David Bilocca

A delayed diagnosis of Mounier-Kuhn syndrome

Following a provisional diagnosis of asthma of several years’ duration by his general practitioner, a 43-year-old otherwise healthy man who was a non-smoker was referred to a pulmonologist with worsening productive cough and exertional breathlessness. A thoracic CT scan revealed dilated airways (tracheal diameter 35 mm, left bronchial diameter 20 mm, right bronchial diameter 18 mm). Inflamed and easily collapsible airways were seen on bronchoscopy. The patient remained stable and was followed up with regular spirometry. A follow-up CT scan 7 years later showed tracheobronchomegaly (tracheal diameter 42 mm, left bronchial diameter 25 mm, right bronchial diameter 23 mm) with large cystic spaces consistent with Mounier-Kuhn syndrome. Repeat bronchoscopy showed a massively dilated trachea and generalised collapse on expiration with a dilated thin-walled bronchial tree. He was deemed ineligible for lung transplantation due to the extent of airway involvement making it difficult to anastomose donor lung to native tissue.

A diagnostic dilemma in a case of pulmonary inflammatory myofibroblastic tumour

An 18-year-old man presented to the local hospital in Malta, with dyspnoea, cough, mild haemoptysis, chest pain and night sweats. CT revealed a right hilar mass. Pleural tap, bronchoscopy and open lung biopsy were inconclusive. Biopsies obtained at repeat bronchoscopy and endobronchial ultrasound (EBUS) revealed a likely diagnosis of inflammatory myofibroblastic tumour (IMT). The patient subsequently underwent right pneumonectomy, and histology revealed the presence of two further nodules apart from the main tumour. Follow-up with positron emission tomography (PET)/CT showed the development of a right basal paracardial lesion due to recurrence and the presence of lymph node, pleural and skeletal disease. Despite radiotherapy to the recurrent nodule and chemotherapy, there was skeletal disease progression. Treatment with an anaplastic lymphoma kinase inhibitor, ceritinib, resulted in very good metabolic response. This case report highlights the importance of keeping IMT in mind when the diagnosis of lung tumours is difficult, as delayed diagnosis may lead to worsened prognosis.

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.

Relationship between domestic smoking and metals and rare earth elements concentration in indoor PM2.5 - The Indoor and Outdoor Air Quality and Respiratory Health in Malta and Sicily

Abstract Cigarette smoke is the main source of indoor chemical and toxic elements. Cadmium (Cd), Thallium (Tl), Lead (Pb) and Antimony (Sb) are important contributors to smoke‐related health risks. Data on the association between Rare Earth Elements (REE) Cerium (Ce) and Lanthanum (La) and domestic smoking are scanty. To evaluate the relationship between cigarette smoke, indoor levels of PM2.5 and heavy metals, 73 children were investigated by parental questionnaire and skin prick tests. The houses of residence of 41 “cases” and 32 “controls” (children with and without respiratory symptoms, respectively) were evaluated by 48‐h PM2.5 indoor/outdoor monitoring. PM2.5 mass concentration was determined by gravimetry; the extracted and mineralized fractions of elements (As, Cd, Ce, La, Mn, Pb, Sb, Sr, Tl) were evaluated by ICP‐MS. PM2.5 and Ce, La, Cd, and Tl indoor concentrations were higher in smoker dwellings. When corrected for confounding factors, PM2.5, Ce, La, Cd, and Tl were associated with more likely presence of respiratory symptoms in adolescents. We found that: i) indoor smoking is associated with increased levels of PM2.5, Ce, La, Cd, and Tl and ii) the latter with increased presence of respiratory symptoms in children. Graphical abstract Figure. No Caption available. HighlightsTobacco smoke releases heavy metals (HM) and REE in indoor dwellings.Elemental content in indoor PM2.5 was measured in smoking and non smoking dwellings.Extracted and mineralized fractions of elements were evaluated by ICP‐MS.Indoor smoke was associated with increase of specific elemental fractions in PM2.5.Increased levels of HM and REE are associated with respiratory symptoms in children.

Sensitization to dust mite defines different phenotypes of asthma: A multicenter study

Indoor allergens are risk factors for asthma: Thus, the characterization of indoor air quality is important for studying environment–health relationships in children. In particular, Dermatophagoides pteronyssinus is the dominant allergen for asthma. We cross‐sectionally investigated the relationships among respiratory symptoms and function, airway inflammation, allergen sensitization, and indoor allergen concentration.