Analysis of motor and respiratory function in Duchenne muscular dystrophy patients

被引:5
|
作者
Luiz, Livia Cocato [1 ]
Lima Marson, Fernando Augusto [1 ,2 ,3 ]
Bresciani Almeida, Celize Cruz [1 ]
Dalbo Contrera Toro, Adyleia Aparecida [1 ]
Nucci, Anamarli [4 ]
Ribeiro, Jose Dirceu [1 ,3 ]
机构
[1] Univ Estadual Campinas, Sch Med Sci, Dept Pediat, POB 6111, BR-13081970 Campinas, SP, Brazil
[2] Univ Estadual Campinas, Sch Med Sci, Dept Med Genet & Genom Med, POB 6111, BR-13081970 Campinas, SP, Brazil
[3] Univ Estadual Campinas, Sch Med Sci, Ctr Pediat Invest, Lab Pulm Physiol, POB 6111, BR-13081970 Campinas, SP, Brazil
[4] Univ Estadual Campinas, Sch Med Sci, Dept Neurol, POB 6111, BR-13081970 Campinas, SP, Brazil
基金
巴西圣保罗研究基金会;
关键词
Duchenne; Genetic disease; Lung function; motor skills; Muscular dystrophy; Spirometry; 6-MINUTE WALK TEST; PEAK COUGH FLOW; PULMONARY-FUNCTION; MUSCLE STRENGTH; ETEPLIRSEN; YOUNG; PRESSURE; CAPACITY; CHILDREN; THERAPY;
D O I
10.1016/j.resp.2019.01.009
中图分类号
Q4 [生理学];
学科分类号
071003 ;
摘要
Introduction: Duchenne muscular dystrophy(DMD) shows motor and respiratory impairment. Methods: 19 DMD patients (DMDG) (nine ambulatory and 10 non-ambulatory) were evaluated through motor function measure (MFM), 6-minute walk test (6MWT), respiratory muscle strength, cough peak flow, spirometry and volumetric capnography (VCap) tools. Control group that performed spirometry and VCap (CG1-n = 17) were different from those that performed the 6MWT (CG2-n = 8). Results: The follow tools were assessed (p < 0.05): (i) MFM: Ambulatory patients showed higher values than non-ambulatory patients; (ii) 6MWT: DMDG walked a shorter distance and showed higher respiratory rate at rest and heart rate (HR) at rest than CG2; (iii) Spirometry: DMDG and non-ambulatory patients had minor values achieved in spirometry when compared with CG1 and ambulatory patients, respectively; (iv) VCap: DMDG when compared with CG1 showed: (< 11 years-old) lower values in VCap parameters; (> 11 years-old): higher HR and lower slope 2. There was correlation between spirometry, mainly for zFEV(1)/FVC, and MFM. Conclusion DMDG showed motor (MFM/6MWT) and respiratory (spirometry/VCap) deterioration when compared with CG. Non-ambulatory condition was associated with worse MFM and spirometry.
引用
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页码:1 / 11
页数:11
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