Home-based pulmonary rehabilitation in people with bronchiectasis: a randomised controlled trial.

AIM: To investigate the short- and long-term effects of home-based pulmonary rehabilitation (HBPR) on functional capacity, quality of life, peripheral muscle strength, dyspnoea and daily physical activity in people with bronchiectasis. METHODS: Randomised controlled trial with 63 participants with bronchiectasis. The HBPR group performed three sessions per week for 8 weeks (aerobic exercise: step training for 20 min; resistance training: exercises for quadriceps, hamstrings, deltoids and biceps brachii using elastic bands). The control group received a recommendation to walk at moderate intensity, three times per week. A weekly phone call was conducted for all participants, and the HBPR group received a home visit every 15 days. The primary outcome was distance in the incremental shuttle walk test (ISWT). Secondary outcomes were time in the endurance shuttle walk test (ESWT), number of steps in the incremental step test, quality of life, quadriceps muscle strength and daily physical activity. Measures were taken before and after intervention and 6 months later. RESULTS: After the intervention, the HBPR group had increased ISWT distance compared with the control group with between-group difference 87.9 m (95% CI 32.4-143.5 m). In addition, between-group differences were found in the ESWT, incremental step test, quality of life and quadriceps muscle strength, favouring the HBPR group. After 6 months, no differences were observed between the groups. CONCLUSION: HBPR is an effective alternative offering of pulmonary rehabilitation for people with bronchiectasis. However, the programme was not effective in maintaining the benefits after 6 months of follow-up.

Quality of Life Questionnaire-Bronchiectasis: a study of the psychometric properties of the Brazilian Portuguese version.

OBJECTIVE: To evaluate the psychometric properties of the Brazilian Portuguese version of the Quality of Life Questionnaire-Bronchiectasis. DESIGN: Cross-sectional study. SETTING: Outpatient clinic. SUBJECTS: Clinically stable individuals with a diagnosis of bronchiectasis. MEASURES: The evaluations performed were spirometry, incremental shuttle walk test, Saint George's Respiratory Questionnaire, and the modified Medical Research Council dyspnea scale. The Quality of Life Questionnaire-Bronchiectasis was administered twice (seven to 14 days apart). Psychometric analyses were performed as follows: reliability, construct validity, criterion validity, and interpretability. RESULTS: In total, 108 individuals (48 ± 14 years, 61 women) participated in the study. Internal consistency was considered adequate (Cronbach's alpha ⩾ 0.70) for the majority of scales (from 0.58 to 0.93). Test-retest coefficients were moderate to excellent (intraclass correlation coefficients from 0.70 to 0.93). In the construct validity, 35 of 37 items correlated more strongly with their assigned scale than a competing scale. The convergent validity showed significant correlations between scales of the Quality of Life Questionnaire-Bronchiectasis with modified Medical Research Council dyspnea scale, and incremental shuttle walk test (r from 0.20 to 0.59). A low to moderate correlations was revealed between all scales of the Quality of Life Questionnaire-Bronchiectasis and the Saint George's Respiratory Questionnaire domains (r from 0.26 to 0.70). The standard error of measurement was acceptable. Ceiling effects were found for the Social Functioning and Treatment Burden scales. CONCLUSIONS: The Quality of Life Questionnaire-Bronchiectasis is a reliable, valid instrument with adequate internal consistency for the evaluation of the impact of bronchiectasis on the health-related quality of life of Brazilian adults.

'Go for it, dream big, work hard and persist': A message to the next generation of CF leaders in recognition of International Women's Day 2020.

The focus for International Women's Day 2020 is gender equity:'We can actively choose to challenge stereotypes, fight bias, broaden perceptions, improve situations and celebrate women's achievements. Collectively, each one of us can help create a gender equal world.' We have come together as an international group of women holding senior positions within CF to raise awareness. There is growing recognition of gender imbalance within our sector in senior leadership, grant and publication success. Several institutions, such as National Institutes of Health, have missions to tackle this. The issues raised by our panellists were wide-ranging: decisions around starting a family, impact on career progression; experiences of bias in appointments or promotions; selfbelief. We hope that raising these issues will encourage future leaders in CF to step up, to build teams based on fairness, equity and diversity, and to catalyse steps towards this goal in their institutions and society more widely.

Physiological Responses During Field Walking Tests in Adults with Bronchiectasis.

BACKGROUND: Field walking tests are commonly used in patients with chronic pulmonary diseases for assessment of functional capacity. However, the physiological demands and magnitude of desaturation on 6-min walk test (6MWT), incremental shuttle walk test (ISWT), and endurance shuttle walk test (ESWT) have not been investigated in patients with bronchiectasis. The objective of this study was to compare the physiological responses and the magnitude of desaturation of subjects with bronchiectasis when performing the 6MWT, ISWT, and ESWT. METHODS: Thirty-two subjects underwent the 6MWT, ISWT, and ESWT on 3 different days. Pulmonary gas exchange, heart rate, and [Formula: see text] were measured in all tests. RESULTS: There were no differences in the peak rate of oxygen uptake, ventilation, dyspnea, and leg fatigue between the tests. Equivalent cardiac demand (ie, heart rate at peak) was observed with the 6MWT (137 ± 21 beats/min) and the ESWT (142 ± 21 beats/min), but this was lower in the ISWT (135 ± 19 beats/min) compared to ESWT (P < .05). Most subjects achieved a vigorous exercise intensity (heart rate of 70-90% of predicted) in all tests. There was no difference in desaturation among the tests (6MWT: -6.8 ± 6.6%, ISWT: -6.1 ± 6.0%, and ESWT: -7.0 ± 5.4%). CONCLUSIONS: The 6MWT, ISWT, and ESWT induced similar physiological responses at the peak of exercise, eliciting a vigorous exercise intensity. The magnitude of desaturation was similar across tests. This means these tests can be used interchangeably for evaluation of exercise-induced desaturation.

Determinants of Peripheral Muscle Strength and Activity in Daily Life in People With Bronchiectasis.

Background: Bronchiectasis is characterized by a progressive structural lung damage, recurrent infections and chronic inflammation which compromise the exertion tolerance, and may have an impact on skeletal muscle function and physical function. Objective: The purpose of this study was to compare peripheral muscle strength, exercise capacity, and physical activity in daily life between participants with bronchiectasis and controls and to investigate the determinants of the peripheral muscle strength and physical activity in daily life in bronchiectasis. Design: This study used a cross-sectional design. Methods: The participants' quadriceps femoris and biceps brachii muscle strength was measured. They performed the incremental shuttle walk test (ISWT) and cardiopulmonary exercise testing, and the number of steps/day was measured by a pedometer. Results: Participants had reduced quadriceps femoris muscle strength (mean difference to control group = 7 kg, 95% CI = 3.8-10.1 kg), biceps brachii muscle strength (2.1 kg, 95% CI = 0.7-3.4 kg), ISWT (227 m, 95% CI = 174-281 m), peak VO2 (6.4 ml/Kg/min, 95% CI = 4.0-8.7 ml/Kg/min), and number of steps/day (3,332 steps/day, 95% CI = 1,758-4,890 steps/day). A lower quadriceps femoris strength is independently associated to an older age, female sex, lower body mass index (BMI), higher score on the modified Medical Research Council scale, and shorter distance on the ISWT (R2 = 0.449). Biceps brachii strength is independently associated with sex, BMI, and dyspnea (R2 = 0.447). The determinants of number of daily steps were dyspnea and distance walked in ISWT, explaining only 27.7% of its variance. Limitations: Number of steps per day was evaluated by a pedometer. Conclusions: People with bronchiectasis have reduced peripheral muscle strength, and reduced aerobic and functional capacities, and they also are less active in daily life. Modifiable variables such as BMI, dyspnea, and distance walked on the ISWT are associated with peripheral muscle strength and physical activity in daily life.

Does home-based pulmonary rehabilitation improve functional capacity, peripheral muscle strength and quality of life in patients with bronchiectasis compared to standard care?

BACKGROUND: Home-based pulmonary rehabilitation is a promising intervention that may help patients to overcome the barriers to undergoing pulmonary rehabilitation. However, home-based pulmonary rehabilitation has not yet been investigated in patients with bronchiectasis. OBJECTIVES: To investigate the effects of home-based pulmonary rehabilitation in patients with bronchiectasis. METHODS: An open-label, randomized controlled trial with 48 adult patients with bronchiectasis will be conducted. INTERVENTIONS: The program will consist of three sessions weekly over a period of 8 weeks. Aerobic exercise will consist of stepping on a platform for 20min (intensity: 60-80% of the maximum stepping rate in incremental step test). Resistance training will be carried out using an elastic band for the following muscles: quadriceps, hamstrings, deltoids, and biceps brachii (load: 70% of maximum voluntary isometric contraction). CONTROL: The patients will receive an educational manual and a recommendation to walk three times a week for 30min. All patients will receive a weekly phone call to answer questions and to guide the practice of physical activity. The home-based pulmonary rehabilitation group also will receive a home visit every 15 days. MAIN OUTCOME MEASURES: incremental shuttle walk test, quality of life, peripheral muscle strength, endurance shuttle walk test, incremental step test, dyspnea, and physical activity in daily life. The assessments will be undertaken at baseline, after the intervention, and 8 months after randomization. DISCUSSION: The findings of this study will determine the clinical benefits of home-based pulmonary rehabilitation and will contribute to future guidelines for patients with bronchiectasis. TRIAL REGISTRATION: www.ClinicalTrials.gov (NCT02731482). https://register.clinicaltrials.gov/prs/app/action/SelectProtocol?sid=S00060X6&selectaction=Edit&uid=U00028HR&ts=2&cx=1jbszg.

Incremental shuttle walking test: a reproducible and valid test to evaluate exercise tolerance in adults with noncystic fibrosis bronchiectasis.

OBJECTIVE: To analyze the reliability, validity, and determinants of the incremental shuttle walk test (ISWT) in adults with noncystic fibrosis bronchiectasis. DESIGN: Cross-sectional study. SETTING: Outpatient clinic. PARTICIPANTS: Subjects (N=75; 26 men) underwent, on different days, cardiopulmonary exercise testing (CPET) and 2 ISWTs, 30 minutes apart. The number of steps in daily life was recorded. Concurrent validity was tested by the relation between distance walked with peak load and oxygen consumption (V˙o2). INTERVENTIONS: None. MAIN OUTCOME MEASURES: Distance walked (m) was compared between the first and second ISWTs; greatest distance walked was correlated with peak load and Vo2peak obtained from CPET, steps per day, and dyspnea evaluated by the Medical Research Council (MRC) scale; and desaturation was compared between CPET and the ISWT. RESULTS: Distance walked was equivalent between the first ISWT (441±152m) and the second ISWT (445±153m) with an excellent intraclass correlation coefficient (.995; 95% confidence interval, .99-.997). There were significant correlations between distance walked and peak load (r=.82), V˙o2 (r=.72), steps per day (r=.61), and the MRC scale (r=-.69). Age, body mass index, sex, forced vital capacity (% predicted), dyspnea, and steps per day explained 70% of the variation in distance walked (m) and 60% of the variance when expressed as percent predicted. Higher desaturation was observed during the ISWT (-4%±4%) than cycling (-2±3%) (P<.001). CONCLUSIONS: The ISWT is reliable, represents functional capacity, and induces greater desaturation than cycling. Age, body composition, pulmonary function, dyspnea, and physical activity in daily life are determinants of the distance walked on the ISWT.

[Massive rhabdomyolysis: an uncommon complication of cholesterol embolization syndrome]. / Rhabdomyolyse massive: une complication rare de la maladie des emboles de cholestérol.

A 79-year-old man with atherosclerosis presented blue toes and livedo reticularis. The patient had eroded aortic atheromatous plaques, and cholesterol embolization syndrome was suspected. An endovascular technique to exclude sources of cholesterol emboli was however performed. The patient immediately presented with severe muscle pain and total functional disability of lower limbs, new ischemic lesions of toes, anal and genital necrosis, and a livedo extended up to the abdomen. A massive rhabdomyolysis occurred associated with acute kidney injury and hyperkaliemia treated by continuous renal replacement therapy with regional citrate anticoagulation. Steroids have been introduced and renal function improved. Cholesterol crystals were also found on a skin biopsy, performed before endovascular procedure.

Oxidative stress in systemic lupus erythematosus and rheumatoid arthritis patients: relationship to disease manifestations and activity.

AIM: The present work was undertaken to study the status and contribution of oxidative stress in systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) patients. Relationship of the markers of oxidative stress to clinical manifestations, disease activity, damage and medications used were well considered. METHODS: Thirty SLE and 30 RA female patients were included in the study and clinical examination and investigations were performed and disease activity was assessed. Markers of oxidative stress, including malondialdehyde (MDA) and antioxidant scavengers with glutathione (GSH) and glutathione peroxidase (GSH Px) were assessed. RESULTS: Level of MDA, GSH and GSH Px were remarkably altered in RA and SLE patients compared to controls. Markers of increased oxidative stress and impaired antioxidant capacity were profound in RA and significantly reflected disease activity in RA and SLE, with special attention to alopecia and lupus nephritis. RA patients receiving methotrexate had significantly altered parameters and the steroid dose in SLE patients correlated with these markers. CONCLUSION: Oxidative stress was increased and more profound in RA than SLE and could well reflect disease activity, with special attention to SLE patients with alopecia and nephritis. Medications used are closely related to the oxidant/antioxidant imbalance. Considering antioxidants in novel therapeutic strategies is important in SLE and RA patients.