Significant inconsistencies in respiratory care provision for Duchenne muscular dystrophy (DMD) are reported across different specialist neuromuscular centres in the UK. The absence of robust clinical evidence and expert consensus is a barrier to the implementation of care recommendations in public healthcare systems as is the need to increase awareness of key aspects of care for those living with DMD. Here, we provide evidenced-based and/or consensus-based best practice for the respiratory care of children and adults living with DMD in the UK, both as part of routine care and in an emergency.
METHODS: Initiated by an expert working group of UK-based respiratory physicians (including British Thoracic Society (BTS) representatives), neuromuscular clinicians, physiotherapist and patient representatives, draft guidelines were created based on published evidence, current practice and expert opinion. After wider consultation with UK respiratory teams and neuromuscular services, consensus was achieved on these best practice recommendations for respiratory care in DMD.
RESULTS: The resulting recommendations are presented in the form of a flow chart for assessment and monitoring, with additional guidance and a separate chart setting out key considerations for emergency management. The recommendations have been endorsed by the BTS.
CONCLUSIONS: These guidelines provide practical, reasoned recommendations for all those managing day-to-day and acute respiratory care in children and adults with DMD. The hope is that this will support patients and healthcare professionals in accessing high standards of care across the UK.

To determine reference values for maximum static respiratory pressures in healthy children from a Brazilian region, following recommendations of the European Respiratory Society (ERS) and the Brazilian Society of Pneumology and Tisiology (SBPT).
A cross-sectional observational study was conducted with healthy children (6 to 11 years) of both sexes. The maximum inspiratory and expiratory pressures (PImax and PEmax, respectively) were measured using a digital manometer. Each child performed a minimum of three and a maximum of five maneuvers; three acceptable and reproducible maneuvers were considered for analysis. Minimum time for each maneuver was 1.5 seconds, with a one-second plateau, and one minute of rest between them. A stepwise multiple linear regression analysis was conducted for PImax and PEmax, considering correlations between independent variables: age, weight, and sex.
We included 121 children (62 girls [51%]). Boys reached higher values for maximum respiratory pressures than girls. Respiratory pressures increased with age showing moderate effect sizes (PImax: f = 0.36; PEmax: f = 0.30) between the stratified age groups (6-7, 8-9, and 10-11 years). Age and sex were included in the PImax equation (PImax = 24.630 + 7.044 x age (years) + 13.161 x sex; R2 = 0.189). PEmax equations were built considering age for girls and weight for boys [PEmax (girls) = 55.623 + 4.698 x age (years) and PEmax (boys) = 82.617 + 0.612 x weight (kg); R2 = 0.068].
This study determined new reference equations for maximal respiratory pressures in healthy Brazilian children, following ERS and SBPT recommendations.

In amyotrophic lateral sclerosis (ALS), respiratory muscle weakness leads to respiratory failure. Non-invasive ventilation (NIV) maintains adequate ventilation in ALS patients. NIV alleviates symptoms and improves survival. In 2006, French guidelines established criteria for NIV initiation based on limited evidence. Their impact on clinical practice remains unknown. Our objective was to describe NIV initiation practices of the main French ALS tertiary referral centre with respect to guidelines. In this retrospective descriptive study, 624 patients followed in a single national reference centre began NIV between 2005 and 2013. We analysed criteria used to initiate NIV, including symptoms, PaCO2, forced vital capacity, maximal inspiratory pressures and time spent with SpO2 <90% at night. At NIV initiation, 90% of patients were symptomatic. Median PaCO2 was 48 mmHg. The main criterion to initiate NIV was \'symptoms\' followed by \'hypercapnia\' in 42% and 34% of cases, respectively. NIV was initiated on functional parameters in only 5% of cases. Guidelines were followed in 81% of cases. In conclusion, despite compliance with French guidelines, the majority of patients are treated at the stage of symptomatic daytime hypoventilation, which suggests that NIV is initiated late in the course of ALS. Whether this practice could be improved by changing guidelines or increasing respiratory-dedicated resources remains to be determined.

In patients with chronic obstructive pulmonary disease (COPD), skeletal muscle dysfunction is a major comorbidity that negatively impacts their exercise capacity and quality of life. In the current guidelines, the most recent literature on the various aspects of COPD muscle dysfunction has been included. The Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) scale has been used to make evidence-based recommendations on the different features. Compared to a control population, one third of COPD patients exhibited a 25% decline in quadriceps muscle strength, even at early stages of their disease. Although both respiratory and limb muscles are altered, the latter are usually more severely affected. Numerous factors and biological mechanisms are involved in the etiology of COPD muscle dysfunction. Several tests are proposed in order to diagnose and evaluate the degree of muscle dysfunction of both respiratory and limb muscles (peripheral), as well as to identify the patients\' exercise capacity (six-minute walking test and cycloergometry). Currently available therapeutic strategies including the different training modalities and pharmacological and nutritional support are also described.

BACKGROUND: The maximum static respiratory pressures, namely the maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP), reflect the strength of the respiratory muscles. These measures are simple, non-invasive, and have established diagnostic and prognostic value. This study is the first to examine the maximum respiratory pressures within the Brazilian population according to the recommendations proposed by the American Thoracic Society and European Respiratory Society (ATS/ERS) and the Brazilian Thoracic Association (SBPT).
OBJECTIVE: To establish reference equations, mean values, and lower limits of normality for MIP and MEP for each age group and sex, as recommended by the ATS/ERS and SBPT.
METHODS: We recruited 134 Brazilians living in Belo Horizonte, MG, Brazil, aged 20-89 years, with a normal pulmonary function test and a body mass index within the normal range. We used a digital manometer that operationalized the variable maximum average pressure (MIP/MEP). At least five tests were performed for both MIP and MEP to take into account a possible learning effect.
RESULTS: We evaluated 74 women and 60 men. The equations were as follows: MIP=63.27-0.55 (age)+17.96 (gender)+0.58 (weight), r(2) of 34% and MEP= - 61.41+2.29 (age) - 0.03(age(2))+33.72 (gender)+1.40 (waist), r(2) of 49%.
CONCLUSIONS: In clinical practice, these equations could be used to calculate the predicted values of MIP and MEP for the Brazilian population.

Bronchopulmonary C-fibers and a subset of mechanically sensitive, acid-sensitive myelinated sensory nerves play essential roles in regulating cough. These vagal sensory nerves terminate primarily in the larynx, trachea, carina, and large intrapulmonary bronchi. Other bronchopulmonary sensory nerves, sensory nerves innervating other viscera, as well as somatosensory nerves innervating the chest wall, diaphragm, and abdominal musculature regulate cough patterning and cough sensitivity. The responsiveness and morphology of the airway vagal sensory nerve subtypes and the extrapulmonary sensory nerves that regulate coughing are described. The brainstem and higher brain control systems that process this sensory information are complex, but our current understanding of them is considerable and increasing. The relevance of these neural systems to clinical phenomena, such as urge to cough and psychologic methods for treatment of dystussia, is high, and modern imaging methods have revealed potential neural substrates for some features of cough in the human.

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