Eleanor Gerada

The EMBARC European bronchiectasis registry: Protocol for an international observational study

Bronchiectasis is one of the most neglected diseases in respiratory medicine. There are no approved therapies and few large-scale, representative epidemiological studies. The EMBARC (European Multicentre Bronchiectasis Audit and Research Collaboration) registry is a prospective, pan-European observational study of patients with bronchiectasis. The inclusion criterion is a primary clinical diagnosis of bronchiectasis consisting of: 1) a clinical history consistent with bronchiectasis; and 2) computed tomography demonstrating bronchiectasis. Core exclusion criteria are: 1) bronchiectasis due to known cystic fibrosis; 2) age <18 years; and 3) patients who are unable or unwilling to provide informed consent. The study aims to enrol 1000 patients by April 2016 across at least 20 European countries, and 10 000 patients by March 2020. Patients will undergo a comprehensive baseline assessment and will be followed up annually for up to 5 years with the goal of providing high-quality longitudinal data on outcomes, treatment patterns and quality of life. Data from the registry will be available in the form of annual reports. and will be disseminated in conference presentations and peer-reviewed publications. The European Bronchiectasis Registry aims to make a major contribution to understanding the natural history of the disease, as well as guiding evidence-based decision making and facilitating large randomised controlled trials. The European Bronchiectasis Registry will recruit 10 000 patients over 5 years http://ow.ly/Ul7Pd

Accidental severe bronchial aspiration of barium uncovers diagnosis of bronchiectasis

A 77-year-old man, with a history of chronic obstructive pulmonary disease (COPD) and radiotherapy-treated laryngeal malignancy diagnosed 15 years prior, presented with a 1-year history of intermittent dysphagia. As a part of his investigations, a barium swallow was obtained; but during the initial phases of the procedure, the patient aspirated the contrast agent of barium sulphate. A chest radiograph (Fig. 1a), taken few minutes after the aspiration, showed barium within the different segments and subsegments of the bronchial tree bilaterally, and uncovered features, such as irregular dilatation of the bronchial tree, non-tapering of the bronchi, luminal filling defects and lack of bronchial side branches, characteristic of bronchiectasis. This incident precipitated an exacerbation of COPD with type II respiratory failure, requiring bronchodilators, intravenous antibiotics and non-invasive ventilation using a bilevel positive airway pressure machine. On recovery, direct laryngoscopy revealed post-radiotherapy changes but no evidence of tumour recurrence. A speech and language pathology assessment using a few millilitres of water revealed weak laryngeal elevation, and immediate aspiration of water on swallowing. Given the high risk of further aspirations, advice regarding the benefits and risks of nasogastric tube insertion or percutaneous endoscopic gastrostomy tube insertion were given to the patient, but he declined both procedures. Six months following discharge, he was readmitted with an episode of food aspiration. A chest radiograph (Fig. 1b) revealed residual barium in both lung bases. This event precipitated another exacerbation of the COPD and bronchiectasis, which caused the patient to succumb to the disease. As early as the 1920s, the concept of utilizing contrast agents to aid in the diagnosis of pulmonary diseases, in particular bronchiectasis, had transpired. This involved instilling the contrast medium within the bronchial tree, by means of a catheter introduced in the trachea, and followed by the acquisition of various radiographs; a procedure known as bronchography. The initial contrast agents that were used in this regard were mainly waterand oil-based solutions. Subsequently in 1950s, barium sulphate, an inert and non-toxic agent, became a popular bronchographic medium that produced similar images as the earlier contrast agents, until the mid-1970s, when computed tomography became the gold standard imaging study for the investigation of pulmonary diseases. Although the earlier contrast agents, as well as barium sulphate, were considered safe bronchographic media, reports of pneumonitis and even death had been described with both [1]. The instilled contrast agent was found to cause obstruction of the small bronchi, followed by atelectasis of lung tissue and pneumonitis [2]. COPD patients often developed exacerbations after such procedures, as seen in our case. To minimize these risks, after the acquisition of the radiographs, patients were asked to cough and expectorate as much contrast dye as possible in an attempt to clear the airways. However, this mechanism would have been ineffective in cases of extensive small airway filling or ‘alveolarization’ with the contrast medium, as the alveoli lies outside the range of the normal mechanisms (cough and ciliary action) for cleansing the bronchial tree. The case we present demonstrates bronchographic features of bronchiectasis following accidental aspiration of E. Gerada (&) J. Gerada Department of Medicine, Mater Dei Hospital, Msida MSD 2090, Malta e-mail: egerada@gmail.com

Pulmonary rehabilitation in chronic obstructive pulmonary disease: Outcomes in a 12 week programme

Abstract Objective. The optimal time-frame for pulmonary rehabilitation (PR) in patients diagnosed with chronic obstructive pulmonary disease (COPD) is still debated. A 12 week programme was designed looking at whether the benefits were reached at or before a 12 week period of PR for COPD patients. Method. Seventy-five patients (59 males, 16 females) aged 40 75 years were referred from the local general hospital in Malta. Baseline assessments were carried out on all patients 2 weeks before initiation of the programme. Sixty patients were eligible to start a twice-weekly, 12 week multidisciplinary programme delivered after the screening process. The Six-Minute Walk Test (6MWT), dyspnoea score using the Borg scale, spirometry testing, plethysmography, COPD Assessment Tool (CAT) score, St Georges Respiratory Questionnaire (SGRQ) and Hospital and Anxiety scale score were monitored at 4 weekly intervals throughout the 12 weeks of PR for these COPD patients. Results. The 6MWT distance increased by a mean total of 132.45 m (p < 0.001) by 12 weeks, with the highest change recorded in the first 4 weeks for the milder COPD patients. Lung function test improvements were marginal. Borg scale readings at rest and following exertion decreased significantly from weeks 0 to 4 but remained fairly constant thereafter. The Body mass index, airway Obstruction, Dyspnoea, and Exercise capacity (BODE) index, SGRQ and CAT score values decreased significantly throughout the weeks irrespective of the initial Medical Research Council score. Anxiety scoring decreased significantly by 12 weeks, while the depression rating improved by 8 weeks. Conclusion. These findings show that 12 weeks of PR in this cohort of COPD patients resulted in clinically significant changes in functional outcome measures which are supported by statistically significant changes in health-related quality of life measures. In milder COPD cases, by 4 weeks of PR gains in exercise tolerance had already resulted. The more severe group required more time to obtain improvements. Therefore, hospitals could organize shorter PR programmes for larger numbers of patients with milder COPD.

Atypical Cavitary Lung Lesions: A Case Report and Review of Radiologic Manifestations

A 68-year-old woman presented to our emergency department with a 15 day history of epigastric discomfort radiating to the back. She had undergone a hysterectomy with bilateral salpingo-oophorectomy ten years before, for a FIGO stage 1 endometrioid adenocarcinoma. While abdominal examination was unremarkable, her liver function tests showed a cholestatic picture. An ultrasound scan of the abdomen revealed several gallstones as to the cause of this pain. Incidentally on chest radiograph, several lung lesions were noted bilaterally (Figure 1). On further questioning, the lady admitted to a one-year history of asthma with cough during the day and exertional dyspnoea. Inhaled corticosteroids prescribed for her ‘asthma’ proved to be ineffective. She was a non-smoker. She denied other respiratory symptoms, night sweats, chills, rigors or weight loss. She managed a good appetite and a good exercise tolerance for her age. There was no history of old TB, recurrent chest infections, recent travel or the presence of any mould at home.