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Objectives: The bubble‐positive expiratory pressure (PEP) device may be used for sputum clearance in people with daily sputum production. However, this device has never been studied in people with bronchiectasis. Hence, the objective of this study was to compare the effect of bubble‐PEP device, the active cycle of breathing technique (ACBT) and no intervention (control) on sputum clearance in people with bronchiectasis. Methods: This was a prospective, randomised cross‐over trial with concealed allocation, assessor blinding and intention‐to‐treat analysis. Adult participants with stable bronchiectasis and productive of sputum daily were recruited. Participants performed 30‐min of bubble‐PEP, ACBT or control in random order whilst sitting, followed by 60‐min of quiet sitting, on three separate days at the same time within a 10‐day period. Primary outcome measure was wet weight of expectorated sputum during 30‐min intervention, 60‐min post intervention and total wet weight (30 min plus 60 min). Results: Thirty‐five‐participants (11 males, mean [standard deviation] age 75 [8] years, forced expiratory volume in 1 s 72 [20] % predicted) were recruited and 34 completed the study. There was no significant difference in sputum wet weight between bubble‐PEP and ACBT during 30‐min intervention (mean difference [95% confidence interval]) −0.59 g [−1.37, 0.19] and total wet weight (0.74 g [−0.54, 2.02]). Sputum wet weight was significantly greater in bubble‐PEP than ACBT at 60‐min post intervention (1.33 g [0.19, 2.47]). Conclusion: Sputum wet weight was significantly greater with bubble‐PEP than control at all time periods, and greater than ACBT at 60‐min‐post. Bubble‐PEP could be considered an alternative sputum clearance technique to ACBT. [ABSTRACT FROM AUTHOR]

Background: High frequency chest wall oscillation (HFCWO) has long been used for airway clearance for patients with cystic fibrosis. Only limited research has evaluated this therapy in adult patients with non-cystic fibrosis bronchiectasis (NCFB). Methods: Data from 2596 patients from a registry of adult bronchiectasis patients using HFCWO therapy was used to evaluate hospitalization patterns before and after initiation of HFCWO therapy, as well as antibiotic use and self-reported metrics of quality of life. Self-reported outcomes were also reviewed by cross-checking with sampled patient charts and found to be consistent. Results: The number of patients who had at least one respiratory-related hospitalization decreased from 49.1% (192/391) in the year before to 24.0% (94/391) in the year after starting HFCWO therapy (p -value < 0.001). At the same time, the number of patients who required three or more hospitalizations dropped from 14.3% (56/391) to 5.6% (22/391). Patients currently taking oral antibiotics for respiratory conditions decreased from 57.7% upon initiation of therapy to 29.9% within 1 year (p < 0.001). Patients who subjectively rated their "overall respiratory health" as good to excellent increased from 13.6% upon initiation of therapy to 60.5% in 1 year (p < 0.001) and those who rated their "ability to clear your lungs" as good to excellent increased from 13.9% to 76.6% (p < 0.001). Conclusion: NCFB patients showed improved self-reported outcomes associated with the initiation of HFCWO therapy as measured by number of hospitalizations, antibiotic use, and the subjective experience of airway clearance. The improvement was observed early on after initiation of therapy and sustained for at least 1 year. The reviews of this paper are available via the supplemental material section. [ABSTRACT FROM AUTHOR]

Non-adherence to prescribed treatment is considered the foremost cause of treatment failure in chronic medical conditions. Airway clearance techniques (ACT) play a key role in the management of chronic suppurative lung disease yet, along with inhaled therapies such as nebulised antibiotics, adherence to these is often lower than to other treatments. In this review we discuss methods of monitoring adherence to these therapies and potential barriers and outline suggestions for improving adherence in the paediatric population. [ABSTRACT FROM AUTHOR]

Cough is an important mechanism of airway clearance. In patients who present weak and ineffective cough, augmentation techniques aim to assist or simulate the maneuver. These techniques target different phases of the cough cycle, mainly the inspiratory and expiratory phases, through assisted inspiration, assisted expiration and their combination. They include the manual hyperinflation, ventilator hyperinflation, glossopharyngeal breathing, manually assisted cough and mechanical insufflatorexsufflator, each applied individually or in different combinations. The aim of this review is to investigate the effectiveness and safety of cough augmentation techniques. Findings support that all commonly used techniques can theoretically improve airway clearance, as they generate higher cough peak flows compared to unassisted cough. Still, the studies assessing cough augmentation present considerable limitations and the direct comparison of different techniques is challenging. Current evidence indicate that cough peak flow shows higher increase with the combination of assisted inspiration and expiration, and improvement is greater in patients with lower unassisted values. Associated adverse events are infrequent. [ABSTRACT FROM AUTHOR]

Respiratory physiotherapy and rehabilitation are important therapeutic options in non-cystic fibrosis bronchiectasis (NCFB). The aims of this review of clinical trials were to evaluate the safety and the effects on physiologic and clinical outcomes of airway clearance techniques (ACTs) and rehabilitation in NCFB patients, in comparison to usual care. The search was performed on March 2018 by using PubMed and PeDro databases. 33 studies were selected. The use of ACTs for NCFB were effective in increasing sputum volume although no benefit in quality of life (QoL) or pulmonary exacerbations were observed. There were no differences in effectiveness between the several techniques used. Humidification and saline inhalation were able to aid airway clearance. Hypertonic solution (HS) was more effective than isotonic solutions (IS) in improving expectoration and sputum viscosity. Pulmonary rehabilitation (PR) was found to be associated with short term benefits in exercise capacity, dyspnea and fatigue. Exercise training seems to improve quality of life and lower exacerbation rate, but long-term data are not available. Further studies are necessary to identify the most feasible long-term outcomes such as QoL and exacerbation rate. [ABSTRACT FROM AUTHOR]

Patients with nontuberculous mycobacterial (NTM) lung infection require life-long attention to their bronchiectasis, whether or not their NTM infection has been cured. The identification of the cause of bronchiectasis and/or coexisting diseases is important because it may affect therapeutic strategies. Airway clearance is the mainstay of bronchiectasis management. It can include multiple breathing techniques, devices, and mucoactive agents. The exact airway clearance regimen should be customized to each individual patient. Chronic pathogenic airway bacteria, such as Pseudomonas aeruginosa, may warrant consideration of eradication therapy and/or chronic use of maintenance inhaled antibiotics., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Atelectasis is a common problem in intensive care units. It usually occurs due to airway obstruction caused by pulmonary secretions. As the left main bronchus angulates from carina more sharply, suctioning of the secretions from left side is less effective, causing failure in treatment of left lung atelectases. If chest physiotherapy and medical treatment fail, bronchoscopy should be considered; but it requires experience, time, effort and a higher cost. Objectives: To evaluate the success of a novel method "selective left bronchial aspiration (SeLBA)" in the treatment of left lung atelectases. Methods: The study consisted of 10 patients who suffered left lung atelectasis and treated with double catheter suctioning technique, SeLBA in Pediatric Intensive Care Unit of Hacettepe University Ihsan Dogramaci Children's Hospital between June 2017 and February 2019. The outcomes were evaluated by comparing the chest X‐rays before and after the procedure. Results: All of the patients' atelectases resolved successfully by SeLBA procedure. Conclusion: We introduce this novel technique of reopening the atelectased left lung as a rapid, safe and practical alternative for bronchoscopic clear away. [ABSTRACT FROM AUTHOR]

Introduction/Objectives: The aim of our study was to assess the safety, efficacy and feasibility of an Expiratory Flow Accelerator (EFA) device, Free Aspire, in reducing the need for daily suctions in tracheostomised (TCS) patients. Methods: Twenty‐five patients (13 males, 12 females, aged 69.88 ± 9.06 years) were investigated. The number of superficial/deep suctions, shallows, arterial blood gas (ABG) analysis, perception of mucus encumbrance (Visual Numeric Scale, VNS) and adverse event were recorded for five days. On the first two days (T1‐T2), suctioning was performed as usual, on the following three days (T3, T4, T5), patients were treated also with Free Aspire (20 min, 3 times a day). Results/Conclusion: The use of the non‐invasive device was associated with a decrease of total number of aspirations from T2 to T5 (8.48 ± 2.62 vs 4.48 ± 3.08, P = 0.0003). Total number of daily aspirations decreased over the five days (8.68 ± 3.64 vs 4.48 ± 3.08, P = 0.0009). Deep aspirations decreased from T1 to T5 (6.16 ± 3.53 vs 1.80 ± 1.50, P = 0.0001). ABG data confirmed that no significant side effects occurred. VNS score decreased from 7.03 ± 1.42 at T1 to 4.05 ± 1.80 at T5 (P < 0.0001). These data suggest that EFA may be useful for managing secretions in TCS patients, as it can reduce the number of daily suctions, particularly the deep ones, and may improve the perception of mucus encumbrance, without side effects. More studies are needed to confirm these data and to understand in which categories of TCS patients this device can be introduced. [ABSTRACT FROM AUTHOR]

Physiotherapy has been accepted as an integral part of critical care in developed countries. Preventing and managing pulmonary complications of ventilated and other critically ill patients is still considered to be the primary task of physiotherapists in India. As movement experts of the intensive care unit team, physiotherapists play a major role in preventing neuromuscular complications through early mobilization and therapeutic exercise prescription in the developed countries. This critical review analyses the physiological rationale behind the physiotherapeutic technique and existing research evidence pertaining to the role of physiotherapy in critical care. The paper also highlights the simple evidence-based protocol which was successfully implemented in a tertiary care hospital of India. [ABSTRACT FROM AUTHOR]

The mechanisms by which transepithelial pressure changes observed during exercise and airway clearance can benefit lung health are challenging to study. Here, we have studied 117 mature, fully ciliated airway epithelial cell filters grown at air-liquid interface grown from 10 cystic fibrosis (CF) and 19 control subjects. These were exposed to cyclic increases in apical air pressure of 15 cmH2O for varying times. We measured the effect on proteins relevant to lung health, with a focus on the CF transmembrane regulator (CFTR). Immunoflourescence and immunoblot data were concordant in demonstrating that air pressure increased F508Del CFTR expression and maturation. This effect was in part dependent on the presence of cilia, on Ca2+ influx, and on formation of nitrogen oxides. These data provide a mechanosensory mechanism by which changes in luminal air pressure, like those observed during exercise and airway clearance, can affect epithelial protein expression and benefit patients with diseases of the airways. [ABSTRACT FROM AUTHOR]

Primary benefits of high flow nasal cannula therapy include washout of carbon dioxide rich exhaled gas and increased airway pressures during tidal breathing. This work reports on the influence of high flow nasal cannula outlet area on upper airways gas clearance and tracheal pressures using measurements in five realistic adult nose-throat airway replicas. Two commercial high flow nasal cannulas and one generic nasal cannula of varying size were compared. 100% oxygen was supplied via cannulas at flow rates ranging from 30 to 90 l/min to replicas originally filled with air, and oxygen concentrations at the larynx and trachea were compared over time. Additionally, and separately, replicas were connected to a mechanical lung simulator to simulate tidal breathing while undergoing high flow nasal cannula therapy, with tracheal pressure–time waveforms recorded. Faster gas clearance corresponded with higher flow rates (P < 0.001), and with smaller cannula outlet area (P < 0.001). Observed pressures were in approximate agreement with limited available in-vivo data in the literature. Between 0 and 60 L/min cannula flow rates, tracheal positive end expiratory pressures increase was greater with the smallest cannula (∆ P PEEP = 785 SD(185) Pa) compared to the largest cannula (∆ P PEEP = 380 SD(120) Pa). Regression analysis indicates that positive end expiratory pressure is proportional to the square of flow velocities exiting the cannula and nares (R2 = 0.906). Since increased pressure and clearance rate have been associated with improved clinical outcomes in previous studies, our results suggest that smaller cannula outlet area may be preferable. [ABSTRACT FROM AUTHOR]

Bronchiectasis is an important clinical syndrome because of its increasing prevalence, substantial economic burden on health care, and associated morbidity. Until recently, the disease was considered an orphan and essentially neglected from a therapeutic standpoint, but many recent advances have been made in the field. Several national registries have formed to provide databases from which to study patients with bronchiectasis. Experts published a consensus definition of a bronchiectasis-specific exacerbation that will serve as a unified definition for future clinical trials. Several inhaled antibiotic trials aimed at reducing exacerbation frequency have been completed. Researchers have investigated nonculture techniques, such as 16S ribosomal RNA (rRNA) and whole genome sequencing, to characterize the microbiological characteristics. Studies of anti-Pseudomonas antibodies are providing interesting insight into varying host responses to chronic Pseudomonas infection. After three successful trials demonstrating that macrolides reduce exacerbations in bronchiectasis, other antiinflammatory agents have been investigated, and a trial of a novel antiinflammatory drug is ongoing. A relatively robust study has been published in airway clearance, a therapy that is accepted universally as beneficial but that has never been accompanied by strong evidence. To build on the successes with bronchiectasis thus far, investigators must develop better definitions of phenotypes of bronchiectasis. In this regard, clinical tools have been developed to quantify disease severity and predict prognosis. Studies of different clinical phenotypes of bronchiectasis in patients with bronchiectasis have been published. With continued advances in the field of bronchiectasis, there is hope that evidenced-based therapies will become available. [ABSTRACT FROM AUTHOR]

BACKGROUND: Positive expiratory pressure (PEP) devices are widely used in clinical settings, yet the performance characteristics of these devices remain relatively unknown. This study compared the performance characteristics of 6 airway clearance devices by varying resistance and flow. METHODS: Mean PEP, peak PEP, oscillation frequency, and amplitude PEP of the Flutter, Pari PEP S, Acapella Choice, Acapella DM, Acapella DH, and Aerobika devices were obtained across flows of 5, 10, 15, 20, 25 and 30 L/min and at low, medium, and high resistance using an experimental apparatus custom-built for this bench study. RESULTS: Performance characteristics of the devices differed across flows and resistance settings (device x flow/resistance interaction; P < .001). At a fixed resistance, increasing flows increased mean PEP produced by the Acapella Choice, Acapella DH, Aerobika, and Pari PEP S. Increasing flow resulted in minimal change in mean PEP produced by the Flutter and Acapella DM. Increasing flow increased peak PEP and amplitude PEP produced by all devices except the Acapella DH and Acapella Choice. Increasing flow maintained or increased oscillation frequency for all devices except the Flutter. At a fixed flow, increasing resistance increased mean PEP produced by all devices except the Acapella Choice. Increasing resistance increased peak PEP produced by the Acapella DM, Aerobika, and Pari PEP S but resulted in minimal change in peak PEP for the Flutter and Acapella Choice. Increasing resistance either maintained or increased oscillation frequency for all devices. Amplitude PEP was either maintained or increased during oscillations when increasing resistance for all devices except the Flutter. CONCLUSIONS: PEP devices produced small but statistically significant variations in performance characteristics across a range of flows and resistance settings. There appear to be flow-dependent and non--flow-dependent devices. Varying flow or resistance typically maintained or increased the production of mean, peak, and amplitude PEP and oscillation frequency. [ABSTRACT FROM AUTHOR]

BACKGROUND: Oscillating positive expiratory pressure devices aid removal of excess secretions and reduce gas trapping in patients with hypersecretory pulmonary diseases, for example, cystic fibrosis. Oscillating positive expiratory pressure works when the patient exhales actively against a fixed resistor, which generates mean intrapulmonary pressures of 10-20 cm H2O with rapid fluctuations of at least 1 cm H2O from the mean. In this study, we evaluated the performance of oscillating positive expiratory pressure therapy by pediatric subjects with cystic fibrosis to determine adherence to target therapeutic pressures. METHODS: Twenty-one pediatric subjects were recruited. Each had a history of using an oscillating positive expiratory pressure device twice daily and had received standardized training and instructions from the same specialist physiotherapist. Performance was evaluated by using a flow and pressure sensor placed in-line between the participant's mouth and the device. The participants performed expirations as per their normal routine. RESULTS: None of the participants achieved target therapeutic pressure ranges during expiration. The mean ± SD pressure generated was 16.2 ± 6.8 cm H2O, whereas mean ± SD flow was 31.3 ± 8.9 L/min. The mean ± SD expiration length was 2.5 ± 1.4 s. CONCLUSIONS: Despite standardized instruction, the results demonstrated considerable variation among the participants and overall poor technique during use. Outcomes of this study indicated that airway clearance effects of oscillating positive expiratory pressure were compromised due to poor technique. [ABSTRACT FROM AUTHOR]

Abstract Many children seen under the umbrella of respiratory medicine benefit from physiotherapy. Physiotherapists bring a wide range of skills in the clinical management of this group of patients. These roles include secretion or airway clearance, breathing re-education, inhaled therapy, education for respiratory health, postural and musculoskeletal management, exercise prescription, support with weaning ventilation and admission prevention. Within this article we aim to explore some of the key roles physiotherapists undertake. [ABSTRACT FROM AUTHOR]

This paper aims to provide physiological rationale for airway clearance, mucoactive therapy and pulmonary rehabilitation (PR) (or exercise interventions) in bronchiectasis. There is increasing emphasis on the role of airway clearance techniques (ACT) in the management of bronchiectasis. No single ACT has currently shown superior effect over another. Given the large range of different techniques available, consideration of the physiological effects underpinning a technique including expiratory flow, ventilation and oscillation, is essential to effectively personalize ACT. Key clinical trials of mucoactives in bronchiectasis are underway and will provide clarity on the role of these agents in the management of patients with bronchiectasis. Prescription of mucoactive therapies should be done in conjunction with ACT and therefore the mechanism of action of mucoactive drugs and their timing with ACT should be taken into consideration. PR and/or exercise training are recommended in all current bronchiectasis guidelines. There is a clear physiological rationale that muscle weakness and physical inactivity may play a role in disease progression as well as impacting health‐related quality of life, frequency of pulmonary exacerbations and ability to mobilize sputum. However, there are residual unanswered questions surrounding the delivery and accessibility to PR. This review summarizes the physiological principles and supporting evidence for airway clearance, mucoactive medication and PR, which are key components in the management of bronchiectasis. [ABSTRACT FROM AUTHOR]

Amyotrophic lateral sclerosis is a progressive neuromuscular disease characterized by both lower motor neuron and upper motor neuron dysfunction. Although clinical presentations can vary, there is no cure for ALS, and the disease is universally terminal, with most patients dying of respiratory complications. Patients die, on average, within 3 to 5 years of diagnosis, unless they choose to undergo tracheostomy, in which case, they may live, on average, 2 additional years. Up to 95% of patients with ALS in the United States choose not to undergo tracheostomy; management of respiratory failure is therefore aimed at both prolonging survival as well as improving quality of life. Standard of care for patients with ALS includes treatment from multidisciplinary teams, but many patients do not have consistent access to a pulmonary physician who regularly sees patients with this disease. The goal of this review was to serve as an overview of respiratory considerations in the management of ALS. This article discusses noninvasive ventilation in the management of respiratory muscle weakness, mechanical insufflation/exsufflation devices for airway clearance, and treatment of aspiration, including timing of placement of a percutaneous endoscopic gastrostomy tube, as well as secretion management. In addition, it is important for physicians to consider end-of-life issues such as advanced directives, hospice referral, and ventilator withdrawal. [ABSTRACT FROM AUTHOR]

BACKGROUND: Regular monitoring combined with early and appropriate use of airway clearance can reduce unplanned hospital admissions for patients with neuromuscular disease (NMD) and spinal cord injury (SCI). We aimed to describe and compare knowledge of guidelines, monitoring of cough effectiveness, clinician prescription/provision of airway clearance strategies, and service provision constraints in the United Kingdom and Canada. METHODS: This was a cross-sectional survey of clinicians affiliated with NMD and SCI clinics in Canada, 2016 attendees at the Home Mechanical Ventilation Conference in the United Kingdom, and United Kingdom physiotherapist networks. RESULTS: We received 155 surveys (92 from Canada; 63 from the United Kingdom). More UK respondents (76%) were aware of airway clearance guidelines than Canadian (56%) respondents (P = .02). Routine assessment of cough effectiveness was reported by more UK respondents (59%) than Canadian (42%) respondents (P = .044). Cough peak flow (CPF) was the most common method used in both countries, although it was more commonly used in the UK (96%) than in Canada (81%, P = .02). Fewer Canadian respondents reported using CPF before initiation of airway clearance (81% vs 94%, P = .046), and fewer Canadian respondents showed results to patients for technique feedback (76% vs 97%, P = .007). Similar participant numbers reported using CPF after initiation to ensure adequate technique (73% vs 72%, P = .92). Mechanical insufflation-exsufflation (MI-E) + lung volume recruitment (LVR) + manually assisted cough when CPF ≤ 270 L/min was most routinely recommended (41% overall). Monotherapy was infrequent (LVR 15%, manually assisted cough 7%, and MI-E 4%). More Canadians identified constraints on service provision, specifically insufficient public funding for equipment (68% vs 39%, P = .002) and inadequate community workers' knowledge (56% vs 34%, P = .002). Funding for community support was a common constraint in both countries (49% vs 42%). CONCLUSIONS: The somewhat variable cough effectiveness monitoring and airway clearance practices identified in this survey confirm the need for further work on knowledge translation related to guideline recommendations and the need to address common constraints to optimal service delivery. [ABSTRACT FROM AUTHOR]

Introduction: Bronchiectasis is a chronic debilitating condition with abnormal permanent dilatation of the airways causing impaired mucus clearance, despite regular chest physiotherapy being mainstay of management for bronchiectasis, there is little evidence supporting regular chest physiotherapy in bronchiectasis which aims to mobilize secretions and facilitate effective expectoration, providing control of cough and improving airway clearance. The objective of this study was to compare between the efficacy of 2 techniques of chest physiotherapy ACBT with postural drainage and conventional chest physical therapy as a method of airway clearance in adults with productive bronchiectasis. Methods: The study included 30 subjects, 20 males and 10 females; all having bronchiectasis, the study was carried out on October 6 at the University Hospital. The participating subjects underwent conventional chest physical therapy or ACBT following postural drainage as the airway clearance technique in random order on 14 successive days with twice daily frequency. Results: There was a significant difference regarding mMRC before and after both ACBT and conventional physiotherapy, there was a significant improvement regarding FVC and MMEF after ACBT while there was a significant improvement of FEV1 and MMEF after conventional physiotherapy. As regards arterial blood gas data comparison, there were significant improvements regarding PaCO2, PaO2 and PAO2 while there was no significant difference as regards P (A-a) O2 after both types of physiotherapy techniques. Comparison between the 2 groups regarding mMRC dyspnea score, spirometry, arterial blood gas data, Leicester cough questionnaire (LCQ) and sputum wet volume before starting physiotherapy shows no significant difference while there were significant differences in advance to post ACBT physiotherapy sessions as regards PaO2, P (A-a) O2 gradient, LCQ (physical domain score and total score) and sputum wet volume. Conclusion: ACBT with postural drainage is found to be more effective than conventional chest physical therapy management of bronchiectasis during infective exacerbation.

Background: Patients receiving mechanical ventilation often require airway clearance and inhaled therapies. Intrapulmonary percussive ventilation (IPV) combines a high-frequency percussive ventilator with a jet nebulizer. Data on aerosol delivery efficiency of the device are scarce. We evaluated albuterol delivery efficiency while using an IPV in-line adapter under different conditions., Methods: A pediatric lung model of invasive mechanical ventilation was used. The following independent variables were evaluated: lung condition (normal vs ARDS), ventilator mode (adaptive pressure ventilation vs pressure control), percent opening of adapter (0% vs 25% vs 50%), IPV driving pressure (25 psi vs 40 psi), IPV percussion setting (easy vs hard), and endotracheal tube (ETT) size (3.5 mm vs 5.5 mm). Albuterol delivery efficiency (mass captured in the filter expressed as percentage of loading dose) was selected as the dependent variable. Albuterol was captured on a filter at the tip of the ETT and quantified via spectrophotometry (276 nm)., Results: Albuterol delivery efficiency ranged from 0-2.89%. Median (interquartile range) and 95% CI around the median were 0.54% (0.37-1.00) and 0.50-0.63%, respectively. The coefficient of determination (R 2 ) for the model including all variables was 0.363. The 2 main contributors were percent of adapter opening (R 2 0.30) and IPV setting (R 2 0.039)., Conclusions: Albuterol delivery during invasive mechanical ventilation via in-line IPV in a pediatric lung model was inefficient. Alternative methods of delivering bronchodilators and other inhaled medications should be considered when IPV is used., (Copyright © 2023 by Daedalus Enterprises.)

Background: Tracheobronchomalacia (TBM) is estimated to be present in 1 in 2100 children. Previous reports suggest the prevalence is higher in children with cystic fibrosis (CF). This has clinical implications with potential to influence airway clearance and lung health., Aim: To determine the prevalence and clinical associations of TBM in Western Australian children with CF., Methods: Children with CF born between 2001 and 2016 were included. Operation reports from bronchoscopies performed until the age of 4 were retrospectively reviewed. Data were collected on the presence, persistence defined as a repeat diagnosis, and severity of TBM. Data on genotype, pancreatic status, and symptoms at CF diagnosis were extracted from the medical record. Associations between categorical variables were compared using χ 2 and Fisher's exact test., Results: Of 167 children (79 male), 68 (41%) were diagnosed with TBM at least once, with TBM persistent in 37 (22%) and severe in 31 (19%). TBM was significantly associated with pancreatic insufficiency (χ 2  = 7.874, p < 0.05, odds ratio [OR] 3.4), delta F508 gene mutation (χ 2  = 6.489, p < 0.05, OR 2.3), and a presentation of meconium ileus (χ 2  = 8.615, p < 0.05, OR 5.0). Severe malacia was less likley in females (χ 2 = 4.523, p < 0.05, OR 0.42) . No significant relationship was found with respiratory symptoms at the time of CF diagnosis (χ 2  = 0.742, p = 0.39)., Conclusions: TBM was common in this group of children under the age of 4 with CF. A high index of suspicion for airway malacia should be considered in children with CF, particularly those who present with meconium ileus and have gastrointestinal symptoms at diagnosis., (© 2023 Wiley Periodicals LLC.)

BACKGROUND: Standard mechanical insufflation-exsufflation (MI-E) therapy is applied with fast insufflation-exsufflation pressures to achieve high peak expiratory flows (PEF) and assist airway clearance. No attention is given to the resultant high peak inspiratory flows (PIF), although it may impair secretion removal. It has been proposed that an expiratory flow bias (ie, PEF higher than PIF) might be the key determinant for mucus clearance instead of the PEF alone. We examined the effects of 2 MI-E maneuvers, standard versus optimized, with fast and slow insufflation, respectively, along with different MI-E pressure settings on secretion displacement in 3 lung-impedance scenarios that simulated a patient on mechanical ventilation. METHODS: The MI-E device was connected to a lung model that simulated a patient on mechanical ventilation. Known quantities of mucus simulant were injected into the system and exposed to various MI-E ventilation conditions. Mucus movement was examined with image-analysis software. RESULTS: The optimized MI-E maneuver resulted in a much lower PIF (37.5 L/min [interquartile range, 24.9-47.9 L/min] vs 101.8 L/min [interquartile range, 89.1-115.7 L/min], P < .001). Consequently, the expiratory flow bias, expressed by PEF:PIF and the PEF-PIF difference, was much higher in the optimized maneuver. The higher expiratory flow bias in the optimized maneuver displaced the mucus outward, with a difference of 2.6 cm compared with the standard maneuver. Multivariate analysis revealed that the type of maneuver (optimized vs standard), PEF-PIF difference and MI-E pressure gradient were significantly correlated with mucus displacement (r² = 0.817, P < .001), whereas the PEF was not. PEF:PIF and the PEF-PIF difference were lower in the obstructive lung scenario when compared with the restrictive and normal lung scenarios. CONCLUSIONS: The optimized MI-E maneuver, applied with slow insufflation, resulted in a higher expiratory flow bias, which made the therapy more effective at moving mucus outward, compared with the standard MI-E maneuver, typically applied with fast insufflation. [ABSTRACT FROM AUTHOR]

Nasotracheal suctioning (NTS) is a procedure commonly performed by respiratory physiotherapists and nurses to remove excess respiratory secretions from the tracheobronchial tree in a self-ventilating, non-intubated and non-tracheotomized patient. NTS is an important treatment modality for patients with acute secretion retention who are at high risk of progressive respiratory deterioration and arrest. However, NTS is a blind invasive procedure with risk of serious adverse events, and the patient experience of NTS is often extremely negative. Capsaicin, a substance extracted from cayenne pepper, elicits reflex coughs when inhaled. It is hypothesized that capsaicin-induced reflex cough may offer an alternative treatment option to NTS. It is suggested that repeated reflex cough bouts, elicited through inhalation of nebulized capsaicin via a facemask, could achieve clearance of retained secretions from the tracheobronchial tree to the oropharynx, thereby avoiding the need for NTS. This hypothesis is supported by preliminary cough flow data from a stroke trial. Stroke patients underwent assessments of peak cough flow (PCF, a measure of cough effectiveness) of both maximal volitional cough and capsaicin-induced reflex cough. In a sub-group of 20 stroke patients with weak volitional cough (mean PCF 220 L/min, SD 80), PCF of capsaicin-induced reflex cough was on average 184 L/min (SD 130) higher than PCF of subjects' maximal volitional cough effort. Cough flow traces indicate a pattern of cough augmentation during consecutive reflex cough bouts. It is suggested that the hypothesis may best be tested in a pragmatic applied clinical study, i.e. through the application of nebulized capsaicin in relevant clinical situations, as opposed to observational or experimental physiological studies. [ABSTRACT FROM AUTHOR]

BACKGROUND: Closed suction catheters are commonly used with pressurized metered-dose inhalers (pMDIs) in ventilator-dependent patients receiving bronchodilator therapy. Although heated/ humidified circuits result in a decrease in drug delivery, no studies have investigated the impact of a closed suction catheter with a pMDI on aerosol delivery during mechanical ventilation. We sought to quantify aerosol delivery with a variety of closed suction catheters and pMDI adapters in a simulated ventilator-dependent adult lung model with and without exhaled humidity. METHODS: A ventilator (tidal volume 450 mL, PEEP 5 cm H2O, breathing frequency 15 breaths/min, peak flow 60 L/min, and bias flow 2 L/min) was attached to 3 closed suction catheter designs (T-piece, double swivel elbow, and a multi-access port) with pMDI adapters (small- and large-unidirectional adapters, a bidirectional adapter, and a built-in port) connected to an endotracheal tube (ETT) with a collecting filter and a passive test lung. To simulate exhaled humidity, a heated humidifier was placed between the collection filter and the distal tip of the ETT, set at 36°C. Experiments without exhaled humidity were performed with no heat and humidification. For each test, 4 puffs of albuterol sulfate (432 µg) were administered. The drug was eluted from the collecting filter and analyzed via spectrophotometer (276 nm). Each experiment was run 3 times. RESULTS: Delivery efficiency of the bidirectional adapter was greatest compared to other adapters tested in this study (P = .01). Aerosol deposition obtained from the combination of the T-piece closed suction catheter and the bidirectional adapter was more than that from the multi-access port (P = .42) and double swivel closed suction catheter (P = .27). Drug delivery without exhaled humidity exceeded values with exhaled humidity by 20-90% using the small unidirectional, large unidirectional, and bidirectional adapters (P = .01, P = .02 and P = .02, respectively). CONCLUSIONS: Closed suction catheter, pMDI spacer/ adapters, and exhalation conditions have an impact on drug delivery during simulated adult mechanical ventilation. Aerosol delivery without exhaled humidity appears to report unrealistically high values in comparison to the more accurate values with simulated exhalation humidity. [ABSTRACT FROM AUTHOR]

Spinal muscular atrophy [SMA] is the most common genetic cause of childhood mortality, primarily from the most severe form SMA type 1. It is a severe, progressive motor neurone disease, affecting the lower brainstem nuclei and the spinal cord. There is a graded level of severity with SMA children from a practical viewpoint described as "Non-sitters", "Sitters" and less commonly, "Ambulant" correlating with SMA Type 0/Type 1, Type 2 and Type 3 respectively. Children with SMA Type 0 have a severe neonatal form whilst those with SMA Type 1 develop hypoventilation, pulmonary aspiration, recurrent lower respiratory tract infections, dysphagia and failure to thrive before usually succumbing to respiratory failure and death before the age of 2 years. The recent introduction of the antisense oligonucleotide nusinersen into clinical practice in certain countries, following limited trials of less than two years duration, has altered the treatment landscape and improved the outlook considerably for SMN1 related SMA. Approximately 70% of infants appear to have a clinically significant response to nusinersen with improved motor function. It appears the earlier the treatment is initiated the better the response. There are other rarer genetic forms of SMA that are not treated with nusinersen. Clinical expectations will change although it is unclear as yet what the extent of response will mean in terms of screening initiatives [e.g., newborn screening], "preventative strategies" to maintain respiratory wellbeing, timing of introduction of respiratory supports, and prolonged life expectancy for the subcategory of children with treated SMA type 1. This article provides a review of the strategies available for supporting children with respiratory complications of SMA, with a particular emphasis on SMA Type 1. [ABSTRACT FROM AUTHOR]

BACKGROUND: Airway clearance techniques are regularly proposed as a part of the treatment in chronic obstructive airway diseases. Intrapulmonary percussive ventilation (IPV) is used as an airway clearance technique in patients affected by excessive lung secretions. The aim of this systematic review is to summarize the physiological and clinical effects related to the use of IPV as an airway clearance technique in chronic obstructive airway diseases. METHODS: This systematic review followed the PRISMA guidelines. Randomized, controlled, comparative, and cohort studies investigating IPV as an airway clearance technique were identified and reviewed from 3 databases. Two reviewers independently assessed study quality and reviewed the selected studies. RESULTS: 278 subjects from 12 studies were included in the final analysis, with 3 diseases studied. Only one of the included studies had a sample size > 50 subjects. The main findings showed that IPV improves gas exchange during exacerbation and could reduce the hospital length of stay for patients with COPD. In subjects with cystic fibrosis, neither lung function nor other parameters were improved. CONCLUSIONS: The systematic use of IPV as an airway clearance technique in chronic obstructive airway diseases is not supported by sufficiently strong evidence to recommend routine use in this patient population. [ABSTRACT FROM AUTHOR]

BACKGROUND: The cough mechanism is often impaired in children with quadriplegic spastic cerebral palsy, accounting for the high prevalence of pneumonia and atelectasis requiring prolonged hospitalization. Conventional chest physiotherapy (CPT) is a current technique recommended at the onset of lower-respiratory infections in cerebral palsy. Previous studies have demonstrated the usefulness of mechanical insufflation-exsufflation (MI-E) in children with neuromuscular disease. To date, there has been no study of MI-E in children with quadriplegic spastic cerebral palsy. The objective of the study is to compare the efficacy in reducing hospital stay and improvement of atelectasis between MI-E and CPT in children with quadriplegic spastic cerebral palsy with lower-respiratory infections. METHODS: This study is a randomized controlled trial. Children with quadriplegic spastic cerebral palsy, age 6 months to 18 y, admitted for lowerrespiratory infections and/or atelectasis at King Chulalongkorn Memorial Hospital between June 1, 2014, and March 31, 2015, were recruited. Those with pneumothorax, severe pneumonia, active tuberculosis, and shock were excluded. Children were randomized into the MI-E or CPT group. The MI-E group received MI-E (3 therapies/d), and the CPT group received CPT (1 therapy/d). Vital signs per protocol and chest radiograph as needed were recorded. RESULTS: There were 22 children enrolled in the study, 11 in the MI-E and 11 in the CPT group. Demographic data were comparable in both groups. The length of hospital stay was similar in both groups (MI-E 4-24 d vs CPT 6-42 d, P = .15). There were 17 subjects with atelectasis (MI-E [n = 9] versus CPT [n = 8]). In this atelectasis subgroup, MI-E had shortened therapy time when compared with CPT (2.9 ± 0.8 d vs 3.9 ± 0.6 d, P = .01). No complications were observed. CONCLUSIONS: MI-E is proven to be beneficial in shortening the duration of airway clearance in children with quadriplegic spastic cerebral palsy presenting with lowerrespiratory infections and atelectasis. MI-E is a safe and efficient intervention for airway clearance. [ABSTRACT FROM AUTHOR]

Physiotherapy remains the cornerstone of cystic fibrosis (CF) management alongside medical treatment. Traditionally, physiotherapy intervention focussed on airway clearance during the clinically stable stage and chest infections. Research evidence consistently supports greater mucus clearance with chest physiotherapy compared to cough alone or no treatment. Various methods and techniques of airway clearance have been developed and investigated, and data suggest that most of them are of similar effectiveness. Nowadays physiotherapy management also extends to other areas, supported by studies and clinical practice. The physiotherapists plan, supervise and follow-up systematic exercise or personalised rehabilitation programs, which, similarly to airway clearance, are recommended in all patients with CF. Furthermore, based on a comprehensive assessment, physiotherapists incorporate the management of accompanying musculoskeletal problems such as back pain and postural disorders, as well as urine incontinence issues. In the era that aims to improve quality of life, it is essential that physiotherapists are aware of specific conditions that might affect the management of CF. Their role is to work alongside and within the CF multi-disciplinary team throughout patient's treatment and consistently support the patient and carers, in particular whilst on clinical pathways of the lung transplantation and palliative care. [ABSTRACT FROM AUTHOR]

The opportunistic pathogen Pseudomonas aeruginosa colonizes the lungs of susceptible individuals by deploying virulence factors targeting host defenses. The secreted factor Cif (cystic fibrosis transmembrane conductance regulator inhibitory factor) dysregulates the endocytic recycling of CFTR and thus reduces CFTR abundance in host epithelial membranes. We have postulated that the decrease in ion secretion mediated by Cif would slow mucociliary transport and decrease bacterial clearance from the lungs. To test this hypothesis, we explored the effects of Cif in cultured epithelia and in the lungs of mice. We developed a strategy to interpret the "hurricane-like" motions observed in reconstituted cultures and identified a Cif-mediated decrease in the velocity of mucus transport in vitro. Presence of Cif also increased the number of bacteria recovered at two time points in an acute mouse model of pneumonia caused by P. aeruginosa. Furthermore, recent work has demonstrated an inverse correlation between the airway concentrations of Cif and 15-epi-lipoxin A4, a proresolving lipid mediator important in host defense and the resolution of pathogen-initiated inflammation. Here, we observe elevated levels of 15-epi-lipoxin A4 in the lungs of mice infected with a strain of P. aeruginosa that expresses only an inactive form of cif compared with those mice infected with wild-type P. aeruginosa. Together these data support the inclusion of Cif on the list of virulence factors that assist P. aeruginosa in colonizing and damaging the airways of compromised patients. Furthermore, this study establishes techniques that enable our groups to explore the underlying mechanisms of Cif effects during respiratory infection. [ABSTRACT FROM AUTHOR]