BACKGROUND AND AIM: This study was carried out to assess relationship between quality of life (QoL) and disease severity expressed by multifactorial prognostic index (BODE) in ex-smokers suffering from chronic obstructive pulmonary disease (COPD), minimally 8 weeks free of exacerbation. MATERIALS AND METHODS: The evaluation was performed in 98 randomly recruited COPD patients enrolled into a cross-sectional, observational CILIARY study at the Department of Pneumology, Charles University, Faculty of Medicine in Hradec Králové. In them, quality of life evaluation using the SGRQ questionnaire and the BODE index calculation was performed. We statistically compared interrelationship between BODE and COPD stages, SGRQ and COPD stages and interrelation of BODE and SGRQ. RESULTS: We found significant differences in QoL of COPD patients and QoL in group of healthy volunteers (p <0.001). Lower QoL and higher BODE score were associated with a higher stages of COPD (p < 0.001), with the exception non-significant difference in QoL (SGRQ score) and BODE index between stages I and II. Our study found positive correlation between the all SGRQ scores and multidimensional prognostic BODE index (r = 0.431-0.704). The strongest correlation (r = 0.704) was evident in activity domain of SGRQ. CONCLUSION: Our results proved close correlation ofquality of life (SGRQ) and multidimensional prognostic score (BODE) in stable COPD exsmokers' population. Both these scoring systems are useful tools for the assessment of clinical course and stratification of severity of COPD. However at present both scales are minimally used in the Czech Republic.

• MeSH
• chronická obstrukční plicní nemoc patofyziologie   MeSH
• dyspnoe MeSH
• kvalita života * MeSH
• lidé středního věku MeSH
• lidé MeSH
• odvykání kouření * MeSH
• prognóza MeSH
• průzkumy a dotazníky MeSH
• senioři MeSH
• usilovný výdechový objem MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH

OBJECTIVES: The BODE (BMI, Obstruction - FEV1, Dyspnoea - mMRC, Exercise - 6-MWT) and the ADO (Age, Dyspnoea - mMRC, Obstruction - FEV1) indices are widely used prognosis assessment tools for long-term mortality prediction in COPD patients but subject to limitations for use in daily clinical practice. The aim of this research was to construct a prognostic instrument that prevents these limitations and which would serve as a complementary prognostic tool for clinical use in these patients. METHODS AND PARTICIPANTS: The data of 699 COPD subjects were extracted from the Czech Multicentre Research Database (CMRD) of COPD patients (the derivation cohort) and analysed to identify factors associated with the long-term risk of mortality. These were entered into the ROC analysis and reclassification analysis. Those with the strongest discriminative power were used to construct the new index (CADOT). The new index was validated on 187 patients of the CIROCO+ cohort (Netherlands; the validation cohort). RESULTS: The CADOT was constructed by adding two newly identified prognosis-determining factors, chronic heart failure (CHF) and TLCO, to the ADO index. In a head-to-head comparison, the CADOT index showed highest c-statistic values compared to the BODE and ADO indices (0.701 vs 0.677 vs 0.644, respectively). The prognostic power was more definitive when applied to the Dutch validation (CIROCO+) cohort (0.842 vs 0.799 vs 0.825, respectively). CONCLUSIONS: The CADOT index has comparable prognostic power to the BODE and ADO indices. The CADOT is complementary/an alternative to the BODE (if 6-MWT is not feasible) and ADO (with less dependence on the age factor) indices. TRIAL REGISTRATION: ClinicalTrials.gov (NCT01923051).

• Klíčová slova
• COPD, mortality, prognostic index, pulmonary function,
• MeSH
• chronická obstrukční plicní nemoc * mortalita   MeSH
• dyspnoe MeSH
• index tělesné hmotnosti MeSH
• lidé MeSH
• prognóza MeSH
• respirační funkční testy MeSH
• stupeň závažnosti nemoci MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: The evaluation of chronic obstructive pulmonary disease (COPD) has been shifting from spirometry to focus on the patients' overall health. Despite the existence of many COPD prognostic scales, there remains a large gap for improvement, in particular a scale that incorporates the current focus on overall health. METHODS: We proposed a new prognostic scale (the COPD Prognostic Score) through discussion among the authors based on published studies. Validation was retrospective, using data from the National Emphysema Treatment Trial. RESULTS: The scores ranged from 0-16, where 16 indicated the poorest prognosis. We assigned 4 points each for forced expiratory volume in one second (%predicted), the modified Medical Research Council dyspnea scale, and age; 2 points for the hemoglobin level; and one point each for decreased activity and respiratory emergency admission in the last two years. The validation cohort included 607 patients and consisted of 388 men (73.9%) and 219 women (36.1%), mean age 67 ± 6 years and an average forced expiratory volume in one second (% predicted) of 27 ± 7%. A one-point increase in the score was associated with increased all-cause death, with a hazard ratio of 1.28 (95%CI: 1.21-1.36. P < 0.001). The areas under the receiver operating characteristic curves for two-year and five-year all-cause death for the new scale were 0.72 and 0.66, respectively. These values were higher than those given by the body mass index, airflow obstruction, dyspnea, and exercise capacity (BODE) index and age, dyspnea, airway obstruction (ADO) index. CONCLUSION: The preliminary validation for a new COPD prognostic scale: the COPD Prognostic Score was developed with promising results thus far. Above mentioned 16-point score accurately predicted 2-year and 5-year all-cause mortality among subjects who suffered from severe and very severe COPD.

• Klíčová slova
• COPD, age, dyspnea, hemoglobin, prognosis, spirometry,
• MeSH
• chronická obstrukční plicní nemoc mortalita   patofyziologie   MeSH
• chůze fyziologie   MeSH
• dyspnoe mortalita   patofyziologie   MeSH
• hemoglobiny metabolismus   MeSH
• Kaplanův-Meierův odhad MeSH
• lidé středního věku MeSH
• lidé MeSH
• prognóza MeSH
• průzkumy a dotazníky MeSH
• retrospektivní studie MeSH
• senioři MeSH
• spirometrie metody   MeSH
• stupeň závažnosti nemoci * MeSH
• usilovný výdechový objem fyziologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• validační studie MeSH
• Názvy látek
• hemoglobiny MeSH

PURPOSE: Patients with COPD present peripheral muscle dysfunction and atrophy, expressed as muscle strength and endurance reduction. The goal of this study was direct dynamometric assessment of hand grip endurance and strength in relation to the stage of disease, multidimensional predictors of mortality, and 6-minute walk test (6MWT). To the best of our knowledge, there has been no previous study determining these parameters. PATIENTS AND METHODS: In this observational study, 58 consecutive outpatients with stable COPD and 25 volunteers without respiratory problems were compared. All COPD subjects underwent a comprehensive examination to determine COPD severity, prognostic scales, and 6MWT. Body composition, basic spirometric parameters, and hand grip strength and endurance were determined in all study participants. RESULTS: Patients in the COPD group had a 15% decrease in maximum strength (P=0.012) and a 28% decrease in area under the force/time curve (AUC) of the endurance test (P<0.001) compared to the control group. Dynamometric parameters were significantly negatively associated with the stage of disease and values of multivariable prediction indexes, and positively associated with the results of 6MWT. In most cases, closer associations were found with AUC than with 6MWT and in the gender-specific groups. CONCLUSION: Both hand grip strength and endurance are impaired in COPD patients in comparison with the control group. In particular, AUC could be considered as an attractive option not only to assess exercise capacity but also as a predictive marker with a better prognostic value than 6MWT in COPD patients. This is the first study to observe the dependence of hand grip endurance on combined COPD assessment.

• Klíčová slova
• BODE index, dynamometry, muscle endurance, muscle strength,
• MeSH
• chronická obstrukční plicní nemoc diagnóza   patofyziologie   MeSH
• fyzická vytrvalost * MeSH
• kosterní svaly patofyziologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• plíce patofyziologie   MeSH
• plocha pod křivkou MeSH
• prediktivní hodnota testů MeSH
• prognóza MeSH
• reprodukovatelnost výsledků MeSH
• ROC křivka MeSH
• senioři MeSH
• síla ruky * MeSH
• složení těla MeSH
• spirometrie MeSH
• studie případů a kontrol MeSH
• stupeň závažnosti nemoci MeSH
• test chůzí * MeSH
• tolerance zátěže * MeSH
• zdravotní stav MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• pozorovací studie MeSH

INTRODUCTION: Recently, the Global Initiative for Chronic Obstructive Lung Disease (GOLD) has published an update on the Global Strategy for Prevention, Diagnosis and Management of COPD, introducing a new classification of chronic obstructive pulmonary disease (COPD). Our aim was to assess the prognostic value of the new GOLD classification system in comparison with the previous GOLD classification systems (GOLD stages I-IV and GOLD groups A-D) and the BODE index. METHODS: We used the data of 784 patients with COPD from the Czech Multicenter Research Database of COPD. Patient survival was analyzed with the use of Kaplan-Meier estimate and Cox model of proportional risks. ROC analysis and area under curve (AUC) were used for comparison of GOLD classifications and BODE index. The analyses were performed with the use of software R (version 4.2.0). RESULTS: We analyzed data of 782 patients with complete data on GOLD classifications. The study population comprised 72.9% of men, 89.1% current or former smokers, with a mean age of 66.6 years, a mean BMI of 27.4 and a mean FEV1 44.9% of predicted. Probability of 5-year survival differed by GOLD classification. Application of the 2023 GOLD classification showed increased risk of death in group B (HR 1.82, 95% CI 1.14-2.92; p = 0.013) and in group E (HR 2.48, 95% CI 1.54-3.99; p˂0.001). The ROC analysis showed that the overall prognostic value of the 2023 GOLD classification was similarly weak to previous A-D GOLD classification schemes (AUCs 0.557-0.576) and was lower compared to the GOLD 1-4 system (AUC 0.614) and even lower when compared to the BODE index (AUC 0.715). CONCLUSION: We concluded that the new GOLD classification system has poor prognostic properties and that specific prediction tools (eg, the BODE index) should be used for mortality risk assessment.

• Klíčová slova
• COPD, GOLD classification, mortality, prognosis,
• MeSH
• chronická obstrukční plicní nemoc * diagnóza   terapie   MeSH
• hodnocení rizik MeSH
• lidé MeSH
• prognóza MeSH
• progrese nemoci MeSH
• proporcionální rizikové modely MeSH
• senioři MeSH
• stupeň závažnosti nemoci MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

BACKGROUND: Primary ciliary dyskinesia (PCD) represents a group of rare hereditary disorders characterised by deficient ciliary airway clearance that can be associated with laterality defects. We aimed to describe the underlying gene defects, geographical differences in genotypes and their relationship to diagnostic findings and clinical phenotypes. METHODS: Genetic variants and clinical findings (age, sex, body mass index, laterality defects, forced expiratory volume in 1 s (FEV1)) were collected from 19 countries using the European Reference Network's ERN-LUNG international PCD Registry. Genetic data were evaluated according to American College of Medical Genetics and Genomics guidelines. We assessed regional distribution of implicated genes and genetic variants as well as genotype correlations with laterality defects and FEV1. RESULTS: The study included 1236 individuals carrying 908 distinct pathogenic DNA variants in 46 PCD genes. We found considerable variation in the distribution of PCD genotypes across countries due to the presence of distinct founder variants. The prevalence of PCD genotypes associated with pathognomonic ultrastructural defects (mean 72%, range 47-100%) and laterality defects (mean 42%, range 28-69%) varied widely among countries. The prevalence of laterality defects was significantly lower in PCD individuals without pathognomonic ciliary ultrastructure defects (18%). The PCD cohort had a reduced median FEV1 z-score (-1.66). Median FEV1 z-scores were significantly lower in CCNO (-3.26), CCDC39 (-2.49) and CCDC40 (-2.96) variant groups, while the FEV1 z-score reductions were significantly milder in DNAH11 (-0.83) and ODAD1 (-0.85) variant groups compared to the whole PCD cohort. CONCLUSION: This unprecedented multinational dataset of DNA variants and information on their distribution across countries facilitates interpretation of the genetic epidemiology of PCD and indicates that the genetic variant can predict diagnostic and phenotypic features such as the course of lung function.

• MeSH
• axonemální dyneiny genetika   MeSH
• cytoskeletální proteiny MeSH
• dítě MeSH
• dospělí MeSH
• fenotyp * MeSH
• genetická variace MeSH
• genetické asociační studie * MeSH
• genotyp * MeSH
• Kartagenerův syndrom genetika   patofyziologie   MeSH
• kojenec MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mutace MeSH
• předškolní dítě MeSH
• proteiny MeSH
• registrace MeSH
• senioři MeSH
• usilovný výdechový objem MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• kojenec MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa MeSH
• Názvy látek
• axonemální dyneiny MeSH
• CCDC39 protein, human MeSH Prohlížeč
• CCDC40 protein, human MeSH Prohlížeč
• cytoskeletální proteiny MeSH
• DNAH11 protein, human MeSH Prohlížeč
• proteiny MeSH

This position paper has been drafted by experts from the Czech national board of diseases with bronchial obstruction, of the Czech Pneumological and Phthisiological Society. The statements and recommendations are based on both the results of randomized controlled trials and data from cross-sectional and prospective real-life studies to ensure they are as close as possible to the context of daily clinical practice and the current health care system of the Czech Republic. Chronic Obstructive Pulmonary Disease (COPD) is a preventable and treatable heterogeneous syndrome with a number of pulmonary and extrapulmonary clinical features and concomitant chronic diseases. The disease is associated with significant mortality, morbidity and reduced quality of life. The main characteristics include persistent respiratory symptoms and only partially reversible airflow obstruction developing due to an abnormal inflammatory response of the lungs to noxious particles and gases. Oxidative stress, protease-antiprotease imbalance and increased numbers of pro-inflammatory cells (mainly neutrophils) are the main drivers of primarily non-infectious inflammation in COPD. Besides smoking, household air pollution, occupational exposure, low birth weight, frequent respiratory infections during childhood and also genetic factors are important risk factors of COPD development. Progressive airflow limitation and airway remodelling leads to air trapping, static and dynamic hyperinflation, gas exchange abnormalities and decreased exercise capacity. Various features of the disease are expressed unequally in individual patients, resulting in various types of disease presentation, emerging as the "clinical phenotypes" (for specific clinical characteristics) and "treatable traits" (for treatable characteristics) concept. The estimated prevalence of COPD in Czechia is around 6.7% with 3,200-3,500 deaths reported annually. The elementary requirements for diagnosis of COPD are spirometric confirmation of post-bronchodilator airflow obstruction (post-BD FEV1/VCmax <70%) and respiratory symptoms assessement (dyspnoea, exercise limitation, cough and/or sputum production. In order to establish definite COPD diagnosis, a five-step evaluation should be performed, including: 1/ inhalation risk assessment, 2/ symptoms evaluation, 3/ lung function tests, 4/ laboratory tests and 5/ imaging. At the same time, all alternative diagnoses should be excluded. For disease classification, this position paper uses both GOLD stages (1 to 4), GOLD groups (A to D) and evaluation of clinical phenotype(s). Prognosis assessment should be done in each patient. For this purpose, we recommend the use of the BODE or the CADOT index. Six elementary clinical phenotypes are recognized, including chronic bronchitis, frequent exacerbator, emphysematous, asthma/COPD overlap (ACO), bronchiectases with COPD overlap (BCO) and pulmonary cachexia. In our concept, all of these clinical phenotypes are also considered independent treatable traits. For each treatable trait, specific pharmacological and non-pharmacological therapies are defined in this document. The coincidence of two or more clinical phenotypes (i.e., treatable traits) may occur in a single individual, giving the opportunity of fully individualized, phenotype-specific treatment. Treatment of COPD should reflect the complexity and heterogeneity of the disease and be tailored to individual patients. Major goals of COPD treatment are symptom reduction and decreased exacerbation risk. Treatment strategy is divided into five strata: risk elimination, basic treatment, phenotype-specific treatment, treatment of respiratory failure and palliative care, and treatment of comorbidities. Risk elimination includes interventions against tobacco smoking and environmental/occupational exposures. Basic treatment is based on bronchodilator therapy, pulmonary rehabilitation, vaccination, care for appropriate nutrition, inhalation training, education and psychosocial support. Adequate phenotype-specific treatment varies phenotype by phenotype, including more than ten different pharmacological and non-pharmacological strategies. If more than one clinical phenotype is present, treatment strategy should follow the expression of each phenotypic label separately. In such patients, multicomponental therapeutic regimens are needed, resulting in fully individualized care. In the future, stronger measures against smoking, improvements in occupational and environmental health, early diagnosis strategies, as well as biomarker identification for patients responsive to specific treatments are warranted. New classes of treatment (inhaled PDE3/4 inhibitors, single molecule dual bronchodilators, anti-inflammatory drugs, gene editing molecules or new bronchoscopic procedures) are expected to enter the clinical practice in a very few years.

• Klíčová slova
• COPD; position paper; clinical phenotypes; treatable traits; bronchodilators; individualized care; personalized medicine,
• MeSH
• bronchodilatancia normy   terapeutické užití   MeSH
• chronická nemoc terapie   MeSH
• chronická obstrukční plicní nemoc diagnóza   genetika   patofyziologie   terapie   MeSH
• dospělí MeSH
• fenotyp * MeSH
• lidé středního věku MeSH
• lidé MeSH
• péče orientovaná na pacienta normy   MeSH
• pneumologie normy   MeSH
• prospektivní studie MeSH
• průřezové studie MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• směrnice pro lékařskou praxi jako téma * MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH
• Názvy látek
• bronchodilatancia MeSH