The Value of Respiratory Muscle Testing in Children With Neuromuscular Disease

Fauroux, Brigitte; Quijano‐Roy, Susana; Desguerre, Isabelle; Khirani, Sonia

doi:10.1378/chest.14-0819

Cited by 32 publications

(21 citation statements)

References 67 publications

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“…The FVC usually increases with age through 10 years and plateaus before declining after age 18 years in DMD patients 8. It has a poor specificity for the respiratory muscle weakness 8, 9. A >25% postural FVC drop may indicate diaphragm weakness 10.…”

Section: Introductionmentioning

confidence: 99%

Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy

Mankodi

Kovacs

Norato

et al. 2017

Ann Clin Transl Neurol

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ObjectiveTo examine the diaphragm and chest wall dynamics with cine breathing magnetic resonance imaging (MRI) in ambulatory boys with Duchenne muscular dystrophy (DMD) without respiratory symptoms and controls.MethodsIn 11 DMD boys and 15 controls, cine MRI of maximal breathing was recorded for 10 sec. The lung segmentations were done by an automated pipeline based on a Holistically‐Nested Network model (HNN method). Lung areas, diaphragm, and chest wall motion were measured throughout the breathing cycle.ResultsThe HNN method reliably identified the contours of the lung and the diaphragm in every frame of each dataset (~180 frames) within seconds. The lung areas at maximal inspiration and expiration were reduced in DMD patients relative to controls (P = 0.02 and <0.01, respectively). The change in the lung area between inspiration and expiration correlated with percent predicted forced vital capacity (FVC) in patients (r s = 0.75, P = 0.03) and was not significantly different between groups. The diaphragm position, length, contractility, and motion were not significantly different between groups. Chest wall motion was reduced in patients compared to controls (P < 0.01).InterpretationCine breathing MRI allows independent and reliable assessment of the diaphragm and chest wall dynamics during the breathing cycle in DMD patients and controls. The MRI data indicate that ambulatory DMD patients breathe at lower lung volumes than controls when their FVC is in the normal range. The diaphragm moves normally, whereas chest wall motion is reduced in these boys with DMD.

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Section: Introductionmentioning

confidence: 99%

Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy

Mankodi

Kovacs

Norato

et al. 2017

Ann Clin Transl Neurol

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“…adicionalmente, la evaluación de la fuerza de los músculos inspiratorios (PImax) y espiratorios (PEmax) mediante la medición de las presiones generadas en la boca complementan las pruebas de función pulmonar, debido a que muestran una fuerte asociación con las capacidades pulmonares principalmente en pacientes neuromusculares 8,9,10 . En virtud de lo anterior, estas pruebas de función respiratoria son consideradas para el seguimiento del curso clínico de la enfermedad, así como también, como medida de efecto en la evaluación de alguna intervención con fines terapéuticos 7,10 .…”

Section: Introductionunclassified

“…Lo cual, se encuentra sujeto al grado de aprendizaje de la técnica y experiencia del paciente en su ejecución 1,6,8 .…”

Section: Introductionunclassified

Confiabilidad de la fuerza muscular respiratoria y flujos espiratorios forzados en adolescentes sanos

2015

Rev. chil. enferm. respir.

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“…In non-injured subjects, these muscles are also used during forced expiratory maneuvers like a cough. Weak or paralyzed abdominal muscles results in a weak or an inadequate cough, leading to impaired secretion clearance, atelectasis, pneumonia and other respiratory complications (Fauroux et al, 2015;Inal-Ince et al, 2009;Khirani et al, 2014).…”

Section: Resultsmentioning

confidence: 99%

“…Routine testing of lung functions and respiratory muscle testing are recommended in children with neuromuscular diseases (Fauroux, Quijano-Roy, Desguerre, & Khirani, 2015 (Jain, Brown, Tun, Gagnon, & Garshick, 2006). However, standard pulmonary testing does not evaluate the multi-muscle motor control by the CNS.…”

Section: Respiratory Motor Control In Typically Children Introductionmentioning

confidence: 99%

Trunk and respiratory motor control in typically developing children and its implications in children with chronic spinal cord injury.

Singh¹

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Singh, Goutam, "Trunk and respiratory motor control in typically developing children and its implications in children with chronic spinal cord injury." (2017 Independent sitting is a major milestone and is also a prerequisite for optimal performance of activities of daily living (ADLs). Development of sitting posture control is a dynamic process involving control of degrees of freedom of head and trunk. Traditionally, trunk has been modeled as a single unit (segment).However, recent studies have suggested that it is made up multiple spinal units, controlled by a combination of trunk muscles. During typical development, posture control of trunk is different for different trunk segments. This motor development of trunk control is a complex process due to constant interaction between the nervous system and environment. Any interruption in the normal processes would further complicate it, affecting the typical development of the child.Poor trunk control and respiratory complications are characteristic features among children, adolescents, and adults with neuromuscular disorders.vi Pediatric spinal cord injuries (SCIs) also pose a unique challenge compared to SCI in adults because of the continuous physical and cognitive development.Children with SCI exhibit deficits in trunk motor control, which impair their ability to sit or ambulate. These motor deficits can lead to compensatory changes in other segments of the body, which cause further deviations from typical postures.Trunk muscles have the dual function of supporting both, breathing and trunk posture. Therefore, in children with SCI, impairment of posture control will also affect respiratory functions. Depending on level and severity of injury, can potentially lead to severe respiratory insufficiency. Symptoms of respiratory insufficiency are highly correlated with the level and severity of spinal lesions.Injury at higher cervical and thoracic cord levels causes paresis and paralysis of most of the respiratory muscles, which increase the workload of breathing. As a result, respiratory complications are the leading cause of death among children with SCI. Therefore, impairment of posture control following SCI also affects respiratory functions.Lack of appropriate tools to evaluate trunk motor control following SCI restricts the ability to understand its development and therefore it is a challenge to design treatments and strategies to slow down or prevent the progress of longterm effects of SCI in children. In this dissertation, we studied the postural control using a Segmental Assessment Trunk Control (SATCo) test and respiratory motor control using Respiratory Motor Control Assessment (RMCA) protocol in typically developing (TD) children and compared their results to age-matched children with SCI.vii Chapter I describes the background information about the trunk and respiratory motor control and how the injury to spinal cord impacts these motor functions. Chapter II illustrates the specific aims and hypothesis of this dissertation. Methods and protocols used to ...

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The Value of Respiratory Muscle Testing in Children With Neuromuscular Disease

Cited by 32 publications

References 67 publications

Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy

Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy

Confiabilidad de la fuerza muscular respiratoria y flujos espiratorios forzados en adolescentes sanos

Trunk and respiratory motor control in typically developing children and its implications in children with chronic spinal cord injury.

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