The act of breathing depends on coordinated activity of the respiratory muscles to generate subatmospheric pressure. This action is compromised by disease states affecting anatomical sites ranging from the cerebral cortex to the alveolar sac. Weakness of the respiratory muscles can dominate the clinical manifestations in the later stages of several primary neurologic and neuromuscular disorders in a manner unique to each disease state. Structural abnormalities of the thoracic cage, such as scoliosis or flail chest, interfere with the action of the respiratory muscles-again in a manner unique to each disease state. The hyperinflation that accompanies diseases of the airways interferes with the ability of the respiratory muscles to generate subatmospheric pressure and it increases the load on the respiratory muscles. Impaired respiratory muscle function is the most severe consequence of several newly described syndromes affecting critically ill patients. Research on the respiratory muscles embraces techniques of molecular biology, integrative physiology, and controlled clinical trials. A detailed understanding of disease states affecting the respiratory muscles is necessary for every physician who practices pulmonary medicine or critical care medicine.

Disorders of the Respiratory Muscles

Assessing respiratory mechanics and muscle function is critical for both clinical practice and research purposes. Several methodological developments over the past two decades have enhanced our understanding of respiratory muscle function and responses to interventions across the spectrum of health and disease. They are especially useful in diagnosing, phenotyping and assessing treatment efficacy in patients with respiratory symptoms and neuromuscular diseases. Considerable research has been undertaken over the past 17 years, since the publication of the previous American Thoracic Society (ATS)/European Respiratory Society (ERS) statement on respiratory muscle testing in 2002. Key advances have been made in the field of mechanics of breathing, respiratory muscle neurophysiology (electromyography, electroencephalography and transcranial magnetic stimulation) and on respiratory muscle imaging (ultrasound, optoelectronic plethysmography and structured light plethysmography). Accordingly, this ERS task force reviewed the field of respiratory muscle testing in health and disease, with particular reference to data obtained since the previous ATS/ERS statement. It summarises the most recent scientific and methodological developments regarding respiratory mechanics and respiratory muscle assessment by addressing the validity, precision, reproducibility, prognostic value and responsiveness to interventions of various methods. A particular emphasis is placed on assessment during exercise, which is a useful condition to stress the respiratory system.

https://erj.ersjournals.com/content/erj/53/6/1801214.full.pdf

ERS statement on respiratory muscle testing at rest and during exercise

Chronic obstructive pulmonary disease (COPD) is a major health care problem. Formerly mainly a disease of men, women are increasingly frequently afflicted. In many of these patients, exercise intolerance is the chief complaint. Few effective therapies are available. In recent years, dysfunction of the muscles of ambulation has been identified as a source of a portion of the exercise intolerance these patients experience, and this dysfunction has been shown to be, at least in part, remediable. Mechanisms inducing muscle dysfunction include disuse atrophy, malnutrition, low levels of anabolic steroids, and myopathy from corticosteroid use. Endurance exercise training has been conclusively demonstrated to improve exercise tolerance in COPD. Recent studies suggest that strength training is beneficial as well. A new frontier of therapy for muscle dysfunction in COPD is the use of anabolic hormones. Testosterone supplementation has been shown to increase muscle mass and strength in both hypogonadal and eugonadal healthy men. Low-dose testosterone supplementation is being considered for use in postmenopausal women. Though short-term administration of testosterone in moderate doses seems to be well tolerated in both men and women, further studies are required before safety and effectiveness can be established for routine use in COPD patients.

Skeletal muscle dysfunction in chronic obstructive pulmonary disease.

The use of ultrasonography has become increasingly popular in the everyday management of critically ill patients. It has been demonstrated to be a safe and handy bedside tool that allows rapid hemodynamic assessment and visualization of the thoracic, abdominal and major vessels structures. More recently, M-mode ultrasonography has been used in the assessment of diaphragm kinetics. Ultrasounds provide a simple, non-invasive method of quantifying diaphragmatic movement in a variety of normal and pathological conditions. Ultrasonography can assess the characteristics of diaphragmatic movement such as amplitude, force and velocity of contraction, special patterns of motion and changes in diaphragmatic thickness during inspiration. These sonographic diaphragmatic parameters can provide valuable information in the assessment and follow up of patients with diaphragmatic weakness or paralysis, in terms of patient–ventilator interactions during controlled or assisted modalities of mechanical ventilation, and can potentially help to understand post-operative pulmonary dysfunction or weaning failure from mechanical ventilation. This article reviews the technique and the clinical applications of ultrasonography in the evaluation of diaphragmatic function in ICU patients.

/pdf/sonographic-evaluation-of-the-diaphragm-in-critically-ill-45nerj5yfh.pdf

Sonographic evaluation of the diaphragm in critically ill patients. Technique and clinical applications.

Rationale: Diaphragm atrophy and dysfunction have been reported in humans during mechanical ventilation, but the prevalence, causes, and functional impact of changes in diaphragm thickness during routine mechanical ventilation for critically ill patients are unknown.Objectives: To describe the evolution of diaphragm thickness over time during mechanical ventilation, its impact on diaphragm function, and the influence of inspiratory effort on this phenomenon.Methods: In three academic intensive care units, 107 patients were enrolled shortly after initiating ventilation along with 10 nonventilated intensive care unit patients (control subjects). Diaphragm thickness and contractile activity (quantified by the inspiratory thickening fraction) were measured daily by ultrasound.Measurements and Main Results: Over the first week of ventilation, diaphragm thickness decreased by more than 10% in 47 (44%), was unchanged in 47 (44%), and increased by more than 10% in 13 (12%). Thickness did not vary over time follow...

Evolution of Diaphragm Thickness during Mechanical Ventilation. Impact of Inspiratory Effort

BACKGROUND: There is little information on the morphometric characteristics of the diaphragm in patients with Duchenne muscular dystrophy. METHODS: The thickness of the diaphragm was measured at the zone of apposition using B mode ultrasonography in 10 boys with Duchenne muscular dystrophy of mean (SD) age 10.3 (1.3) years and 12 normal controls of mean (SD) age 11.3 (2.0) years during relaxation (DiTrelax) and during maximum effort inspiratory manoeuvres (DiTPimax) at functional residual capacity. RESULTS: DiTrelax was greater in the patients with Duchenne muscular dystrophy (1.74 (0.21) mm) than in controls (1.48 (0.20) mm), mean difference (95% CI) 0.26 (0.08 to 0.44), despite considerable impairment of maximum effort inspiratory mouth pressure (Pimax) (patients with Duchenne muscular dystrophy -37 (8) cm H2O, controls -80 (33) cm H2O), mean difference (95% CI) 43 (65 to 20). During a Pimax manoeuvre, compared with measurements taken during relaxation, the diaphragm thickened 1.6 times in patients with Duchenne muscular dystrophy and 2.3 times in controls (DiTPimax 2.62 (0.7) mm and 3.5 (0.85) mm, respectively), mean difference (95% CI) -0.88 (-1.58 to -0.18). CONCLUSIONS: Resting diaphragm thickness is increased in young patients with Duchenne muscular dystrophy with impaired respiratory muscle force. This finding could be analogous to the pseudo-hypertrophy that is observed in some limb muscle groups.

https://thorax.bmj.com/content/thoraxjnl/52/5/472.full.pdf

Diaphragm thickness and inspiratory strength in patients with Duchenne muscular dystrophy.

There have been few studies of respiratory and limb muscle size and function in middle-aged patients with asthma and persistent airways obstruction. We have compared the forces generated by the respiratory and thigh muscles with their dimensions assessed by ultrasound in nine middle-aged patients with chronic asthma (mean age 56 (SD 8) yrs; functional residual capacity/total lung capacity ratio (FRC/TLC) 60 (10)%), and in nine normal subjects (aged 53 (7) yrs; FRC/TLC 55 (5)%). Diaphragm thickness was measured at the zone of apposition by B-mode ultrasound during relaxation (DiTrelax) and during a maximum-effort inspiratory manoeuvre (DiTpI,max) at FRC. Cross-sectional area of the relaxed rectus femoris muscle (ARF) was determined by ultrasound at mid-thigh level. Isometric strength of the right quadriceps muscle group was measured during maximum voluntary contraction. Asthmatic patients had preserved quadriceps strength and ARF but moderately impaired maximum inspiratory pressure (PI,max) (-52 (18) cmH2O) and thicker DiTrelax (2.2 (0.4) mm), compared to normal subjects (-73 (21) cmH2O and 1.7 (0.3) mm, respectively). Middle-aged patients with chronic asthma and a small increase in functional residual capacity/total lung capacity ratio have preserved limb muscle force and dimensions, modestly impaired inspiratory muscle strength, and slightly increased thickness of the costal diaphragm. Future studies of respiratory muscle function in asthma should be aided by measurement of diaphragm thickness and of limb muscle strength and size. Such studies are required particularly in older patients with severe hyperinflation who are most likely to have impairment of muscle function.

https://erj.ersjournals.com/content/erj/10/1/59.full.pdf

Size and strength of the respiratory and quadriceps muscles in patients with chronic asthma

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Diaphragm thickness and inspiratory strength in patients with Duchenne muscular dystrophy.

Size and strength of the respiratory and quadriceps muscles in patients with chronic asthma