Pulmonary function testing in patients with neuromuscular disease

Miller, Kelly R.; Mayer, Oscar H.

doi:10.1002/ppul.25182

Cited by 9 publications

(13 citation statements)

References 48 publications

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“…Point-of-care measurement of oxy-hemoglobin saturation and end-tidal or transcutaneous carbon dioxide are also commonly assessed. 18,23 Chronic respiratory failure in NMD begins with nocturnal hypoventilation and hypoxemia. As neuromuscular function worsens, respiratory disruptions first occur during the rapid eye movement (REM) stages of sleep, followed by non-REM (NREM) stages, and eventually during the wakeful period.…”

Section: Pulmonary Function Testingmentioning

confidence: 99%

“…Moreover, PFTs offer objective milestones to prompt timely intervention and supportive treatments, and thus help reduce significant respiratory morbidity. Measurements of lung volumes, flows, pressure, and gas exchange each provide vital information about the functional status of the respiratory system 17–19 . Herein, we review the common PFT findings in pediatric NMD.…”

Section: Introductionmentioning

confidence: 99%

“…Maximal expiratory pressure (MEP) is obtained with maximal exhalation from TLC and measures abdominal muscle strength. A third option is sniff nasal inspiratory pressure (SNIP), which is assessed by maximum inhalation from FRC 18,20 …”

Section: Introductionmentioning

confidence: 99%

“…2. Respiratory Flows: Measurements of respiratory flow may indicate respiratory muscle strength (peak expiratory flow or PEF) or airway clearance capability (peak cough flow or PCF) 18,21,22 …”

Section: Introductionmentioning

confidence: 99%

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ATS Core Curriculum 2022. Pediatric Pulmonary Medicine: Updates in pediatric neuromuscular disease

Leon‐Astudillo

Okorie

McCown

et al. 2023

Pediatric Pulmonology

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The American Thoracic Society Core Curriculum updates clinicians annually in pediatric pulmonary disease. This is a concise review of the Pediatric Pulmonary Medicine Core Curriculum presented at the 2022 American Thoracic Society International Conference. Neuromuscular diseases (NMD) comprise a variety of conditions that commonly affect the respiratory system and cause significant morbidity including dysphagia, chronic respiratory failure, and sleep disordered breathing. Respiratory failure is the most common cause of mortality in this population. Substantial progress has been made in diagnosis, monitoring and treatment for NMD over the last decade. Pulmonary function testing (PFT) is utilized to objectively measure respiratory pump function and PFT milestones are utilized in NMD‐specific pulmonary care guidelines. New disease modifying therapies are approved for the treatment of patients with Duchenne muscular dystrophy and spinal muscular atrophy (SMA), including the first ever approved systemic gene therapy, in the case of SMA. Despite extraordinary progress in the medical management of NMD, little is known regarding the respiratory implications and long‐term outcomes for patients in the era of advanced therapeutics and precision medicine. The combination of technological and biomedical advancements has increased the complexity of the medical decision‐making process for patients and families, thus emphasizing the importance of balancing respect for autonomy with the other foundational principles of medical ethics. This review features an overview of PFT, noninvasive ventilation strategies, novel and developing therapies, as well as the ethical considerations specific to the management of patients with pediatric NMD.

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Section: Pulmonary Function Testingmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

ATS Core Curriculum 2022. Pediatric Pulmonary Medicine: Updates in pediatric neuromuscular disease

Leon‐Astudillo

Okorie

McCown

et al. 2023

Pediatric Pulmonology

View full text Add to dashboard Cite

show abstract

“…Determination of PEF and PCF can be applied to estimate respiratory muscle strength if maximal static mouth pressures cannot be performed ( Suárez et al, 2002 ). Patients with PCF below 160 L/min benefit from mechanical in-exsufflation ( Bach and Saporito, 1996 ; Bach et al, 1997 ; Polkey and Moxham, 2001 ; Suárez et al, 2002 ; Miller and Mayer, 2021 ).…”

Section: Evaluation Of Respiratory Muscle Function and Physiologic Ch...mentioning

confidence: 99%

Methods and Applications in Respiratory Physiology: Respiratory Mechanics, Drive and Muscle Function in Neuromuscular and Chest Wall Disorders

2022

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Individuals with neuromuscular and chest wall disorders experience respiratory muscle weakness, reduced lung volume and increases in respiratory elastance and resistance which lead to increase in work of breathing, impaired gas exchange and respiratory pump failure. Recently developed methods to assess respiratory muscle weakness, mechanics and movement supplement traditionally employed spirometry and methods to evaluate gas exchange. These include recording postural change in vital capacity, respiratory pressures (mouth and sniff), electromyography and ultrasound evaluation of diaphragmatic thickness and excursions. In this review, we highlight key aspects of the pathophysiology of these conditions as they impact the patient and describe measures to evaluate respiratory dysfunction. We discuss potential areas of physiologic investigation in the evaluation of respiratory aspects of these disorders.

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Duchenne muscular dystrophy respiratory profiles from real world registry data

Hnaini

Downs

Miller

et al. 2023

Pediatric Pulmonology

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IntroductionUnderstanding real‐world profiles from neuromuscular databases is helpful for optimizing clinical care and planning research studies. The Canadian Neuromuscular Disease Registry (CNDR) has respiratory data from a population of boys with Duchenne Muscular Dystrophy (DMD).ObjectivesTo describe cross‐sectional respiratory profiles from a national DMD real‐world dataset. To explore the relationship between forced vital capacity percent predicted (FVC%) and disease severity parameters: scoliosis, ambulation and ventilation status.MethodsDescriptive statistics summarized the respiratory profiles. The CNDR registry enrolls and collects DMD clinic data from 36 Canadian centers.ResultsThere were 414 participants enrolled. The age ranged from 2 to 36 years old. Pulmonary function test data were available for 323 participants. The use of ventilatory support was seen in a significant proportion (19.5%) of subjects by age 14–16 years and was used by the majority (69.2%) by age 20–22 years. FVC% declined at a rate of 3.19% per year with every 1‐year increase in age. FVC% declined annually by 2.47% in nonambulatory participants versus by 0.96% in ambulatory participants. FVC% did not significantly change over age with the presence of scoliosis or use of ventilatory technology.ConclusionsThe data from this large cohort are valuable for understanding real‐world patterns of clinical care and disease progression. There is a significant association between the loss of ambulation and the rate of FVC% decline. Further longitudinal studies are needed to better understand the impact of disease parameters on pulmonary function decline and the need for ventilatory support.

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Pulmonary function testing in patients with neuromuscular disease

Cited by 9 publications

References 48 publications

ATS Core Curriculum 2022. Pediatric Pulmonary Medicine: Updates in pediatric neuromuscular disease

ATS Core Curriculum 2022. Pediatric Pulmonary Medicine: Updates in pediatric neuromuscular disease

Methods and Applications in Respiratory Physiology: Respiratory Mechanics, Drive and Muscle Function in Neuromuscular and Chest Wall Disorders

Duchenne muscular dystrophy respiratory profiles from real world registry data

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