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.

PIVET-ED: A Prospective, Randomised, Single-Blinded, Sham Controlled Study of Pelvic Vein Embolisation for Treatment of Erectile Dysfunction.

PURPOSE: Erectile Dysfunction (ED) is defined as the inability to obtain or maintain an erection firm enough for satisfactory sexual performance and affects the quality of life of over 50% of men aged over 40 years. Venogenic ED is elucidated as a cause in a subgroup of patients. The study aims to investigate the clinical success, technical success, safety and durability of venous embolisation for management of venogenic ED. METHODS: After providing informed consent, and subsequent to confirmation of venogenic ED by Doppler ultrasound (dUS) and cavernosography, 80 men referred for cavernosography and pelvic vein embolisation, will undergo randomisation by a computer system either to treatment or sham groups. Efficacy will be assessed using dUS and a validated questionnaire, the International Index of Erectile Function (IIEF). Pharmacologic agents used during the trial will be recorded. The primary outcome of PiVET-ED is to establish clinical success at 3 and 6 months post venous embolisation, as defined by end diastolic velocity in the cavernosal artery < 5 cm/s with dUS and by a > 4-point improvement in IIEF. Durability of the embolisation procedure will be assessed annually to 5 years. Quality of life will be assessed at all study time points using the 36-Item Short Form Survey (SF-36). DISCUSSION: The PiVET-ED trial is a prospective, randomised, single-blinded, single centre, sham controlled study, which aims to establish the safety, efficacy and durability of pelvic vein embolisation for the treatment of venogenic erectile dysfunction. CLINICAL REGISTRATION: Australian New Zealand Clinical Trials Registry ACTRN12620001023943, 08/10/2020.

Diagnosis and management of idiopathic pulmonary fibrosis: Thoracic Society of Australia and New Zealand and Lung Foundation Australia position statements summary.

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with debilitating symptoms of dyspnoea and cough, resulting in respiratory failure, impaired quality of life and ultimately death. Diagnosing IPF can be challenging, as it often shares many features with other interstitial lung diseases. In this article, we summarise recent joint position statements on the diagnosis and management of IPF from the Thoracic Society of Australia and New Zealand and Lung Foundation Australia, specifically tailored for physicians across Australia and New Zealand. Main suggestions: A comprehensive multidisciplinary team meeting is suggested to establish a prompt and precise IPF diagnosis. Antifibrotic therapies should be considered to slow disease progression. However, enthusiasm should be tempered by the lack of evidence in many IPF subgroups, particularly the broader disease severity spectrum. Non-pharmacological interventions including pulmonary rehabilitation, supplemental oxygen, appropriate treatment of comorbidities and disease-related symptoms remain crucial to optimal management. Despite recent advances, IPF remains a fatal disease and suitable patients should be referred for lung transplantation assessment.

The interstitial lung disease multidisciplinary meeting: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia.

Interstitial lung diseases (ILD) are a diverse group of pulmonary diseases for which accurate diagnosis is critical for optimal treatment outcomes. Diagnosis of ILD can be challenging and a multidisciplinary approach is recommended in international guidelines. The purpose of this position paper is to review the evidence for the use of the multidisciplinary meeting (MDM) in ILD and suggest an approach to its governance and constitution, in an attempt to provide a standard methodology that could be applied across Australia and New Zealand. This position paper is endorsed by the Thoracic Society of Australia and New Zealand (TSANZ) and the Lung Foundation Australia (LFA).

Health-related quality of life in idiopathic pulmonary fibrosis: Data from the Australian IPF Registry.

BACKGROUND AND OBJECTIVE: Studies analysing the effect of worsening pulmonary physiological impairment in idiopathic pulmonary fibrosis (IPF) with respect to quality of life have been limited to single centres or highly selected trial populations. The aim of this study was to determine the principal determinants of baseline and longitudinal health-related quality of life (HRQoL) in a large unselected IPF population. METHODS: We used the Australian IPF Registry to examine the relationship between HRQoL, measured using the St George Respiratory Questionnaire (SGRQ), and demographic features, physiological features, co-morbidities and symptoms. Linear regression analysis was performed to identify predictors of baseline HRQoL, linear mixed model analysis to determine the effect of time and forced vital capacity (FVC) on SGRQ and Cox proportional hazards regression to examine the relationship between HRQoL and all-cause mortality. RESULTS: Baseline data from 516 patients were available (347 males; mean (SD) age: 71.3 ± 8.6 years). Univariate analysis showed significant associations between HRQoL and demographic, clinical and physiological features. However, multivariate analysis demonstrated independent associations only between SGRQ and dyspnoea (University of California San Diego Shortness of Breathlessness Questionnaire (UCSD-SOBQ); R2 = 0.71, P < 0.0001), cough severity (visual analogue scale; R2 = 0.06, P < 0.0001) and depression (Hospital Anxiety and Depression Scale; R2 = 0.04, P < 0.0001). Linear mixed-effects modelling of combined baseline and longitudinal data confirmed these associations, as well as for FVC% predicted (P = 0.005). Multivariate Cox proportionate-proportional hazards regression analysis demonstrated no association between HRQoL and risk of mortality. CONCLUSION: Cough, dyspnoea and depression are major symptomatic determinants of HRQoL in IPF. FVC decline is associated with worsening HRQoL.

Clinical impact of the interstitial lung disease multidisciplinary service.

BACKGROUND AND OBJECTIVE: Multidisciplinary discussions (MDDs) have been shown to improve diagnostic accuracy in interstitial lung disease (ILD) diagnosis. However, their clinical impact on patient care has never been clearly demonstrated. We describe the effect that an ILD multidisciplinary service has upon the diagnosis and management of patients with suspected ILD. METHODS: Patients at two specialized centres with suspected ILD underwent ILD multidisciplinary team review (ILD-MDT) (standard ILD clinic visit and diagnostic review at ILD-MDD). We compared changes in ILD diagnosis and management at referral to those following the ILD-MDT. RESULTS: Ninety patients, 60% males (54/90), aged 67.3 years (SD = 11.4) were reviewed for suspected ILD. Overall, the ILD-MDT resulted in a change in specific ILD diagnosis in 48/90 (53%) patients. Of the 27 patients referred with a diagnosis of idiopathic pulmonary fibrosis (IPF), the diagnosis was changed at MDD in 10 patients. In contrast, seven patients had their diagnosis changed to IPF. There was also a significant reduction in 'unclassifiable' diseases and disease behaviour classifications provided additional information beyond ILD diagnosis. CONCLUSION: Dedicated tertiary ILD-MDT service has an important clinical impact on the care of the ILD patient, with frequent changes in ILD diagnosis and subsequent management. Further research to investigate long-term clinical outcomes of ILD-MDT is required.

Proximal and distal gastro-oesophageal reflux in chronic obstructive pulmonary disease and bronchiectasis.

BACKGROUND AND OBJECTIVE: The aims of this observational study were (i) to examine the prevalence of symptomatic and clinically silent proximal and distal gastro-oesophageal reflux (GOR) in adults with chronic obstructive pulmonary disease (COPD) or bronchiectasis, (ii) the presence of gastric aspiration, and (iii) to explore the possible clinical significance of this comorbidity in these conditions. METHODS: Twenty-seven participants with COPD, 27 with bronchiectasis and 17 control subjects completed reflux symptom evaluation and dual-channel 24 h oesophageal pH monitoring. In those with lung disease, pepsin levels in sputum samples were measured using enzyme-linked immunosorbent assay, with disease severity (lung function and high-resolution computed tomography) also measured. RESULTS: The prevalence of GOR in COPD was 37%, in bronchiectasis was 40% and in control subjects was 18% (P = 0.005). Of those diagnosed with GOR, clinically silent reflux was detected in 20% of participants with COPD and 42% with bronchiectasis. While pepsin was found in 33% of COPD and 26% of bronchiectasis participants, the presence of pepsin in sputum was not related to a diagnosis of GOR based on oesophageal pH monitoring in either condition. Neither a diagnosis of GOR nor the presence of pepsin was associated with increased severity of lung disease in COPD or bronchiectasis. CONCLUSIONS: The prevalence of GOR in COPD or bronchiectasis is twice that of the control population, and the diagnosis could not be based on symptoms alone. Pepsin was detected in sputum in COPD and bronchiectasis, suggesting a possible role of pulmonary aspiration, which requires further exploration.

Investigation of the safety of irreversible electroporation in humans.

PURPOSE: A single-center prospective nonrandomized cohort study was performed to investigate the safety of irreversible electroporation (IRE) for tumor ablation in humans. MATERIALS AND METHODS: Thirty-eight volunteers with advanced malignancy of the liver, kidney, or lung (69 separate tumors) unresponsive to alternative treatment were subjected to IRE under general anesthesia. Clinical examination, biochemistry, and computed tomography (CT) scans of the treated organ were performed before, immediately after, and at 1 month and 3 months after the procedure. RESULTS: No mortalities occurred at 30 days. Transient ventricular arrhythmia occurred in four patients, and electrocardiographically (ECG) synchronized delivery was used subsequently in the remaining 30 patients, with two further arrhythmias (supraventricular tachycardia and atrial fibrillation). One patient developed obstruction of the upper ureter after IRE. One adrenal gland was unintentionally directly electroporated, which produced transient severe hypertension. There was no other evidence of adjacent organ damage related to the electroporation. Other adverse events were not directly related to IRE, but two patients developed temporary neurapraxia as a result of arm extension during a prolonged period of anesthesia. Although not a primary aim of this preliminary study, complete target tumor ablation verified by CT was achieved in 46 of the 69 tumors treated with IRE (66%). Most treatment failures occurred in renal and lung tumors. Biopsy in three patients showed coagulative necrosis in the regions treated by IRE. CONCLUSIONS: IRE appears to be safe for human clinical use provided ECG-synchronized delivery is used. Comparative evaluation with alternative ablative technologies is warranted.

Should lung transplant recipients routinely perform airway clearance techniques? A randomized trial.

BACKGROUND AND OBJECTIVE: Despite the widespread use of airway clearance (AC) techniques to clear excessive secretions and improve lung function, little is known about their efficacy following lung transplantation (LTx). This study compared the effects of two AC strategies (proactive vs reactive) on a range of clinical outcomes following LTx. METHODS: A prospective randomized trial was conducted. Uncomplicated patients were recruited 1 month postoperatively. Patients performed AC using positive expiratory pressure either twice daily (proactive strategy) or only in the presence chest infection (reactive strategy). Lung function (FEV(1) and FVC), CXR (Brasfield score) and bronchoscopic airway characteristics (anastomotic healing, patency and secretions) were assessed at 1, 2 and 3 months postoperatively. Adherence was measured. RESULTS: Of 60 consecutive patients, 36 (18 proactive, 18 reactive) were recruited and completed the study. Both groups improved lung function (FEV(1) 72 +/- 4% to 81 +/- 4%, P < 0.0001; FVC 69 +/- 3% to 81 +/- 3%, P < 0.0001) and Brasfield scores (17.8 +/- 0.5 to 19.8 +/- 0.5, P < 0.002) over the study period. No significant differences for any outcome were found between the two groups. The vast majority of patients had fully healed, 100% patent anastomoses without secretions at 3 months. There were no significant differences between the two groups in airway characteristics and the incidence of chest infection. Adherence to both strategies was high (84% proactive, 100% reactive). CONCLUSIONS: Proactive AC following lung transplantation was not associated with a reduced incidence of respiratory infection, alteration of CXR findings or improvement in airway characteristics.

'Benign' hepatic portal venous gas.

The presence of portal venous gas within the hepatic parenchyma is usually associated with a guarded prognosis and a mortality rate approaching 75%. However, there are infrequent causes of portal venous gas not associated with dire clinical outcomes. We describe three patients who made uneventful clinical recoveries after presenting with clinical and imaging manifestations of ischaemic bowel and hepatic portal venous gas, two of which had distended but non-necrotic bowel at laparotomy.