Six-minute walking distance and desaturation-distance ratio in allogeneic stem cell transplantation.

BACKGROUND: Most patients with haematological malignancies who undergo allogeneic haematopoietic stem cell transplant (HSCT) receive chemotherapy before the transplant to control the disease. Certain chemotherapy drugs can cause lung toxicity. Conversely, in patients with chronic respiratory conditions, the 6-min walking test (6MWT) and the desaturation-distance ratio (DDR) have demonstrated prognostic significance. Our objective was to determine whether the 6MWD and DDR, assessed prior to HSCT, have a prognostic impact on survival at 24 months post-HSCT. METHODS: A prospective experimental study was conducted in consecutive patients referred for allogeneic HSCT at Hospital Clinic, Barcelona, Spain. A complete functional respiratory study, including the 6MWT and DDR, was conducted prior to admission. The area under the curve (AUC) and cut-off points were calculated. Data on patients' characteristics, HSCT details, main events, with a focus on lung complications, and survival at 24 months were analysed. RESULTS: One hundred and seventy-five patients (39% women) with mean age of 48 ± 13 years old were included. Before HSCT, forced vital capacity and forced expiratory volume in the first second were 96% ± 13% predicted and 92% ± 14% predicted, respectively; corrected diffusing capacity for carbon monoxide 79% ± 15% predicted; 6MWD was 568 ± 83 m and DDR of .27 (.20-.41). The cut-off points for 6MWD and DDR were 566 m, [.58 95% CI (.51-.64)], p = .024 and .306, [.63 95% CI (.55-.70)], p = .0005, respectively. The survival rate at 24 months was 55%. CONCLUSION: Our results showed that individuals who exhibit a 6MWD shorter than 566 ms or a decline in DDR beyond .306 experienced reduced survival rates at 24 months after HSCT.

Respiratory Muscle Training in Patients with Obstructive Sleep Apnoea: A Systematic Review and Meta-Analysis.

Background: Effective treatments for obstructive sleep apnoea (OSA) include positive pressure, weight loss, oral appliances, surgery, and exercise. Although the involvement of the respiratory muscles in OSA is evident, the effect of training them to improve clinical outcomes is not clear. We aimed to determine the effects of respiratory muscle training in patients with OSA. Methods: A systematic review was conducted in seven databases. Studies that applied respiratory muscle training in OSA patients were reviewed. Two independent reviewers analysed the studies, extracted the data and assessed the quality of evidence. Results: Of the 405 reports returned by the initial search, eight articles reporting on 210 patients were included in the data synthesis. Seven included inspiratory muscle training (IMT), and one included expiratory muscle training (EMT). Regarding IMT, we found significant improvement in Epworth sleepiness scale in −4.45 points (95%CI −7.64 to −1.27 points, p = 0.006), in Pittsburgh sleep quality index of −2.79 points (95%CI −4.19 to −1.39 points, p < 0.0001), and maximum inspiratory pressure of −29.56 cmH2O (95%CI −53.14 to −5.98 cmH2O, p = 0.01). However, the apnoea/hypopnea index and physical capacity did not show changes. We did not perform a meta-analysis of EMT due to insufficient studies. Conclusion: IMT improves sleepiness, sleep quality and inspiratory strength in patients with OSA.

Physical activity in children and adolescents with cystic fibrosis: A systematic review and meta-analysis.

BACKGROUND: Exercise and physical activity (PA) are essential components of the care of cystic fibrosis (CF) patients. Lower PA levels have been associated with worse pulmonary function, aerobic fitness, glycemic control, and bone mineral density. Most people with CF do not engage in the recommended amounts of PA. OBJECTIVE: To determine the level of PA in children and adolescents with CF. METHODS: A systematic review with meta-analysis was conducted without language restrictions in five databases. Were included studies that analyzed PA measured by objective and subjective instruments in children and adolescents with CF. Two independent reviewers analyzed the studies, extracted the data, and assessed the quality of evidence. The risk of bias of the included studies was assessed with the National Heart, Lung, and Blood Institute's risk-of-bias tool. RESULTS: Of the 1535 reports returned by the initial search, 20 articles reporting on 785 patients were included in the data synthesis. The forest plot showed that the CF group had a similar moderate-to-vigorous PA (MVPA) (mean difference, -7.79; 95% CI -15.65 to 0.08 min/d; P = .05) and sedentary time (mean difference, -50.81; 95%CI, -109.96 to 8.35 min/d; P = .09) to the control group. CONCLUSION: Children and adolescents with CF have a similar MVPA and sedentary time compared to controls. There are many options, subjective and objective, for assessing PA in this population. Optimal tool selection should guarantee more valid results.

Kinesiología respiratoria en niños con disquinesia ciliar primaria / Respiratory phisioterapy in children with primary ciliary dyskinesia

Primary ciliary dyskinesia is a rare autosomal recessive disease with compromised mucociliary drainage. Among the most commonly recommended non-pharmacological therapeutic strategies are secretion drainage techniques. However, the evidence for the use and effectiveness of these techniques is low, and they are generally based on extrapolated evidence of cystic fibrosis. This article reviews the recommendations and available evidence of chest physiotherapy, mainly manual and instrumental techniques of bronchial drainage and physical exercise in children with primary ciliary dyskinesia.

La disquinesia ciliar primaria es una enfermedad autosómica recesiva rara con compromiso del drenaje mucociliar. Entre las estrategias terapéuticas no farmacológicas más comúnmente recomendadas se encuentra las técnicas de drenaje de secreciones. Sin embargo, la evidencia del uso y efectividad de estas técnicas es reducida y generalmente se basan en evidencia extrapolada de la fibrosis quística. Este artículo revisa las recomendaciones y la evidencia disponible de la kinesiología respiratoria, principalmente las técnicas manuales e instrumentales de drenaje bronquial y el ejercicio físico en niños con disquinesia ciliar primaria.

Impact of Hypertonic Saline Solutions on Sputum Expectoration and Their Safety Profile in Patients with Bronchiectasis: A Randomized Crossover Trial.

BACKGROUND: The role of hyaluronic acid plus hypertonic saline (HA+HS) as a mucoactive treatment in patients with bronchiectasis is still unknown. This study evaluated whether HA+HS solution enhances similar sputum quantity with better safety profile than HS alone in patients with bronchiectasis. METHODS: In this double-blind randomized crossover trial, three solutions (7% HS; 0.1% HA +7%HS; and 0.9% isotonic saline, IS) were compared in outpatients with bronchiectasis and chronic sputum expectoration. Participants inhaled each solution across four consecutive sessions. All sessions, except on session 3, also included 30 minutes of airway clearance technique. A 7-day washout period was applied. Sputum weight was collected during the sessions (primary outcome) as well as during a 24-hour follow-up. The Leicester Cough Questionnaire (LCQ) and lung function were measured before/after each treatment arm. Safety was assessed by the monitoring of adverse events (AEs). RESULTS: Twenty-eight patients with bronchiectasis (mean age of 64.0 (17.9) and FEV1% 60.9 (24.6) of predicted) were recruited. HS and HA+HS promoted similar expectoration during sessions, both being greater than IS [median difference HS vs. IS 3.7 g (95% CI 0.5-6.9); HA+HS vs. IS 3.2 g (95%CI 0.5-5.9)]. Sputum expectorated exclusively during the ACT period was similar across all treatment arms [HS vs. IS -0.3 g (95% CI -1.7 to 0.9); HA+HS vs. IS 0.0 g (95% CI -1.3 to 1.4); HS vs. HA+HS 0.0 g (95% CI -1.2 to 0.4)]. Sputum collected over the 24-hour follow-up tended to be lower for HS and HA+HS compared with IS [HS vs. IS -1.7 g (95% CI -4.2 to 0.0); HA+HS vs. IS -1.1 g (95%CI -3.6 to 0.7)]. No differences in LCQ or lung function were observed. Most severe AEs were reported using HS. CONCLUSION: HS and HA+HS were more effective on sputum expectoration than IS in patients with bronchiectasis, reporting HA+HS better safety profile than HS.

Effectiveness of a Home-Based Active Video Game Programme in Young Cystic Fibrosis Patients.

BACKGROUND: Exercise-based rehabilitation is already a part of cystic fibrosis (CF) treatment; however, patient adherence is low. OBJECTIVES: To assess the effectiveness of a home exercise programme using active video games (AVGs) as a training modality for children and adolescents with CF. METHODS: Thirty-nine children with CF were randomised to a control group (CG, n = 20, age 11 ± 6 years; FEV1 86.2 ± 20.5% of predicted) or a training group (AVGG, n = 19, age 13 ± 3 years; FEV1 82.7 ± 21.7% of predicted). The home training protocol consisted of 30- to 60-min sessions, 5 days/week, for 6 weeks using a Nintendo Wii™ platform. Exercise capacity was measured by the 6-min walk test (6MWT) and modified shuttle walk test (MSWT); muscular strength was estimated using the horizontal jump test (HJT), medicine ball throw (MBT), and hand grip strength (right [RHG]; left [LHG]); and quality of life was rated using the Cystic Fibrosis Questionnaire-Revised (CFQ-R). All the children were measured at baseline, after rehabilitation, and at 12 months. RESULTS: For the group × time interaction ANOVAs, the AVGG showed significant between-group differences in exercise capacity: 6MWT farthest walking distance, 38.4 m (p < 0.01); MSWT farthest walking distance, 78.4 m (p < 0.05); and muscular strength: HJT 9.8 cm, MBT 30.8 cm, RHG 7 kg, and LHG 6.5 kg (p < 0.01), before versus after intervention. The CFQ-R reported significantly higher scores on respiratory symptoms after the intervention and favoured the AVGG, and there was an improvement in other domains after 12 months. Adherence to the home exercise programme was 95% during the 6- week intervention period. CONCLUSION: A home-based programme using AVGs can effectively improve exercise capacity, muscular strength and quality of life in the short-term in children and adolescents with CF. The effects of training on muscle performance and quality of life were sustained over 12 months.

Evaluación de la actividad física en pacientes con enfermedad pulmonar obstructiva crónica / Tools for physical activity assessment in chronic obstructive pulmonary disease

The prevalence and mortality of chronic obstructive pulmonary disease (COPD) is increasing in Chile, constituting a public health problem. Pulmonary and systemic consequences of COPD affect physical activity, as the disease progresses. There are multiple means for physical activity assessment, from low cost and easily applicable questionnaires to sophisticated laboratory tests. Physical inactivity is a modifiable risk factor for morbidity and mortality in patients with COPD. Physical activity interventions not only contribute to decrease the likelihood of mortality, but also protect from comorbidities, especially cardiovascular ones. It also plays a major role avoiding functional limitations of these subjects. Dyspnea and fatigue render exercise as an unpleasant activity for most patients with COPD. If psychological alterations such as anxiety and depression are summed, these patients drift towards an inactive lifestyle. This article analyzes several tools available to assess physical activity is patients with COPD, useful in clinical practice.

European Respiratory Society guidelines for the management of adult bronchiectasis.

Bronchiectasis in adults is a chronic disorder associated with poor quality of life and frequent exacerbations in many patients. There have been no previous international guidelines.The European Respiratory Society guidelines for the management of adult bronchiectasis describe the appropriate investigation and treatment strategies determined by a systematic review of the literature.A multidisciplinary group representing respiratory medicine, microbiology, physiotherapy, thoracic surgery, primary care, methodology and patients considered the most relevant clinical questions (for both clinicians and patients) related to management of bronchiectasis. Nine key clinical questions were generated and a systematic review was conducted to identify published systematic reviews, randomised clinical trials and observational studies that answered these questions. We used the GRADE approach to define the quality of the evidence and the level of recommendations. The resulting guideline addresses the investigation of underlying causes of bronchiectasis, treatment of exacerbations, pathogen eradication, long term antibiotic treatment, anti-inflammatories, mucoactive drugs, bronchodilators, surgical treatment and respiratory physiotherapy.These recommendations can be used to benchmark quality of care for people with bronchiectasis across Europe and to improve outcomes.

Validity of a Spanish Version of the Leicester Cough Questionnaire in Children With Cystic Fibrosis.

BACKGROUND: Cystic fibrosis (CF) patients present chronic cough as one of the main symptoms, which has an important effect on quality of life and social relations. Our goal was to validate the Spanish version of the Leicester Cough Questionnaire (LCQ) in a group of children and teenagers with CF. METHODS: After adapting to Spanish by standardized translation and retro-translation methodology, a sample of 58 stable CF patients from 7 to 18 years were recruited from three CF specialized centers in Spain. The questionnaire was administered twice; the second administration (LCQ2) was performed between 2-4 weeks later than the first one (LCQ1), in order to analyse the reliability and validity of the Spanish version. To correlate results with health related quality of life (HRQoL) we used the Cystic Fibrosis Questionnaire-Revised (CFQ-R). RESULTS: Population was composed by 62% male, age 11.7 ± 3.1 years and body mass index (BMI) 19 ± 3 kg/m(2). Total scores from LCQ were: LCQ1 19 (17.75-21) vs LCQ2 19 (16-21) (P=.199). Cronbach's Alpha coefficient was 0.83 for the LCQtotal and for each specific domain was: 0.82 LCQphysical; 0.74 LCQpsychological and 0.62 LCQsocial. Intraclass correlation coefficient was: 0.69 LCQphysical; 0.59 LCQpsychological; 0.45 LCQsocial and 0.71 LCQtotal (good reliability). Relations with CFQ-R showed moderated and significant results: for the LCQtotal: respiratory symptom domain r = 0,51 (P<.001) and physical capacity domain r = 0,62 (P<.05). CONCLUSION: The Spanish version of the Leicester Cough Questionnaire is reliable and valid for children and adolescents with CF and it has good relations with health related quality of life in this population.

Non-anaemic iron deficiency impairs response to pulmonary rehabilitation in COPD.

BACKGROUND AND OBJECTIVE: Non-anaemic iron deficiency (NAID) might alter the oxygen pathway in health and disease. The current study aims at assessing the impact of NAID on aerobic capacity in patients with chronic obstructive pulmonary disease (COPD). METHODS: A prospective sample of 70 non-anaemic COPD patients candidate to participate in an 8-week pulmonary rehabilitation (PR) programme was studied. Incremental cycling exercise to peak oxygen uptake (V'O2peak ) and constant work-rate exercise at 80% V'O2peak to exhaustion were assessed pre- and post-PR. Training-induced increase of endurance time (ET) ≥33%, which represented the minimal clinically important difference, classified patients as responders to exercise training. RESULTS: The prevalence of NAID was 48% (n = 34) showing no relationship with the Global Initiative for Chronic Obstructive Lung Disease stages (P = 0.209). Patients with NAID showed lower pre-training ET (P = 0.033) and V'O2peak (P = 0.007) than normal iron status (NIS) patients after adjustment for potential covariates. Significant training-induced physiological changes were seen in the NIS group (ΔV'O2peak 68(132) mL/min; P = 0.009), but not in the NAID group (ΔV'O2peak 26 (126) mL/min; P = 0.269). The NAID group showed lower percentage of responders to training (56%) than the NIS group (78%) (P = 0.041). CONCLUSIONS: COPD patients with NAID showed lower pre-training aerobic capacity and reduced training-induced response than NIS patients after adjusting for potential confounding variables.

La rehabilitación respiratoria no hospitalaria: un futuro prometedor / Respiratory rehabilitation does not hospital: a promising future

Recientemente, en el número 4, 2014 de la RAMR, Martín Sivori ha publicado una interesante revisión sobre la rehabilitación respiratoria en el domicilio de pacientes con enfermedad pulmonar obstructiva crónica (EPOC). En primer lugar, quiero destacar la importancia de este tipo de revisiones que, a partir de un análisis sistemático de la literatura, permiten tener una visión global de un tema aportando conocimientos prácticos fácilmente implementables en la clínica. Sivori, además, va un paso más allá y hace una inestimable aportación mediante las tablas 2 a 5, donde sintetiza de forma clara y acertada los modelos de entrenamiento utilizados, las pautas de ejercicio, los equipos y pacientes candidatos a seguir programas de entrenamiento en el domicilio

Physiological response during activity programs using Wii-based video games in patients with cystic fibrosis (CF).

UNLABELLED: Patients with cystic fibrosis (CF) are characterized by an abnormal ventilation response that limits the exercise capacity. Exercise training increases exercise capacity, decreases dyspnea and improves health-related quality of life in CF. Adherence to pulmonary rehabilitation programs is a key factor to guarantee optimal benefits and a difficult goal in this population. The aim of this study was to determine the physiological response during three Nintendo Wii™ video game activities (VGA) candidates to be used as training modalities in patients with CF. METHOD: 24 CF patients (age 12.6±3.7 years; BMI 18.8±2.9kgm(-2); FEV1 93.8±18.8%pred) were included. All participants performed, on two separate days, 3 different VGA: 1) Wii Fit Plus (Wii-Fit); 2) Wii Active (Wii-Acti), and 3) Wii Family Trainer (Wii-Train), in random order during 5min. The obtained results were compared with the 6-min walk test (6MWT). The physiological variables [oxygen uptake (VO2), minute ventilation (VE), and heart rate (HR)] were recorded using a portable metabolic analyzer. RESULTS: During all VGA and 6MWT, VO2 reached a plateau from the 3rd min. Compared with the 6MWT (1024.2±282.2mLm(-1)), Wii-Acti (1232.2±427.2mLm(-1)) and Wii-Train (1252.6±360.2mLm(-1)) reached higher VO2 levels during the last 3min (p<0.0001 in both cases), while Wii-Fit (553.8±113.2mLm(-1)) reached significantly lower levels of VO2 (p<0.001). Similar effects were seen for the ventilatory volume (VE). No differences in dyspnea and oxygen saturation were seen between the different modalities. All patients were compliant with all three Wii™ modalities. CONCLUSION: Active video game are well tolerated by patients with CF. All the modalities evaluated imposed a constant load but were associated with different physiological responses reflecting the different intensities imposed. Wii-Acti and Wii-Train impose a significantly high metabolic demand comparable to the 6MWT. Further research is needed to evaluate the effects of VGA as a training program to increase exercise capacity for CF patients.

Estrategias terapéuticas para aumentar la eficacia de la tos en pacientes con enfermedades neuromusculares / Therapeutic strategies to increase the effectiveness of cough

Cough is a natural reflex that protects respiratory airways against infections or mucus retention. Cough maintains an adequate cleaning of the airways and is a mainstay of respiratory therapy. It can be triggered voluntarily by the patient or by a specific cough device. Peak cough flow (PCF) is used to assess the effectiveness of the cough. When this value is below 160 L/min, cough is considered inefficient and becomes a risk factor for respiratory problems. Patients with weak cough, especially those with neuromuscular disease, have in common a low tidal volume and a decreased maximum insufflation capacity. Both factors directly affect the inspiratory phase previous to cough, which is considered vital to obtain the optimum flow for a productive cough. Different therapeutic measures may help to increase cough efficiency among patients with cough weakness. These interventions may be performed using manual techniques or by mechanical devices. The aim of this review is to analyze the different techniques available for cough assistance, set a hierarchy of use and establish a scientific basis for their application in clinical practice.

Sildenafil to improve respiratory rehabilitation outcomes in COPD: a controlled trial.

Pulmonary hypertension is a serious complication of chronic obstructive pulmonary disease (COPD) that currently has no established pharmacological treatment. This study aimed to assess whether concomitant treatment with sildenafil would enhance the results of pulmonary rehabilitation in patients with COPD and increased pulmonary arterial pressure (PAP). In this double-blind, randomised controlled trial patients received 20 mg sildenafil or placebo three times daily and underwent pulmonary rehabilitation for 3 months. The primary end-point was the gain in the cycle endurance time at a constant work-rate. Secondary end-points included performance in the incremental exercise test, 6-min walk distance and quality of life. 63 patients with severe COPD and moderately increased PAP were randomised. Cycle endurance time increased by 149 s (95% CI 26-518 s) in the sildenafil group and by 169 s (95% CI 0-768 s) in the placebo group (median change difference -7 s, 95% CI -540-244 s; p=0.77). Gains in the incremental exercise test, 6-min walk distance and quality of life at the end of the study did not differ between groups. Measurements of arterial oxygenation and adverse events were similar in both groups. In patients with severe COPD and moderately increased PAP, concomitant treatment with sildenafil does not improve the results of pulmonary rehabilitation in exercise tolerance.

Global muscle dysfunction as a risk factor of readmission to hospital due to COPD exacerbations.

UNLABELLED: Exacerbations of chronic obstructive pulmonary disease (COPD) are associated with several modifiable (sedentary life-style, smoking, malnutrition, hypoxemia) and non-modifiable (age, co-morbidities, severity of pulmonary function, respiratory infections) risk factors. We hypothesise that most of these risk factors may have a converging and deleterious effects on both respiratory and peripheral muscle function in COPD patients. METHODS: A multicentre study was carried out in 121 COPD patients (92% males, 63 ± 11 yr, FEV(1), 49 ± 17%pred). Assessments included anthropometrics, lung function, body composition using bioelectrical impedance analysis (BIA), and global muscle function (peripheral muscle (dominant and non-dominant hand grip strength, HGS), inspiratory (PI(max)), and expiratory (PE(max)) muscle strength). GOLD stage, clinical status (stable vs. non-stable) and both current and past hospital admissions due to COPD exacerbations were included as covariates in the analyses. RESULTS: Respiratory and peripheral muscle weakness were observed in all subsets of patients. Muscle weakness, was significantly associated with both current and past hospitalisations. Patients with history of multiple admissions showed increased global muscle weakness after adjusting by FEV(1) (PE(max), OR = 6.8, p < 0.01; PI(max), OR = 2.9, p < 0.05; HGSd, OR = 2.4, and HGSnd, OR = 2.6, p = 0.05). Moreover, a significant increase in both respiratory and peripheral muscle weakness, after adjusting by FEV(1), was associated with current acute exacerbations. CONCLUSIONS: Muscle dysfunction, adjusted by GOLD stage, is associated with an increased risk of hospital admissions due to acute episodes of exacerbation of the disease. Current exacerbations further deteriorate muscle dysfunction.

Training depletes muscle glutathione in patients with chronic obstructive pulmonary disease and low body mass index.

BACKGROUND: A physiological increase in muscle glutathione after training is not seen in patients with chronic obstructive pulmonary disease (COPD), indicating abnormal peripheral muscle adaptations to exercise. OBJECTIVE: We hypothesized that oxidative stress is primarily associated with low body mass index (BMI). METHODS: Eleven patients with preserved BMI (BMI(N): 28.2 +/- 1.2 kg.m(-2)), 9 patients with low BMI (BMI(L): 19.7 +/- 0.60 kg.m(-2)) and 5 age-matched controls (26.5 +/- 0.9 kg.m(-2)) were studied before and after 8 weeks of high-intensity endurance training. Reduced glutathione (GSH) and gamma-glutamyl cysteine synthase heavy-subunit chain mRNA expression (gammaGCS-HS mRNA) were measured in the vastus lateralis. RESULTS: After training, exercise capacity increased (DeltaVO(2)PEAK, 13 +/- 5.2%; 10 +/- 5.6% and 15 +/- 4.3% in BMI(L), BMI(N) and controls, respectively; p < 0.05 each). GSH levels decreased in BMI(L) (from 5.2 +/- 0.7 to 3.7 +/- 0.8 nmol/mg protein, DeltaGSH -1.5 +/- 0.7 nmol/mg protein, p < 0.05); no changes were seen in BMI(N) (from 5.4 +/- 0.7 to 6.7 +/- 0.9 nmol/mg protein, DeltaGSH 1.3 +/- 0.9 nmol/mg protein), whereas GSH markedly increased in controls (from 4.6 +/- 1 to 8.7 +/- 0.4 nmol/mg protein, DeltaGSH 4.1 +/- 1 nmol/mg protein, p < 0.01). DeltaGSH in BMI(L) was different from DeltaGSH in BMI(N) and controls (p < 0.05, each). Consistent changes were observed in gammaGCS-HS mRNA expression. CONCLUSIONS: GSH depletion after training in BMI(L) may suggest that oxidative stress plays a key role in muscle wasting in COPD patients.