INTRODUCTION: Respiratory pathogens are frequently isolated from airway samples in primary ciliary dyskinesia (PCD) patients. Few studies have investigated associations between these pathogens and lung function, with current management based on evidence from cystic fibrosis. We investigated the association between commonly isolated respiratory pathogens and lung function in PCD patients. METHODS: Using a cross-sectional design, we prospectively collected clinical and concurrent microbiology data from 408 participants with probable or confirmed PCD, aged ≥5 years, from 12 countries. We used Global Lung Function Initiative 2012 references to calculate forced expiratory volume in 1 s (FEV1) z-scores. For 351 patients (86%) with complete data, we assessed the association of the four most frequently isolated pathogens with lung function by fitting multilevel linear models with country as random intercept, adjusted for age at diagnosis, age at lung function, use of antibiotic prophylaxis and body mass index z-scores. RESULTS: Individuals with Pseudomonas aeruginosa growth in culture had significantly lower FEV1 z-scores (β= -0.87, 95% CI -1.40- -0.34), adjusted for presence of Haemophilus influenzae, methicillin-sensitive Staphylococcus aureus and Streptococcus pneumoniae, and for covariates. When stratified by age, associations remained strong for adults but not for children. Results were similar when ciliary defects by transmission electron microscopy were included in the models and when restricting analysis to only confirmed PCD cases. CONCLUSIONS: We found that P. aeruginosa was associated with worse lung function in individuals with PCD, particularly adults. These findings suggest that it is prudent to aim for P. aeruginosa eradication in the first instance, and to treat exacerbations promptly in colonised patients.

• Publikační typ
• časopisecké články MeSH

INTRODUCTION: In recent years, ventilatory efficiency (minute ventilation (V'E)/carbon dioxide production (V'CO2 ) slope) and partial pressure of end-tidal carbon dioxide (P ETCO2 ) have emerged as independent predictors of postoperative pulmonary complications (PPC). Single parameters may give only partial information regarding periprocedural hazards. Accordingly, our aim was to create prediction models with improved ability to stratify PPC risk in patients scheduled for elective lung resection surgery. METHODS: This post hoc analysis was comprised of consecutive lung resection candidates from two prior prospective trials. All individuals completed pulmonary function tests and cardiopulmonary exercise testing (CPET). Logistic regression analyses were used for identification of risk factors for PPC that were entered into the final risk prediction models. Two risk models were developed; the first used rest P ETCO2 (for patients with no available CPET data), the second used V'E/ V'CO2 slope (for patients with available CPET data). Receiver operating characteristic analysis with the De-Long test and area under the curve (AUC) were used for comparison of models. RESULTS: The dataset from 423 patients was randomly split into the derivation (n=310) and validation (n=113) cohorts. Two final models were developed, both including sex, thoracotomy, "atypical" resection and forced expiratory volume in 1 s/forced vital capacity ratio as risk factors. In addition, the first model also included rest P ETCO2 , while the second model used V'E/V'CO2 slope from CPET. AUCs of risk scores were 0.795 (95% CI: 0.739-0.851) and 0.793 (95% CI: 0.737-0.849); both p<0.001. No differences in AUCs were found between the derivation and validation cohorts. CONCLUSIONS: We created two multicomponental models for PPC risk prediction, both having excellent predictive properties.

• Publikační typ
• časopisecké články MeSH

AIMS: Impaired lung function has been strongly associated with cardiovascular disease (CVD) events. We aimed to assess the additive prognostic value of spirometry indices to the risk estimation of CVD events in Eastern European populations in this study. METHODS: We randomly selected 14,061 individuals with a mean age of 59 ± 7.3 years without a previous history of cardiovascular and pulmonary diseases from population registers in the Czechia, Poland, and Lithuania. Predictive values of standardised Z-scores of forced expiratory volume measured in 1 s (FEV1), forced vital capacity (FVC), and FEV1 divided by height cubed (FEV1/ht3) were tested. Cox proportional hazards models were used to estimate hazard ratios (HRs) of CVD events of various spirometry indices over the Framingham Risk Score (FRS) model. The model performance was evaluated using Harrell's C-statistics, likelihood ratio tests, and Bayesian information criterion. RESULTS: All spirometry indices had a strong linear relation with the incidence of CVD events (HR ranged from 1.10 to 1.12 between indices). The model stratified by FEV1/ht3 tertiles had a stronger link with CVD events than FEV1 and FVC. The risk of CVD event for the lowest vs. highest FEV1/ht3 tertile among people with low FRS was higher (HR: 2.35; 95% confidence interval: 1.96-2.81) than among those with high FRS. The addition of spirometry indices showed a small but statistically significant improvement of the FRS model. CONCLUSIONS: The addition of spirometry indices might improve the prediction of incident CVD events particularly in the low-risk group. FEV1/ht3 is a more sensitive predictor compared to other spirometry indices.

• Klíčová slova
• cardiovascular disease, cohort studies, mortality, pulmonary function test, risk prediction model,
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. METHODS: A retrospective, descriptive multicentre follow-up study initiated from the European network for translational research in children's and adult interstitial lung disease (Cost Action CA16125) and chILD-EU CRC (the European Research Collaboration for Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before the age of 18 years. RESULTS: Data of 124 patients from 26 centres (15 counties) were submitted, of whom 117 patients fulfilled the inclusion criteria. Diagnoses were idiopathic pulmonary haemosiderosis (n=35), DAH associated with autoimmune features (n=20), systemic and collagen disorders (n=18), immuno-allergic conditions (n=10), other childhood interstitial lung diseases (chILD) (n=5), autoinflammatory diseases (n=3), DAH secondary to other conditions (n=21) and nonspecified DAH (n=5). Median (IQR) age at onset was 5 (2.0-12.9) years. Most frequent clinical presentations were anaemia (87%), haemoptysis (42%), dyspnoea (35%) and cough (32%). Respiratory symptoms were absent in 23%. The most frequent medical treatment was systemic corticosteroids (93%), hydroxychloroquine (35%) and azathioprine (27%). Overall mortality was 13%. Long-term data demonstrated persistent abnormal radiology and a limited improvement in lung function. CONCLUSIONS: Paediatric DAH is highly heterogeneous regarding underlying causes and clinical presentation. The high mortality rate and number of patients with ongoing treatment years after onset of disease underline that DAH is a severe and often chronic condition. This large international study paves the way for further prospective clinical trials that will in the long term allow evidence-based treatment and follow-up recommendations to be determined.

• Publikační typ
• časopisecké články MeSH

BACKGROUND: Social differences in lung functioning have been reported, but the role of socioeconomic position (SEP) at different stages of life is less well understood, particularly in Central and Eastern Europe. This study addressed this question. METHODS: The analysis included 10 160 individuals aged 45-70 years from the Czech Republic, Poland and Lithuania. Lung function was either normal if values of forced expiratory volume in the first second divided by forced vital capacity (FEV1/FVC) and FVC were higher than the lower limit of normality or impaired if otherwise. SEP at three stages of life was assessed using maternal education (childhood), participant's education (young adulthood), and current ability to pay for food, clothes and bills (late adulthood). SEP measures were dichotomised as advantaged versus disadvantaged. The associations between impaired lung function and life-course SEP were estimated by logistic regression. RESULTS: Disadvantaged SEP in young and late adulthood had higher odds of impaired lung function. In young adulthood, age-adjusted ORs were 1.26 (95% CI 1.06 to 1.49) in men and 1.56 (95% CI 1.29 to 1.88) in women, while in late adulthood, the ORs were 1.15 (95% CI 0.99 to 1.34) in men and 1.26 (95% CI 1.09 to 1.46) in women. Men and women disadvantaged at all three stages of life had ORs of 1.42 (95% CI 1.06 to 1.91) and 1.83 (95% CI 1.32 to 2.52), respectively, compared with those always advantaged. Smoking substantially attenuated the ORs in men but not in women. CONCLUSION: Reducing socioeconomic inequalities in young and late adulthood may contribute to reducing the risk of impaired lung function in late adulthood.

• Klíčová slova
• epidemiology, health impact assessment, health inequalities, life course epidemiology, social class,
• Publikační typ
• časopisecké články MeSH

Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes lung function trajectories in children, adolescents and young adults with PCD. We analysed data from 486 patients with repeated lung function measurements obtained between the age of 6 and 24 years from the International PCD Cohort and calculated z-scores for forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC) and FEV1/FVC ratio using the Global Lung Function Initiative 2012 references. We described baseline lung function and change of lung function over time and described their associations with possible determinants in mixed-effects linear regression models. Overall, FEV1, FVC and FEV1/FVC z-scores declined over time (average crude annual FEV1 decline was -0.07 z-scores), but not at the same rate for all patients. FEV1 z-scores improved over time in 21% of patients, remained stable in 40% and declined in 39%. Low body mass index was associated with poor baseline lung function and with further decline. Results differed by country and ultrastructural defect, but we found no evidence of differences by sex, calendar year of diagnosis, age at diagnosis, diagnostic certainty or laterality defect. Our study shows that on average lung function in PCD declines throughout the entire period of lung growth, from childhood to young adult age, even among patients treated in specialised centres. It is essential to develop strategies to reverse this tendency and improve prognosis.

• MeSH
• dítě MeSH
• dospělí MeSH
• kohortové studie MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• plíce MeSH
• poruchy ciliární motility * MeSH
• usilovný výdechový objem MeSH
• vitální kapacita MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

It is unclear whether the dose-response relationship between lung function and all-cause and cardiovascular mortality in the Central and Eastern European populations differ from that reported in the Western European and American populations. We used the prospective population-based HAPIEE cohort that includes randomly selected people with a mean age of 59 ± 7.3 years from population registers in Czech, Polish, Russian and Lithuanian urban centres. The baseline survey in 2002-2005 included 36,106 persons of whom 24,944 met the inclusion criteria. Cox proportional hazards models were used to estimate the dose-response relationship between lung function defined as FEV1 divided by height cubed and all-cause and cardiovascular mortality over 11-16 years of follow-up. Mortality rate increased in a dose-response manner from highest to lower FEV1/height3 deciles. Adjusted hazard ratios (HR) of all-cause mortality for persons in the 8th best, the 5th and the worst deciles were 1.27 (95% CI 1.08‒1.49), 1.37 (1.18-1.60) and 2.15 (1.86‒2.48), respectively; for cardiovascular mortality, the respective HRs were 1.84 (1.29-2.63), 2.35 (1.67-3.28) and 3.46 (2.50‒4.78). Patterns were similar across countries, with some statistically insignificant variation. FEV1/height3 is a strong predictor of all-cause and cardiovascular mortality, across full distribution of values, including persons with preserved lung function.

• MeSH
• kardiovaskulární nemoci * epidemiologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• plíce MeSH
• proporcionální rizikové modely MeSH
• prospektivní studie MeSH
• rizikové faktory MeSH
• senioři MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• senioři MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Research Support, N.I.H., Extramural MeSH

BACKGROUND: The association between impaired lung function and mortality has been well documented in the general population of Western European countries. We assessed the risk of death associated with reduced spirometry indices among people from four Central and Eastern European countries. METHODS: This prospective population-based cohort includes men and women aged 45-69 years, residents in urban settlements in Czech Republic, Poland, Russia and Lithuania, randomly selected from population registers. The baseline survey in 2002-2005 included 36,106 persons of whom 24,993 met the inclusion criteria. Cox proportional hazards models were used to estimate the hazard ratios of mortality over 11-16 years of follow-up for mild, moderate, moderate-severe and very severe lung function impairment categories. RESULTS: After adjusting for covariates, mild (hazard ratio (HR): 1.25; 95% CI 1.15‒1.37) to severe (HR: 3.35; 95% CI 2.62‒4.27) reduction in FEV1 was associated with an increased risk of death according to degree of lung impairment, compared to people with normal lung function. The association was only slightly attenuated but remained significant after exclusion of smokers and participants with previous history of respiratory diseases. The HRs varied between countries but not statistically significant; the highest excess risk among persons with more severe impairment was seen in Poland (HR: 4.28, 95% CI 2.14‒8.56) and Lithuania (HR: 4.07, 95% CI 2.21‒7.50). CONCLUSIONS: Reduced FEV1 is an independent predictor of all-cause mortality, with risk increasing with the degree of lung function impairment and some country-specific variation between the cohorts.

• Klíčová slova
• Cohort study, Forced expiratory volume in one second, Mortality, Pulmonary function test,
• MeSH
• kardiovaskulární nemoci * epidemiologie   MeSH
• kohortové studie MeSH
• lidé MeSH
• plíce MeSH
• prospektivní studie MeSH
• rizikové faktory MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Geografické názvy
• Polsko epidemiologie   MeSH

BACKGROUND: Viral infections can cause significant morbidity in cystic fibrosis (CF). The current Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic could therefore have a serious impact on the health of people with CF (pwCF). METHODS: We used the 38-country European Cystic Fibrosis Society Patient Registry (ECFSPR) to collect case data about pwCF and SARS-CoV-2 infection. RESULTS: Up to 30 June 2020, 16 countries reported 130 SARS-CoV-2 cases in people with CF, yielding an incidence of 2.70/1000 pwCF. Incidence was higher in lung-transplanted patients (n=23) versus non-transplanted patients (n=107) (8.43 versus 2.36 cases/1000). Incidence was higher in pwCF versus the age-matched general population in the age groups <15, 15-24, and 25-49 years (p<0.001), with similar trends for pwCF with and without lung transplant. Compared to the general population, pwCF (regardless of transplantation status) had significantly higher rates of admission to hospital for all age groups with available data, and higher rates of intensive care, although not statistically significant. Most pwCF recovered (96.2%), however 5 died, of whom 3 were lung transplant recipients. The case fatality rate for pwCF (3.85%, 95% CI: 1.26-8.75) was non-significantly lower than that of the general population (7.46%; p=0.133). CONCLUSIONS: SARS-CoV-2 infection can result in severe illness and death for pwCF, even for younger patients and especially for lung transplant recipients. PwCF should continue to shield from infection and should be prioritized for vaccination.

• Klíčová slova
• Covid-19, Cystic fibrosis, Epidemiology, Europe, Incidence, SARS-CoV-2,
• MeSH
• COVID-19 diagnóza   epidemiologie   terapie   MeSH
• cystická fibróza komplikace   mortalita   terapie   MeSH
• dítě MeSH
• dospělí MeSH
• hospitalizace MeSH
• incidence MeSH
• kojenec MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• novorozenec MeSH
• péče o pacienty v kritickém stavu MeSH
• předškolní dítě MeSH
• registrace MeSH
• retrospektivní studie MeSH
• transplantace plic MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• kojenec MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• pozorovací studie MeSH
• Geografické názvy
• Evropa epidemiologie   MeSH

The use of pulmonary function tests (PFTs) has been widely described in airway diseases like asthma and cystic fibrosis, but for children's interstitial lung disease (chILD), which encompasses a broad spectrum of pathologies, the usefulness of PFTs is still undetermined, despite widespread use in adult interstitial lung disease. A literature review was initiated by the COST/Enter chILD working group aiming to describe published studies, to identify gaps in knowledge and to propose future research goals in regard to spirometry, whole-body plethysmography, infant and pre-school PFTs, measurement of diffusing capacity, multiple breath washout and cardiopulmonary exercise tests in chILD. The search revealed a limited number of papers published in the past three decades, of which the majority were descriptive and did not report pulmonary function as the main outcome.PFTs may be useful in different stages of management of children with suspected or confirmed chILD, but the chILD spectrum is diverse and includes a heterogeneous patient group in all ages. Research studies in well-defined patient cohorts are needed to establish which PFT and outcomes are most relevant for diagnosis, evaluation of disease severity and course, and monitoring individual conditions both for improvement in clinical care and as end-points in future randomised controlled trials.

• MeSH
• dítě MeSH
• intersticiální plicní nemoci diagnóza   patofyziologie   MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• předškolní dítě MeSH
• respirační funkční testy * MeSH
• věkové faktory MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH