BACKGROUND: The number of older people with cognitive impairment is increasing worldwide. Impaired lung function might be associated with cognitive decline in older age; however, results from large longitudinal studies are lacking. In this study, we examined the longitudinal associations between pulmonary function and the trajectories of cognitive decline using prospective population-based SHARE data from 14 countries. METHODS: The analytic sample included N = 32,049 older adults (Mean age at baseline = 64.76 years). The dependent variable was cognitive performance, measured repeatedly across six waves in three domains: verbal fluency, memory, and numeracy. The main predictor of interest was peak expiratory flow (PEF). The data were analyzed in a multilevel accelerated longitudinal design, with models adjusted for a variety of covariates. RESULTS: A lower PEF score was associated with lower cognitive performance for each domain as well as a lower global cognitive score. These associations remained statistically significant after adjusting for all covariates Q4 vs Q1 verbal fluency: unstandardized coefficient B = -3.15; numeracy: B = -0.52; memory: B = -0.64; global cognitive score B = -2.65, all p < .001). However, the PEF score was not found to be associated with the rate of decline for either of the cognitive outcomes. CONCLUSIONS: In this large multi-national longitudinal study, the PEF score was independently associated with lower levels of cognitive functions, but it did not predict a future decline. The results suggest that pre-existing differences in lung functions are responsible for variability in cognitive functions and that these differences remained stable across aging.

• MeSH
• kognice MeSH
• kognitivní dysfunkce * epidemiologie   MeSH
• lidé MeSH
• longitudinální studie MeSH
• plíce MeSH
• prospektivní studie MeSH
• senioři MeSH
• stárnutí * psychologie   MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• Publikační typ
• časopisecké články MeSH

Chronický zánět dýchacích cest zahrnující infiltraci stěny průdušek aktivovanými eozinofilními leukocyty, žírnými buňkami a T lymfocyty je považován za základní rys bronchiálního astmatu. Remodelace dýchacích cest postihující všechny strukturální složky bronchů je s velkou pravděpodobností paralelním procesem patrným i u pacientů s nově diagnostikovaným bronchiálním astmatem. Možnost ovlivnění remodelace dýchacích cest je v současné době dostupnou protizánětlivou léčbou jen velmi omezená. Pravděpodobně z tohoto důvodu je v průběhu několikaletého sledování pacientů patrná zvýšená rychlost poklesu ventilačních parametrů. V naší retrospektivní studii jsme hodnotili 48 pacientů s perzistujícím bronchiálním astmatem, kteří měli na počátku sledování provedenu indukci sputa s vyšetřením rozpočtu buněčných populací. Průměrná doba sledování byla 5,5 let. Rychlost poklesu FEV1 byla pro celou skupinu 62 ml/rok, přičemž jevila výraznou inter-individuální variabilitu. Neprokázali jsme jakoukoliv její souvislost s úvodním zastoupením eozinofilních či neutrofilních leukocytů v indukovaném sputu. Rychlost poklesu signifikantně pozitivně korelovala s dávkou inhalačních, resp. systémových kortikosteroidů (p<0,05) a byla významně vyšší ve skupině kuřáků a bývalých kuřáků ve srovnání s nekuřáky (p < 0,05).

Airways inflammation, involving infiltration of bronchial wall with activated eosinophils, mast cells and T lymphocytes, is an established feature of asthma. Airway remodelling, involving all components of the airway wall, is probably a parallel process which is seen even in patient with newly diagnosed asthma. Currently available antiinflammatory treatment can hardly influence remodelling of the airway wall. This is presumably the reason for increased rate of decline in pulmonary function tests over several years seen in patients with bronchial asthma. In this retrospective study 48 patients with persistent bronchial asthma, in whom sputum cell count analysis were available at the start of follow-up, were evaluated. Mean duration of follow-up was 5,5 years. The rate of decline of FEV1 was 62 ml/year for the whole group with high inter-individual variability. We were not able to show any relationship between the rate of decline of FEV1 and sputum eosinophils or neutrophils. The rate of decline of FEV1 was positively correlated to the daily dose of inhaled or oral steroid (p<0,05) and was significantly higher in subgroup of current and ex-smokers compared to never-smokers (p<0,05).

• MeSH
• bronchiální astma diagnóza   patologie   MeSH
• bronchiální hyperreaktivita diagnóza   patologie   MeSH
• diagnostické techniky dýchacího ústrojí metody   MeSH
• kouření MeSH
• lidé MeSH
• respirační funkční testy metody   MeSH
• retrospektivní studie MeSH
• Check Tag
• lidé MeSH

The association between oxidative stress and neutrophilic inflammation in cystic fibrosis (CF) lung disease is well recognized. 8-Isoprostane is a product of non-enzymatic oxidation of arachidonic acid. The aim of the present study was to examine the relationship between lung function decline and 8-isoprostane concentrations in exhaled breath condensate (EBC) in CF patients with Burkholderia cenocepacia airway colonization. Concentrations of 8-isoprostane in EBC were measured in 24 stable CF patients with B. cenocepacia airway colonization. The median (interquartile range) age of the cohort was 23.9 (22.0; 26.6) years. All patients underwent clinical examinations and pulmonary function tests at the time of EBC collection and in 1-, 3-, and 5-year intervals. 8-Isoprostane concentrations in EBC correlated to 1- and 3-year declines of forced expiratory volume in 1 s (FEV1) with r(S) values of -0.511 (p = 0.0011) and -0.495 (p = 0.016), respectively. In multiple regression analysis, 8-isoprostane concentrations in EBC were the only independent predictor for 1-year FEV1 decline (p = 0.01). When the median value of 8-isoprostane concentration in EBC (10.0 pg/mL) was used as a cutoff, subgroups of patients with lower and higher level of oxidative stress had significantly different median (interquartile range) FEV1 declines in 1-year interval, -2.4% (-5.3; 0.8) and -7.3% (-10.3; -5.8) predicted (p = 0.009). In conclusion, 8-isoprostane concentrations in EBC correlated to short-term lung function decline in CF patients with B. cenocepacia airway colonization. This correlation reflects the role of oxidative stress in CF lung pathogenesis and contributes to prediction of prognosis in these patients.

• MeSH
• Burkholderia cenocepacia * MeSH
• časové faktory MeSH
• cystická fibróza komplikace   metabolismus   patofyziologie   MeSH
• dinoprost analogy a deriváty   metabolismus   MeSH
• dospělí MeSH
• infekce bakteriemi rodu Burkholderia komplikace   mikrobiologie   MeSH
• lidé MeSH
• mladý dospělý MeSH
• oportunní infekce * MeSH
• oxidační stres * MeSH
• respirační funkční testy MeSH
• usilovný výdechový objem MeSH
• vydechnutí MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

INTRODUCTION: Pirfenidone, an antifibrotic drug, slows-down the disease progression in idiopathic pulmonary fibrosis (IPF) over 12 months, however limited data on the decline of lung function and overall survival (OS) in real-world cohorts on longer follow-up exists. PATIENTS/METHODS: Of the enrolled Czech IPF patients (n = 841) from an EMPIRE registry, 383 (45.5%) received pirfenidone, 218 (25.9%) no-antifibrotic treatment and 240 (28.5%) were excluded (missing data, nintedanib treatment). The 2- and 5-yrs OS and forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO) were investigated at treatment initiation and 6, 12, 18 and 24 months' follow-up. RESULTS: During a 2-yr follow-up, less than a quarter of the patients progressed on pirfenidone as assessed by the decline of ≥10% FVC (17.0%) and ≥ 15% DLCO (14.3%). On pirfenidone, the DLCO (≥10%) declines at 6, 12, 18 and 24 months' and DLCO (≥15%) declines at 6, 18 and 24 months' follow-up were associated with increased mortality. The DLCO decline showed higher predictive value for mortality than FVC decline. In patients with no-antifibrotics, FVC and DLCO declines were not predictive for mortality. Pirfenidone increased 5-yrs OS over no-antifibrotic treatment (55.9% vs 31.5% alive, P = 0.002). CONCLUSION: Our study observed the 2-yrs sustained effect of pirfenidone on the decline of lung function and survival in the real-world patient's IPF cohort. DLCO decline of ≥10% shows a potential as a mortality predictor in IPF patients on pirfenidone, and should be routinely evaluated during follow-up examinations.

• MeSH
• antiflogistika nesteroidní farmakologie   terapeutické užití   MeSH
• idiopatická plicní fibróza diagnóza   farmakoterapie   epidemiologie   MeSH
• kohortové studie MeSH
• lidé středního věku MeSH
• lidé MeSH
• míra přežití trendy   MeSH
• následné studie MeSH
• progrese nemoci * MeSH
• pyridony farmakologie   terapeutické užití   MeSH
• registrace * MeSH
• respirační funkční testy trendy   MeSH
• senioři MeSH
• vitální kapacita účinky léků   fyziologie   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH

We studied lung function in 41 patients, aged 6-27 years, 1-5 years after intracardiac surgical repair (ICR) of tetralogy of Fallot (TOF) and about 5 years after the establishment of the Pediatric Cardiac Center in Prague. The measurements included vital capacity (VC), total lung capacity (TLC), functional residual capacity (FRC), residual volume (RV), forced expiratory flows (FEF), specific airway conductance (SGaw), lung recoil pressure (Pst), and specific static lung compliance (SC1st). Single or multiple abnormal lung function parameters were found in 83% of patients. Lung function was not related to shunting operations prior to ICR, hemoglobin concentration, and hematocrit, and had no specific pattern. Pst at 100% TLC and 90% TLC declined with increasing age at ICR and at lung function testing, while SC1st rose, as did the ratio FRC/TLC. Fifteen patients were studied before and after surgery. Single or multiple lung function tests were abnormal in 93% before and in 84% after ICR. After ICR the ratios FRC/TLC and also RV/TLC, FEF at 25% VC, and FEF at 60% TLC were less frequently abnormal, while Pst at 100% TLC and at 90% TLC, as well as SGaw and TLC, were more frequently abnormal after ICR. The results indicated a regression of smaller airway obstruction and lung hyperinflation after ICR. The evolution of abnormally compliant (emphysematous) lungs with growth of the patients might be a sign of permanent sequelae of early lung damage from abnormal pulmonary hemodynamics.

• MeSH
• časové faktory MeSH
• dítě MeSH
• dospělí MeSH
• Fallotova tetralogie * chirurgie   MeSH
• hematokrit MeSH
• hemoglobiny MeSH
• incidence MeSH
• lidé MeSH
• mladiství MeSH
• plíce * patofyziologie   MeSH
• pooperační období MeSH
• poruchy dýchání epidemiologie   krev   patofyziologie   MeSH
• předoperační péče MeSH
• respirační funkční testy * MeSH
• věkové faktory MeSH
• vitální kapacita MeSH
• vrcholová výdechová rychlost MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH

BACKGROUND: There is no clear evidence whether pirfenidone has a benefit in patients with probable or possible UIP, i.e. when idiopathic pulmonary fibrosis (IPF) is diagnosed with a lower degree of diagnostic certainty. We report on outcomes of treatment with pirfenidone in IPF patients diagnosed with various degrees of certainty. METHODS AND FINDINGS: We followed patients in the multi-national European MultiPartner IPF Registry (EMPIRE) first seen between 2015 and 2018. Patients were assessed with HRCT, histopathology and received a multi-disciplinary team (MDT) IPF diagnosis. Endpoints of interest were overall survival (OS), progression-free survival (PFS) and lung function decline. RESULTS: A total of 1626 patients were analysed, treated with either pirfenidone (N = 808) or receiving no antifibrotic treatment (N = 818). When patients treated with pirfenidone were compared to patients not receiving antifibrotic treatment, OS (one-, two- and three-year probability of survival 0.871 vs 0.798; 0.728 vs 0.632; 0.579 vs 0.556, P = 0.002), and PFS (one-, two- and three-year probability of survival 0.597 vs 0.536; 0.309 vs 0.281; 0.158 vs 0.148, P = 0.043) was higher, and FVC decline smaller (-0.073 l/yr vs -0.169 l/yr, P = 0.017). The benefit of pirfenidone on OS and PFS was also seen in patients with probable or possible IPF. CONCLUSIONS: This EMPIRE analysis confirms the favourable outcomes observed for pirfenidone treatment in patients with definitive IPF and indicates benefits also for patients with probable or possible IPF.

• MeSH
• antiflogistika nesteroidní farmakologie   MeSH
• idiopatická plicní fibróza * diagnóza   MeSH
• lidé MeSH
• plíce MeSH
• pravděpodobnost MeSH
• pyridony farmakologie   MeSH
• retrospektivní studie MeSH
• vitální kapacita MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem 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

Serum or plasma creatinine level determination has been serving for decades as the most commonly used marker of renal function. However, creatinine levels are affected by a number of other factors not related to renal function, particularly age, gender, muscle mass, starvation, wasting diseases, post surgical states, and activity or exertion. Alternatives for the assessment of renal function or glomerular fi ltration rate (GFR), through measurement of the clearance of exogenous substances like, inulin, 51Cr-ethylendiaminotetraacetate (51Cr-EDTA), 99mTc-diethylenetriaminepentaacetic acid or iohexol are more accurate, but too complex, laborious, and not suitable for routine clinical use. Therefore, serum creatinine measurement yet remains the most common method to estimate renal function in routine clinical practice. In the presented case study, nearly 50% decline in serum creatinine level as measured by enzymatic colorimetry method according to the manufacture’s recommendation is described. Relatively rapid serum creatinine concentration decline from 67 µmol/L baseline value to 30 µmol/L within 4 days in a CF patient post bilateral lung transplantation, who, was on courses of essential immunosuppressive, antimycotic, and antibacterial drugs including an aminoglycoside (amikacin) was observed. The change in serum creatinine level within four consecutive days after bilateral lung transplantation (mean difference, -49.25 % µmol/L; with 95% confi dence interval, -29 to -37 µmol/L) was signifi cant. Herein, the event is not essentially refl ecting better renal function, since amikacin trough level was unacceptably high (20 mg/L) at the same time, rather explaining probably low production of creatinine as a result of underlying pathology in cystic fi brosis. Patient related explanations and existing methodological challenges have also been discussed.

Hladina sérového nebo plazmatického kreatininu slouží již desítky let jako nejčastější používaný ukazatel renální funkce. Přesto je koncentrace sérového kreatininu ovlivněna jinými, s funkcí ledvin nesouvisejícími faktory (věk, pohlaví, svalová hmota, stav výživy, nemoci poškozující organismus, pooperační stavy, tělesná aktivita nebo vyčerpávající fyzický výkon). Alternativy pro zjištění renální funkce/gromerulární fi ltrace (GFR) pomocí podání exogenních látek (inulin, 51Cr-ethylendiaminotetraacetát (51Cr-EDTA), 99mTc-diethylenetriaminepentaacetát nebo iohexol) jsou přesnější, ale zároveň velmi složité, pracné a nehodí se pro rutinní praxi. Měření sérového kreatininu proto stále zůstává běžnou metodou k odhadu renální funkce v rutinní klinické praxi. V tomto sdělení popisujeme cca 50% pokles sérového kreatininu měřeného enzymatickou metodou s kolorimetrickou detekcí podle návodu výrobce. Relativně rychlý pokles sérového kreatinu z 67 µmol/l na 30 µmol/l v průběhu 4 dnů byl pozorován u pacientky s cystickou fi brózou po oboustranné transplantaci plic, s náležitou imunosupresivní, antimykotickou i antibakteriální léčbou včetně aminoglykosidů (amikacin). Změny v hladině sérového kreatininu během 4 dnů (průměrný rozdíl 49, 25 %, při 95% intervalu spolehlivosti, -29 až -37 µmol/l) jsou významné. Tento jev přesto nemusel být odrazem lepší clearance vzhledem k nepřijatelně vysoké „trough“ hladině amikacinu a nejspíše poukazuje na možné snížení produkce kreatininu v souvislosti s cystickou fi brózou. Autoři v článku diskutují o stavu nemocné a probírají metodologická úskalí.

• MeSH
• amikacin aplikace a dávkování   metabolismus   MeSH
• cystická fibróza MeSH
• hodnoty glomerulární filtrace účinky léků   MeSH
• kreatinin krev   MeSH
• lidé MeSH
• pooperační období MeSH
• prediktivní hodnota testů MeSH
• transplantace plic MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

• MeSH
• kouření MeSH
• lidé MeSH
• mortalita MeSH
• návykové chování MeSH
• nemoci dýchací soustavy diagnóza   MeSH
• přežívající MeSH
• výměna plynů v plicích MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• srovnávací studie MeSH

Idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis (HP) and sarcoidosis belong to interstitial lung diseases (ILD) where an imbalance of regulatory, profibrotic and antifibrotic cytokines is hypothesized. The relationship of bronchoalveolar lavage (BAL) fluid (BALF) cytokines, BALF cell profile and ILD course is supposed. The aim of our study was to correlate BALF cytokine and chemokine levels with BALF cellular characteristics and lung function parameters in different ILD. Twenty-two sarcoidosis, seven IPF and 11 HP patients underwent lung function tests and BAL. The BALF differential cell counts and superficial cell markers were characterized, and MCP-1, MIP-1alpha, MIP-1beta, RANTES, epithelial neutrophil-activating protein (ENA)-78, FGF, G-CSF, GM-CSF, IFN-gamma, interleukin (IL)-1alpha, IL-1RA, IL-1beta, -2beta, -4beta, -5beta, -6beta, -8beta, -10beta, -17beta, tumour necrosis factor (TNF)-alpha, thromobopoietin (Tpo) and vascular endothelial growth factor (VEGF) values measured. The BALF VEGF values were highest in sarcoidosis (P = 0.0526). IL-1RA values were higher in IPF and HP compared with sarcoidosis (P = 0.0334). IL-8/ENA-78 ratio positively correlated with BALF neutrophil counts in IPF (r = 0.89, P = 0.04). Vital capacity and TL(CO) values positively correlated with VEGF and negatively with IL-8 BALF levels in all ILDs but the correlations were most significant in sarcoidosis group. We suppose that VEGF plays a role in ILDs' early phases and has rather angiogenic than profibrotic effect. On the contrary, IL-8 is probably upregulated in advanced ILDs with prominent fibrosis and marked lung functions decline. We state that BALF VEGF, IL-8 and ENA-78 levels and IL-8/ENA-78 ratio could become useful markers of ILDs' phase, activity and prognosis. They might also be helpful in treatment modality choice.

• MeSH
• bronchoalveolární laváž MeSH
• bronchoalveolární lavážní tekutina cytologie   imunologie   MeSH
• cytokiny metabolismus   MeSH
• financování organizované MeSH
• intersticiální plicní nemoci imunologie   patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• počet buněk MeSH
• respirační funkční testy MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH