1756-0500-5-435.
Finder JD, Birnkrant DJ, Carl J, Farber H, Gozal D, Iannaccone ST, et al. Respiratory care of the patient with Duchenne muscular dystrophy: an official ATS consensus statement. Am J Respir Crit Care Med. 2004;170(4):456-65. doi:10.1164/rccm.200307-885ST.
Lomauro A, D’Angelo MG, Aliverti A. Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options. Ther Clin Risk Manag. 2015;11:1475-88. doi:10.2147/TCRM.S55889.
Schmidt S, et al. Timed function tests, motor function measure, and quantitative thigh muscle MRI in ambulant children with Duchenne muscular dystrophy: a cross-sectional analysis. Neuromuscul Disord. 2018;28:16–23. doi:10.1016/j.nmd.2017.10.003.
Pinchak C, et al. Actualización en el manejo respiratorio de pacientes con enfermedades neuromusculares. Arch Pediatr Urug. 2018;89(1):40-51. Disponível em: http://www.scielo.edu.uy/scielo.php?script=sci_arttext&pid=S1688-12492018000100040&lng=es&nrm=iso. Acesso em: 7 set. 2021. doi:10.31134/ap.89.1.8.
Battini R, Chieffo D, Bulgheroni S, Piccini G, Pecini C, Lucibello S, et al. Cognitive profile in Duchenne muscular dystrophy boys without intellectual disability: the role of executive functions. Neuromuscul Disord. 2017. doi:10.1016/j.nmd.2017.11.018.
Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemens PR, Kaul A, et al. Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurol. 2010;9(1):77-93. doi:10.1016/S1474-4422(09)70271-6.
Bushby K, Finkel R, Birnkrant DJ, Case LE, Clemens PR, Kaul A, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurol. 2010;9(2):177-89. doi:10.1016/S1474-4422(09)70272-8.
Fortes CPDD, Koller ML, Araújo APQC. Cuidados com a pessoa com distrofia muscular de Duchenne: revisando as recomendações. Rev Bras Neurol. 2018;54(2):5-13. Disponível em: https://revistas.ufrj.br/index.php/rbn/article/view/19108. Acesso em: 25 fev. 2025.
Henricson EK, Abresch RT, Cnaan A, Hu F, Duong T, Arrieta A, et al. The cooperative international neuromuscular research group Duchenne natural history study: glucocorticoid treatment preserves clinically meaningful functional milestones and reduces rate of disease progression. Muscle Nerve. 2013;48(1):55-67. doi:10.1002/mus.23808.
Herdy GVH, Pinto RDB, Costa GA, Torbey AFM, Ramos VG, Vasconcelos MM. Estudo clínico e molecular na distrofia muscular de Duchenne. Int J Cardiovasc Sci. 2015;28(3):173-80. doi:10.5935/2359-4802.20150026
Hukuda ME. Responsividade da escala de avaliação funcional do sentar e levantar da cadeira para pacientes com distrofia muscular de Duchenne (FES-DMD-D1), no período de um ano [tese]. São Paulo: Faculdade de Medicina, Universidade de São Paulo; 2015. doi:10.11606/T.5.2015.tde-12052015-101633. Acesso em: 18 set. 2021.
Kang SW. Pulmonary rehabilitation in patients with neuromuscular disease. Yonsei Med J. 2006;47(3):307-14. doi:10.3349/ymj.2006.47.3.307.
Khirani S, Ramirez A, Aubertin G, Boulè M, Chemouny C, Forin V, et al. Respiratory muscle decline in Duchenne muscular dystrophy. Pediatr Pulmonol. 2014;49:473-81. doi:10.1002/ppul.22847.
Luiz LC, Marson FAL, Almeida CCB, Toro AADC, Nucci A, Ribeiro JD. Analysis of motor and respiratory function in Duchenne muscular dystrophy patients. Respir Physiol Neurobiol. 2019;262:1-11. doi:10.1016/j.resp.2019.01.009.
Murray ACS, Carmo SA, Voos MC, Silva RL, Caromano FA. Instrumentation and evaluation of measurements of MIP and MEP in the pulmonary function of people with Duchenne muscular dystrophy: a review. Cad Pós-Grad Distúrb Desenvolv [Internet]. 2021;21(1):109-28. Disponível em: http://dx.doi.org/10.5935/cadernosdisturbios.v21n1p109-128.
Rodini C, Collange L, Juliano Y, Oliveira C, Isola A, Almeida S, Misao M. Influência da adequação postural em cadeira de rodas na função respiratória de pacientes com distrofia muscular de Duchenne. Fisioter Pesqui. 2012;19(2):97-102. doi:10.1590/S1809-29502012000200002.
Downloads
Published
Issue
Section
License
Copyright (c) 2025 Ana Cristina de Sousa Murray , Eneida Yuri Suda , Mariana Callil Voos , Renata Escorcio , Cynthia Bedeschi , Silvana Amando do Carmo , Fernando Alves Vale , Fátima Aparecida Caromano (Autor)

This work is licensed under a Creative Commons Attribution 4.0 International License.
Autores que publicam nesta revista concordam com os seguintes termos:
Autores mantém os direitos autorais e concedem à revista o direito de primeira publicação, com o trabalho simultaneamente licenciado sob a Licença Creative Commons Attribution 4.0 que permite o compartilhamento do trabalho com reconhecimento da autoria e publicação inicial nesta revista.
Autores têm autorização para distribuição não-exclusiva da versão do trabalho publicada nesta revista (ex.: publicar em repositório institucional ou como capítulo de livro), com reconhecimento de autoria e publicação inicial nesta revista.