Background: Lung resection is a controversial and understudied therapeutic modality in Primary Ciliary Dyskinesia (PCD). We assessed the prevalence of lung resection in PCD across countries and compared disease course in lobectomised and non-lobectomised patients. Methods: In the international iPCD cohort, we identified lobectomised and non-lobectomised age and sex-matched PCD patients and compared their characteristics, lung function and BMI cross-sectionally and longitudinally. Results: Among 2896 patients in the iPCD cohort, 163 from 20 centers (15 countries) underwent lung resection (5.6%). Among adult patients, prevalence of lung resection was 8.9%, demonstrating wide variation among countries. Compared to the rest of the iPCD cohort, lobectomised patients were more often females, older at diagnosis, and more often had situs solitus. In about half of the cases (45.6%) lung resection was performed before presentation to specialized PCD centers for diagnostic work-up. Compared to controls (n = 197), lobectomised patients had lower FVC z-scores (− 2.41 vs − 1.35, p = 0.0001) and FEV1 z-scores (− 2.79 vs − 1.99, p = 0.003) at their first post-lung resection assessment. After surgery, lung function continued to decline at a faster rate in lobectomised patients compared to controls (FVC z-score slope: − 0.037/year Vs − 0.009/year, p = 0.047 and FEV1 z-score slope: − 0.052/year Vs − 0.033/year, p = 0.235), although difference did not reach statistical significance for FEV1. Within cases, females and patients with multiple lobe resections had lower lung function. Conclusions: Prevalence of lung resection in PCD varies widely between countries, is often performed before PCD diagnosis and overall is more frequent in patients with delayed diagnosis. After lung resection, compared to controls most lobectomised patients have poorer and continuing decline of lung function despite lung resection. Further studies benefiting from prospective data collection are needed to confirm these findings.

Introduction: Understanding the factors associated with the development of ventilator-associated pneumonia (VAP) in critically ill patients in the intensive care unit (ICU) will allow for better prevention and control of VAP. The aim of the study was to evaluate the incidence of VAP, as well as to determine risk factors and protective factors against VAP. Design: Mixed prospective and retrospective cohort study. Methods: The cohort involved 371 critically ill patients who received standard interventions to prevent VAP. Additionally, patients in the prospective cohort were provided with continuous automatic pressure control in tapered cuffs of endotracheal or tracheostomy tubes and continuous automatic subglottic secretion suction. Logistic regression was used to assess factors affecting VAP. Results: 52 (14%) patients developed VAP, and the incidence density of VAP per 1000 ventilator days was 9.7. The median days to onset of VAP was 7 [4; 13]. Early and late onset VAP was 6.2% and 7.8%, respectively. According to multivariable logistic regression analysis, tracheotomy (OR = 1.6; CI 95%: 1.1 to 2.31), multidrug-resistant bacteria isolated in the culture of lower respiratory secretions (OR = 2.73; Cl 95%: 1.83 to 4.07) and ICU length of stay >5 days (OR = 3.32; Cl 95%: 1.53 to 7.19) were positively correlated with VAP, while continuous control of cuff pressure and subglottic secretion suction used together were negatively correlated with VAP (OR = 0.61; Cl 95%: 0.43 to 0.87). Conclusions: Tracheotomy, multidrug-resistant bacteria, and ICU length of stay >5 days were independent risk factors of VAP, whereas continuous control of cuff pressure and subglottic secretion suction used together were protective factors against VAP.

Introduction: The available literature lacks data regarding the levels of resistin, lysozyme, lactoferrin, α-amylase activity, pH, and saliva buffer capacity, as well as oral health and hygiene in the group of adult patients with cystic fibrosis (CF). The aim of the research was to assess the selected saliva parameters in patients diagnosed with cystic fibrosis. Materials and methods: Examined group was composed of 40 patients diagnosed with CF, while the control group of 40 healthy individuals. Both groups underwent the same scheme of the assessment (DMT index, salivary pH, buffer capacity, analysis of total sialic acid, total protein estimation, lysozyme levels estimation, lactofferin levels measurement, α-amylase activity, estimation of the levels of resistin and TNF-α). Results: In the examined group, there were higher values of decayed teeth as well as values of sialic acid, total protein, lactoferrin, α-amylase, and TNF-α. However, mean lysozyme, and resistin levels, as well as pH and buffer capacity of the saliva, were lower. Conclusions: New diagnostic methods, including the evaluation of selected salivary biochemical parameters, may indicate the existence of factors predisposing to severe tooth decay in the study group. Appropriate preventive treatment to combat dental caries in adult patients with CF will significantly improve their comfort and life expectancy.

In about 3% of children, viral infections of the airways that develop in early childhood lead to narrowing of the laryngeal lumen in the subglottic region resulting in symptoms such as hoarseness, abarking cough, stridor, and dyspnea. These infections may eventually cause respiratory failure. The disease is often called acute subglottic laryngitis (ASL). Terms such as pseudocroup, croup syndrome, acute obstructive laryngitis and spasmodic croup are used interchangeably when referencing this disease. Although the differential diagnosis should include other rare diseases such as epiglottitis, diphtheria, fibrinous laryngitis and bacterial tracheobronchitis, the diagnosis of ASL should always be made on the basis of clinical criteria.

Background: Plastic bronchitis (PB) may occur not only in children following palliative Fontan procedure but also in those without underlying heart disease. We aim to assess the clinical course, therapeutic measures, outcome, and follow-up of PB in children with congenital heart disease (CHD) and children without cardiac problems. Methods: This retrospective case series assessed children with PB admitted to hospital between 2015 and 2019. Parents or guardians of patients were contacted by e-mail or telephone between September 2017 and June 2019 to enquiry about recurrence of PB and strategy of treatment. The diagnosis of PB was based on the expectoration (spontaneous or during bronchoscopy) of endobronchial plugs. Results: This study delineated the clinical, histological, and laboratory features of plastic bronchitis in children following Fontan procedure (Group A) and in those without heart defects (Group B, non-CHD children). The main symptoms were cough accompanied by dyspnea, and hypoxemia with a decrease in oxygen saturation, often leading to acute respiratory failure. In children with CHD, the first episode of PB occurred at a relatively young age. Although chronic, i.e., lasting more than 3 weeks, inhaled therapy was implemented in both groups of patients, the recurrences of PB were observed. The mean time to PB recurrence after the first episode in Group A was longer than that in Group B (1.47 vs. 0.265 years, p = 0.2035). There was no re-episode with recurrence of PB in 3 cases out of 10 in total in Group A (30%) and 1 case out of 4 in total in Group B (25%). While the majority of children in Group A usually developed bronchial casts on the right side, the patients in Group B (without CHD) suffered from bronchial casts located only on the left side. Conclusions: Despite many similarities, clinical, histological, and laboratory studies in the children with plastic bronchitis after Fontan’s surgery and in children without heart defects suggest that there are differences in the course of the disease in patients without CHD, such as a more advanced age of the first episode of PB, the location of plastic casts on the left side, and a stronger role of inflammatory factors and mechanisms. Further research is needed to understand the pathophysiology of PB and choose the most appropriate therapy.