The role of radiology in addressing the challenge of lung cancer after lung transplantation.

The importance of lung cancer as a complication of lung transplantation is increasingly recognised. It may become an important survival-limiting factor in lung transplant patients as management of other complications continues to improve and utilisation of extended criteria donors grows. Radiology can play a key role in tackling this issue at multiple stages in the transplantation pathway and follow-up process.

Treatment-resistant hypertension in a post-transplant patient with cystic fibrosis: a rare case of phaeochromocytoma.

Summary: Phaeochromocytoma is a rare catecholamine-producing tumour. We present the case of phaeochromocytoma in a young man with a background history of a double-lung transplant for cystic fibrosis (CF). Clinical case: A 25-year-old man, with a background history of CF, CF-related diabetes (CFRD) and a double-lung transplant in 2012 was presented to the emergency department with crampy abdominal pain, nausea and vomiting.

Delivery of nucleic acids to ex vivo porcine airways using electrospray.

Aim Of The Study: Nucleic acid-based therapies have the potential to provide clinically meaningful benefit across a wide spectrum of lung disease. However, in vivo delivery remains a challenge. Here we examined the feasibility of using electrospray to deliver nucleic acids to both porcine tracheal tissue sections and whole lung ex vivo.

Near-fatal mucormycosis post-double lung transplant presenting as uncontrolled upper gastrointestinal haemorrhage.

Invasive fungal infections in immunosuppressed transplant patients are associated with significant morbidity and mortality. We present a case of splenic mucormycosis post-double lung transplant, presenting as uncontrolled near-fatal upper gastrointestinal haemorrhage, to remind clinicians of the need to consider pre-transplant invasive fungal infection risk factors if an unexpected fungal infection arises in the post-transplant period. This case also highlights the valuable contribution of molecular technology for fungal identification but also the need for clinical correlation.

Successful surgical management of early esophageal cancer in a patient with cystic fibrosis post-bilateral lung transplantation.

We present the first reported case of successful surgical management of esophageal cancer post-lung transplantation for cystic fibrosis. This case of a 42-year-old man highlights the risk factors for esophageal adenocarcinoma associated with cystic fibrosis and lung transplantation, the management options for early esophageal cancer and the surgical option chosen to minimise respiratory risks. Individualised patient care may allow for curative surgical approaches, even where complex surgery is required.

Anatomy and bronchoscopy of the porcine lung. A model for translational respiratory medicine.

The porcine model has contributed significantly to biomedical research over many decades. The similar size and anatomy of pig and human organs make this model particularly beneficial for translational research in areas such as medical device development, therapeutics and xenotransplantation. In recent years, a major limitation with the porcine model was overcome with the successful generation of gene-targeted pigs and the publication of the pig genome.

Pirfenidone in idiopathic pulmonary fibrosis: expert panel discussion on the management of drug-related adverse events.

Pirfenidone is currently the only approved therapy for idiopathic pulmonary fibrosis, following studies demonstrating that treatment reduces the decline in lung function and improves progression-free survival. Although generally well tolerated, a minority of patients discontinue therapy due to gastrointestinal and skin-related adverse events (AEs). This review summarizes recommendations based on existing guidelines, research evidence, and consensus opinions of expert authors, with the aim of providing practicing physicians with the specific clinical information needed to educate the patient and better manage pirfenidone-related AEs with continued pirfenidone treatment.

Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial.

Background: Idiopathic pulmonary fibrosis (IPF) is characterized by formation and proliferation of fibroblast foci. Endothelin-1 induces lung fibroblast proliferation and contractile activity via the endothelin A (ETA) receptor.

Objective: To determine whether ambrisentan, an ETA receptor-selective antagonist, reduces the rate of IPF progression.

The pathogenesis of pulmonary fibrosis: a moving target.

Pulmonary fibrosis is the end stage of many diffuse parenchymal lung diseases. It is characterised by excessive matrix formation leading to destruction of the normal lung architecture and finally death. Despite an exponential increase in our understanding of potentially important mediators and mechanisms, the delineation of primary pathways has proven to be elusive.

Characterization of outcomes 1 year after endoscopic thermal vapor ablation for patients with heterogeneous emphysema.

Introduction: Endoscopic lung volume reduction has been developed as a therapeutic option for advanced emphysema. Six-month results following treatment with endoscopic thermal vapor ablation (InterVapor; Uptake Medical, Tustin, CA) were described previously, and here we report observations from the 12-month assessment.

Methods: Two multicenter, international, single-arm trials of InterVapor (unilateral upper lobe treatment) in patients with upper lobe predominant emphysema were conducted.

International collaboration: a retrospective study examining the survival of Irish citizens following lung transplantation in both the UK and Ireland.

Objective: Prior to 2005, Irish citizens had exclusively availed of lung transplantation services in the UK. Since 2005, lung transplantation has been available to these patients in both the UK and Ireland. We aimed to evaluate the outcomes of Irish patients undergoing lung transplantation in both the UK and Ireland.

Efficacy predictors of lung volume reduction with Zephyr valves in a European cohort.

The Endobronchial Valve for Emphysema Palliation Trial (VENT) was a multi-centre, prospective, randomised, controlled trial conducted to evaluate the safety and effectiveness of unilateral endobronchial valve (EBV) treatment. The purpose of this analysis was to assess outcomes in the previously unreported European VENT study cohort. Patients with advanced emphysema were randomly assigned (2:1) to receive Zephyr® (Pulmonx Inc.

Endobronchial valve deployment in severe α-1 antitrypsin deficiency emphysema: a case series.

Introduction: Patients with end-stage emphysema because of α-1 antitrypsin (AAT) deficiency represent a challenging clinical management problem, and studies of volume reduction therapy to date have largely excluded these patients. We report the outcome of bronchoscopic volume reduction with the insertion of Emphasys endobronchial valves (Emphasys Medical, Redwood City, CA, USA) in six patients with end-stage emphysema because of AAT deficiency.

Case Series: Of 51 patients with end stage emphysema referred for transplantation, we studied six patients with AAT deficiency and utilized the BODE index and lung allocation score for survival estimation.

Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis.

The aim of this study was to evaluate the risk factors for and outcomes of acute exacerbations in patients with advanced idiopathic pulmonary fibrosis (IPF), and to examine the relationship between disease severity and neovascularisation in explanted IPF lung tissue. 55 IPF patients assessed for lung transplantation were divided into acute (n=27) and non-acute exacerbation (n=28) groups. Haemodynamic data was collected at baseline, at the time of acute exacerbation and at lung transplantation.

Bronchoscopic thermal vapour ablation therapy in the management of heterogeneous emphysema.

The need for a less invasive procedure than surgical lung volume reduction that can produce consistent improvements with reduced morbidity remains a medical goal in patients with emphysema. We sought to determine the effect of bronchoscopic thermal vapour ablation (BTVA) on lung volumes and outcomes in patients with emphysema. 44 patients with upper lobe-predominant emphysema were treated unilaterally with BTVA.

The Novalung interventional lung assist as bridge to lung transplantation for self-ventilating patients - initial experience.

We report the use of the Novalung pumpless device in self-ventilating patients awaiting a lung transplantation. Two patients developed carbon dioxide retention with respiratory acidosis that did not respond to maximum medical therapy. The Novalung interventional lung assist was established as a bridge to lung transplantation.

An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management.

This document is an international evidence-based guideline on the diagnosis and management of idiopathic pulmonary fibrosis, and is a collaborative effort of the American Thoracic Society, the European Respiratory Society, the Japanese Respiratory Society, and the Latin American Thoracic Association. It represents the current state of knowledge regarding idiopathic pulmonary fibrosis (IPF), and contains sections on definition and epidemiology, risk factors, diagnosis, natural history, staging and prognosis, treatment, and monitoring disease course. For the diagnosis and treatment sections, pragmatic GRADE evidence-based methodology was applied in a question-based format.