• MeSH
• laryngální masky klasifikace   MeSH
• lidé MeSH
• novorozenec MeSH
• plicní surfaktanty * aplikace a dávkování   farmakologie   klasifikace   terapeutické užití   MeSH
• plicní ventilace MeSH
• syndrom dechové tísně diagnóza   farmakoterapie   ošetřování   MeSH
• syndrom respirační tísně novorozenců * diagnóza   farmakoterapie   ošetřování   MeSH
• Check Tag
• lidé MeSH
• novorozenec MeSH
• Publikační typ
• přehledy MeSH

• MeSH
• infekce respiračními syncytiálními viry * diagnóza   epidemiologie   farmakoterapie   MeSH
• kazuistiky jako téma MeSH
• lidé MeSH
• novorozenci extrémně nezralí * MeSH
• novorozenec MeSH
• palivizumab farmakologie   terapeutické užití   MeSH
• plicní ventilace MeSH
• preexpoziční profylaxe klasifikace   metody   MeSH
• ribavirin farmakokinetika   terapeutické užití   MeSH
• Check Tag
• lidé MeSH
• novorozenec MeSH
• ženské pohlaví MeSH
• Publikační typ
• přehledy MeSH

BACKGROUND: Factors associated with severe COVID-19 infection have been identified; however, the impact of infection on longer-term outcomes is unclear. The objective of this study was to examine the impact of COVID-19 infection on the trajectory of lung function and nutritional status in people with cystic fibrosis (pwCF). METHODS: This is a retrospective global cohort study of pwCF who had confirmed COVID-19 infection diagnosed between January 1, 2020 and December 31, 2021. Forced expiratory volume in one second percent predicted (ppFEV1) and body mass index (BMI) twelve months prior to and following a diagnosis of COVID-19 were recorded. Change in mean ppFEV1 and BMI were compared using a t-test. A linear mixed-effects model was used to estimate change over time and to compare the rate of change before and after infection. RESULTS: A total of 6,500 cases of COVID-19 in pwCF from 33 countries were included for analysis. The mean difference in ppFEV1 pre- and post-infection was 1.4 %, (95 % CI 1.1, 1.7). In those not on modulators, the difference in rate of change pre- and post-infection was 1.34 %, (95 % CI -0.88, 3.56) per year (p = 0.24) and -0.74 % (-1.89, 0.41) per year (p = 0.21) for those on elexacaftor/tezacaftor/ivacaftor. No clinically significant change was noted in BMI or BMI percentile before and after COVID-19 infection. CONCLUSIONS: No clinically meaningful impact on lung function and BMI trajectory in the year following infection with COVID-19 was identified. This work highlights the ability of the global CF community to unify and address critical issues facing pwCF.

• MeSH
• COVID-19 * patofyziologie   komplikace   epidemiologie   MeSH
• cystická fibróza * patofyziologie   komplikace   MeSH
• dospělí MeSH
• index tělesné hmotnosti MeSH
• lidé MeSH
• nutriční stav * MeSH
• respirační funkční testy metody   MeSH
• retrospektivní studie MeSH
• SARS-CoV-2 MeSH
• usilovný výdechový objem MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články 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

Profesionálna astma je ochorenie, ktoré je charakterizované zápalom dýchacích ciest, rôznym obmedzením prietoku vzduchu a hyperreaktivitou dýchacích ciest, ktoré vzniká z príčin a podmienok, ktoré možno pripísať pracovnému prostrediu, ale nie stimulom, vyskytujúcim sa mimo pracovného prostredia. Profesionálna astma je často nedostatočne diagnostikovaná a počet pacientov s hláseným ochorením je nižší, ako je skutočná prevalencia. Diagnostický prístup profesionálnej astmy preto musí byť komplexný. V prekladanej práci je uvedená kazuistika pacientky s priznanou profesionálnou astmou, u ktorej bolo nutné pre definitívne potvrdenie ochorenia realizovať širšie spektrum objektívnych metód. Pacientka (58 rokov), fajčiarka, pracovala 12 rokov v pekárenskej výrobe. Respiračné ťažkosti v zmysle dyspnoe, neproduktívneho kašľa a intermitentného pískania na hrudníku sa rozvinuli po šiestich rokoch od nástupu do práce. Pacientka na Kliniku pracovného lekárstva a klinickej toxikológie (KPL a KT) v Košiciach bola odoslaná imunoalergológom ako syndróm dráždivého kašľa v diferenciálnej diagnóze bez potvrdenia inhalačnej precitlivenosti. Pred hospitalizáciou na klinike bola práce neschopná 2 mesiace. Realizované vyšetrenia v priebehu hospitalizácie (negatívny auskultačný nález, spirometrické vyšetrenie bez jednoznačnej obštrukčnej ventilačnej poruchy, bronchoprovokačný test metacholínom, bronchodilatačný test salbutamolom, 24-hodinové monitorovanie vrcholového výdychového prietoku PEF) astmu nepotvrdili. Špecifické IgE protilátky preukázali hraničnú senzibilizáciu na ražnú múku. Následne bola pacientka zaradená na pôvodné pracovisko za účelom vykonania reexpozičného testu. Po 2 mesiacoch opätovne vyšetrená na ambulancii KPL a KT, kde uvádzala zvýraznenie respiračných symptómov, auskultačne dýchanie vezikulárne bez vedľajších dýchacích fenoménov s predĺženým exspíriom. Pri spirometrickom vyšetrení boli dynamické pľúcne parametre na dolnej hranici normy, bronchodilatačný test salbutamolom bol pozitívny. Hodnoty PEF v porovnaní s hospitalizáciou boli o 40 % nižšie. Vzhľadom na anamnézu symptómov zhoršenia stavu v pracovnom prostredí, pozitívny bronchodilatačný test, pokles hodnôt PEF o 40 % a pozitivitu špecifických IgE protilátok bola priznaná profesionálna astma s precitlivenosťou na ražnú múku. Kazuistika poukázala nevyhnutnosť realizácie viacerých diagnostických metód nie len na potvrdenie profesionality ochorenia ale aj samotné diagnostikovanie astmy.

Occupational asthma is a disease that is characterized by inflammation of the airways, varying airflow limitation and hyperreactivity of the airways, which arises from causes and conditions attributable to the work environment, but not to stimuli occurring outside the work environment. Occupational asthma is often underdiagnosed and the number of patients with the reported disease is lower than the actual prevalence. The diagnostic approach to occupational asthma must therefore be comprehensive. The translated work presents a case report of a patient with admitted occupational asthma, in whom it was necessary to implement a wider spectrum of objective methods for the definitive confirmation of the disease. The 58-year-old patient, a smoker, worked for 12 years in the bakery industry. Respiratory difficulties in terms of dyspnea, non-productive cough and intermittent wheezing in the chest developed six years after starting work. The patient was sent to the Department of Occupational Medicine and Clinical Toxicology (KPL and KT) in Košice by an immunoallergist as irritable cough syndrome in the differential diagnosis without confirmation of inhalant hypersensitivity. Before hospitalization at the department, she was at sick leave for 2 months. The examinations performed during the hospitalization (negative auscultation findings, spirometric examination without clear obstructive ventilation disorder, bronchoprovocation test with methacholine, bronchodilation test with salbutamol, 24-hour monitoring of PEF peak expiratory flow) did not confirm asthma. Specific IgE antibodies showed borderline sensitization to rye flour. Subsequently, the patient was assigned to the original workplace for the purpose of performing a re-exposure test. After 2 months, she was re-examined at the KPL and KT clinic, where she reported increased respiratory symptoms, and vesicular breathing on auscultation without secondary respiratory phenomena with prolonged expiration. During the spirometric examination, the dynamic lung parameters were at the lower limit of the norm, the bronchodilation test with salbutamol was positive. PEF values compared to hospitalization were 40% lower. Due to the anamnesis of worsening symptoms in the work environment, a positive bronchodilation test, a decrease in PEF values by 40% and the positivity of specific IgE antibodies, occupational asthma with hypersensitivity to rye flour was diagnosed. The case study pointed out the necessity of implementing several diagnostic methods not only to confirm the occupational origin of the disease but also to diagnose asthma itself.

• MeSH
• bronchoprovokační testy metody   MeSH
• diagnostické techniky dýchacího ústrojí klasifikace   MeSH
• diferenciální diagnóza MeSH
• lidé středního věku MeSH
• lidé MeSH
• nemoci z povolání diagnóza   etiologie   klasifikace   MeSH
• profesionální astma * diagnostické zobrazování   diagnóza   etiologie   MeSH
• respirační alergie diagnóza   etiologie   MeSH
• vrcholová exspirační průtoková rychlost MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

BACKGROUND: Inhaled corticosteroids have been widely reported as a preventive measure against the development of severe forms of COVID-19 not only in patients with asthma. METHODS: In 654 Czech and Slovak patients with asthma who developed COVID-19, we investigated whether the correct use of inhaler containing corticosteroids was associated with a less severe course of COVID-19 and whether this had an impact on the need for hospitalisation, measurable lung functions and quality of life (QoL). RESULTS: Of the studied cohort 51.4% had moderate persistent, 29.9% mild persistent and 7.2% severe persistent asthma. We found a significant adverse effect of poor inhaler adherence on COVID-19 severity (p=0.049). We also observed a lower hospitalisation rate in patients adequately taking the inhaler with OR of 0.83. Vital capacity and forced expiratory lung volume deterioration caused by COVID-19 were significantly reversed, by approximately twofold to threefold, in individuals who inhaled correctly. CONCLUSION: Higher quality of inhalation technique of corticosteroids measured by adherence to an inhaled medication application technique (A-AppIT) score had a significant positive effect on reversal of the vital capacity and forced expiratory lung volume in 1 s worsening (p=0.027 and p<0.0001, respectively) due to COVID-19. Scoring higher in the A-AppIT was also associated with significantly improved QoL. All measured variables concordantly and without exception showed a positive improvement in response to better adherence. We suggest that corticosteroids provide protection against the worsening of lungs in patients with COVID-19 and that correct and easily assessable adherence to corticosteroids with appropriate inhalation technique play an important role in preventing severe form of COVID-19.

• MeSH
• bronchiální astma * farmakoterapie   MeSH
• COVID-19 * MeSH
• hormony kůry nadledvin MeSH
• kvalita života MeSH
• lidé MeSH
• usilovný výdechový objem MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

PURPOSE: The Phenotypes of COPD in Central and Eastern Europe (POPE) study assessed the prevalence and clinical characteristics of four clinical COPD phenotypes, but not mortality. This retrospective analysis of the POPE study (RETRO-POPE) investigated the relationship between all-cause mortality and patient characteristics using two grouping methods: clinical phenotyping (as in POPE) and Burgel clustering, to better identify high-risk patients. PATIENTS AND METHODS: The two largest POPE study patient cohorts (Czech Republic and Serbia) were categorized into one of four clinical phenotypes (acute exacerbators [with/without chronic bronchitis], non-exacerbators, asthma-COPD overlap), and one of five Burgel clusters based on comorbidities, lung function, age, body mass index (BMI) and dyspnea (very severe comorbid, very severe respiratory, moderate-to-severe respiratory, moderate-to-severe comorbid/obese, and mild respiratory). Patients were followed-up for approximately 7 years for survival status. RESULTS: Overall, 801 of 1,003 screened patients had sufficient data for analysis. Of these, 440 patients (54.9%) were alive and 361 (45.1%) had died at the end of follow-up. Analysis of survival by clinical phenotype showed no significant differences between the phenotypes (P=0.211). However, Burgel clustering demonstrated significant differences in survival between clusters (P<0.001), with patients in the "very severe comorbid" and "very severe respiratory" clusters most likely to die. Overall survival was not significantly different between Serbia and the Czech Republic after adjustment for age, BMI, comorbidities and forced expiratory volume in 1 second (hazard ratio [HR] 0.80, 95% confidence interval [CI] 0.65-0.99; P=0.036 [unadjusted]; HR 0.88, 95% CI 0.7-1.1; P=0.257 [adjusted]). The most common causes of death were respiratory-related (36.8%), followed by cardiovascular (25.2%) then neoplasm (15.2%). CONCLUSION: Patient clusters based on comorbidities, lung function, age, BMI and dyspnea were more likely to show differences in COPD mortality risk than phenotypes defined by exacerbation history and presence/absence of chronic bronchitis and/or asthmatic features.

• MeSH
• chronická bronchitida * MeSH
• chronická obstrukční plicní nemoc * epidemiologie   MeSH
• dyspnoe epidemiologie   MeSH
• fenotyp MeSH
• lidé MeSH
• progrese nemoci MeSH
• retrospektivní studie MeSH
• usilovný výdechový objem MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

Reticular basement membrane (RBM) thickening may occur in children with allergic bronchial asthma (BA), cystic fibrosis (CF), and primary ciliary dyskinesia (PCD). Its functional consequences remain unknown. We investigated the relationship between baseline RBM thickness and subsequent spirometry. In our cohort follow-up study, patients aged 3-18 yr with BA, CF, and PCD and controls underwent baseline lung clearance index (LCI) measurement, spirometry, and endobronchial biopsy sampling. Total RBM and collagen IV-positive layer thickness were measured. Trends in forced vital capacity (FVC), forced expired volume in 1 s (FEV1), and FEV1/FVC were analyzed during follow-up, and their relationship to baseline characteristics was studied using univariate analysis and multiple regression models. Complete baseline data were available in 19 patients with BA, 30 patients with CF, 25 patients with PCD, and 19 controls. The RBM was thicker in patients with BA (6.33 ± 1.22 μm), CF (5.60 ± 1.39 μm), and PCD (6.50 ± 1.87 μm) than in controls (3.29 ± 0.55 μm) (all P < 0.001). The LCI was higher in patients with CF (15.32 ± 4.58, P < 0.001) and PCD (10.97 ± 2.46, P = 0.002) than in controls (7.44 ± 0.43). The median follow-up times were 3.6, 4.8, 5.7, and 1.9 years in patients with BA, CF, PCD, and controls, respectively. The z-scores of FEV1 and FEV1/FVC deteriorated significantly in all groups except in controls. In patients with CF and PCD, trends in FEV1z-scores correlated with baseline LCI and RBM; in BA, it correlated with collagen IV. In multiple regression models, RBM morphology and ventilation inhomogeneity could predict up to 84.4% of variability in spirometry trends. In conclusion, baseline LCI value and RBM morphology may predict trends in subsequent spirometry.NEW & NOTEWORTHY This paper deals with the relationship between reticular basement membrane (RBM) morphology at baseline and follow-up spirometry in children with asthma, cystic fibrosis, and primary ciliary dyskinesia. For the first time, to our knowledge, the possibility to predict subsequent lung function development using selected baseline characteristics (reticular basement membrane morphology from endobronchial biopsy and ventilation inhomogeneity from nitrogen multiple breath washout test) is proposed. Corresponding predictive models are presented.

• MeSH
• bazální membrána patologie   MeSH
• bronchiální astma * patologie   MeSH
• cystická fibróza * patologie   MeSH
• dítě MeSH
• kolagen MeSH
• lidé MeSH
• následné studie MeSH
• plíce patologie   MeSH
• poruchy ciliární motility * MeSH
• spirometrie MeSH
• usilovný výdechový objem MeSH
• zánět patologie   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

• MeSH
• kongresy jako téma MeSH
• lidé MeSH
• následná péče metody   MeSH
• ošetřovatelská péče o pacienty v kritickém stavu * metody   MeSH
• plicní ventilace MeSH
• tracheostomie ošetřování   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• zprávy MeSH

Současné technologické možnosti umožňují automatickou regulaci frakce kyslíku v podávané ventilační směsi. Koncentrace inspirovaného kyslíku je regulována na základě aktuální informace z pulzního oxymetru implementovaného do plicního ventilátoru. Podmínkou pro toto zpětnovazební řízení je elektronicky řiditelný směšovač plynu. Měření saturace krve kyslíkem je realizováno pomocí pulzní oxymetrie technologie Masimo nebo Nellcor. V textu jsou blíže vysvětleny typy řídicích algoritmů a představeny aktuální, klinicky dostupné technologie. Dosavadní výsledky a zkušenosti se zpětnovazebními systémy poukazují na vyšší efektivitu udržování saturace krve kyslíkem v normoxemické oblasti než u skupin s manuálním nastavováním frakce kyslíku. Zpětnovazební systémy regulace kyslíku celkem dobře brání hyperoxemickým situacím než hypoxemickým. Obecně dokáží rychle řešit hypoxemické epizody, avšak nedokáží jim předejít.

Modern technology has made it possible to automatically control the oxygen levels in the ventilation mixture provided to patients. The concentration of inspired oxygen can be regulated based on real-time information from the pulse oximeter built into the lung ventilator. This feedback control is called automatic closed loop, which requires an electronically controlled gas mixer. Blood oxygen saturation is measured using Masimo or Nellcor pulse oximetry technology. The paper provides further details on the types of automatic closed loop control algorithms and the clinically available technologies. Studies have shown that automatic closed loop systems more efficiently maintain normal blood oxygen levels than manual fraction of inspired oxygen adjustment. While automatic closed loop systems can quickly resolve hypoxemic episodes, they cannot prevent them entirely.

• Klíčová slova
• frakce kyslíku, automatická regulace kyslíku,
• MeSH
• kyslík MeSH
• lidé MeSH
• novorozenec nedonošený MeSH
• novorozenec MeSH
• plicní ventilace * MeSH
• Check Tag
• lidé MeSH
• novorozenec MeSH