Delayed diagnosis of Swyer-James-MacLeod syndrome.

Swyer-James-MacLeod Syndrome is a rare obliterative lung disease typically caused by childhood infection resulting in arrested pulmonary development. Imaging findings include unilateral hyperlucency on chest x-ray, and hyperlucency, hypovascularity and expiratory gas trapping on computed tomography. Recognition of abnormal imaging can lead to earlier diagnosis and institution of appropriate management.

Long-term Telerehabilitation or Unsupervised Training at Home for Patients with Chronic Obstructive Pulmonary Disease: A Randomized Controlled Trial.

Rationale: Despite the benefits of pulmonary rehabilitation in chronic obstructive pulmonary disease (COPD), many patients do not access or complete pulmonary rehabilitation, and long-term maintenance of exercise is difficult. Objectives: To compare long-term telerehabilitation or unsupervised treadmill training at home with standard care. Methods: In an international randomized controlled trial, patients with COPD were assigned to three groups (telerehabilitation, unsupervised training, or control) and followed up for 2 years. Telerehabilitation consisted of individualized treadmill training at home supervised by a physiotherapist and self-management. The unsupervised training group performed unsupervised treadmill exercise at home. The control group received standard care. The primary outcome was the combined number of hospitalizations and emergency department presentations. Secondary outcomes included time free from the first event; exercise capacity; dyspnea; health status; quality of life; anxiety; depression; self-efficacy; and subjective impression of change. Measurements and Main Results: A total of 120 participants were randomized. The incidence rate of hospitalizations and emergency department presentations was lower in telerehabilitation (1.18 events per person-year; 95% confidence interval [CI], 0.94-1.46) and unsupervised training group (1.14; 95% CI, 0.92-1.41) than in the control group (1.88; 95% CI, 1.58-2.21; P < 0.001 compared with intervention groups). Telerehabilitation and unsupervised training groups experienced better health status for 1 year. Intervention participants reached and maintained clinically significant improvements in exercise capacity. Conclusions: Long-term telerehabilitation and unsupervised training at home in COPD are both successful in reducing hospital readmissions and can broaden the availability of pulmonary rehabilitation and maintenance strategies.

Ambulatory Oxygen in Fibrotic Interstitial Lung Disease: A Pilot, Randomized, Triple-Blinded, Sham-Controlled Trial.

BACKGROUND: Despite a lack of evidence, ambulatory oxygen therapy is frequently prescribed for patients with interstitial lung disease (ILD) and exertional desaturation. Patients often prefer portable oxygen concentrators to oxygen cylinders. This study aimed to examine the feasibility of conducting a clinical trial of ambulatory oxygen delivered via portable concentrators in patients with ILD. RESEARCH QUESTION: Is it feasible to conduct a clinical trial of ambulatory oxygen delivered via portable concentrators in patients with ILD? STUDY DESIGN AND METHODS: In this randomized, triple-blinded, sham-controlled trial, 30 participants with ILD and isolated exertional desaturation to < 90% on 6-minute walk tests were randomized to 12-week ambulatory oxygen or air delivered via portable concentrators, with assessments performed at baseline and weeks 4, 12, and 18. Primary outcomes were trial feasibility and the change in 6-minute walk distance (6MWD) on room air at week 12. RESULTS: Study recruitment was completed within 18 months, with six withdrawals. Participant blinding was successful, with the Bang's Blinding Index being 0 (95% CI, -0.40 to 0.40) for the oxygen group and 0 (95% CI, -0.42 to 0.42) for the sham group. No significant difference in 6MWD was seen between groups at week 12 (mean difference of -34 m [95% CI, -105 to 36], P = .34). For secondary outcomes, compared with the sham group, the oxygen group had a significantly higher Leicester Cough Questionnaire psychological domain score, indicating better cough-related quality of life (mean difference of 0.9 [95% CI, 0.2 to 1.6], P = .01), but a shorter duration of moderate-to-vigorous activities (mean difference of -135 [95% CI, -267 to -3], P = .04) at week 12. INTERPRETATION: Based on the results of this pilot study, a definitive randomized controlled trial with a longer intervention duration is warranted to clarify therapeutic impacts of ambulatory oxygen in patients with ILD. TRIAL REGISTRY: Australian New Zealand Clinical Trials Registry; No.: ACTRN12617000054314; URL: www.anzctr.org.au/.

The effect of positive expiratory pressure (PEP) therapy on symptoms, quality of life and incidence of re-exacerbation in patients with acute exacerbations of chronic obstructive pulmonary disease: a multicentre, randomised controlled trial.

BACKGROUND: Positive expiratory pressure (PEP) is a technique used to enhance sputum clearance during acute exacerbations of chronic obstructive pulmonary disease (AECOPD). The impact of PEP therapy during acute exacerbations on clinically important outcomes is not clear. This study sought to determine the effect of PEP therapy on symptoms, quality of life and future exacerbations in patients with AECOPD. METHODS: 90 inpatients (58 men; mean age 68.6 years, FEV(1) 40.8% predicted) with AECOPD and sputum expectoration were randomised to receive usual care (including physical exercise)±PEP therapy. The Breathlessness, Cough and Sputum Scale (BCSS), St George's Respiratory Questionnaire (SGRQ) and BODE index (Body mass index, airflow Obstruction, Dyspnoea, Exercise tolerance) were measured at discharge, 8 weeks and 6 months following discharge, and analysed via linear mixed models. Exacerbations and hospitalisations were recorded using home diaries. RESULTS: There were no significant between-group differences over time for BCSS score [mean (SE) at discharge 5.2 (0.4) vs 5.0 (0.4) for PEP and control group, respectively; p=0.978] or SGRQ total score [41.6 (2.6) vs 40.8 (2.8) at 8 weeks, p=0.872]. Dyspnoea improved more rapidly in the PEP group over the first 8 weeks (p=0.006), however these benefits were not observed at 6 months. Exacerbations (p=0.986) and hospitalisations (p=0.359) did not differ between groups. CONCLUSIONS: We found no evidence that PEP therapy during AECOPD improves important short-term or long-term outcomes. There does not appear to be a routine role for PEP therapy in the management of such individuals.