Pulmonary function was measured in 35 patients (mean age 11.6 years) with simple complete transposition 4.4 years after intracardiac repair. A disturbance in the lung function (greater than 2 SD from the normal value) was found in 88% of the patients. A marked increase in static recoil pressure (P less than 0.001) was most frequent (in 66%). Static lung compliance was only 75.1% of the predicted values. Values of mean vital and total lung capacity were decreased (P less than 0.01 and 0.001). Residual volume, the ratios of functional residual versus total lung capacity and residual volume versus total capacity were all increased. The signs of decreased patency of the airways were found in only 4 patients. A negative correlation was detected between the indices of lung stiffness and the age of assessment of lung function. A positive correlation was found between the ratios between functional residual and total capacity and pulmonary blood flow. No other correlation between lung function data and pulmonary arterial pressure or flow was proved. Increased lung stiffness, restriction of lung volume and hyperinflation could influence unfavorably the long-term results in successfully treated patients with simple complete transposition.

• MeSH
• dítě MeSH
• dospělí MeSH
• hemodynamika MeSH
• lidé MeSH
• měření objemu plic MeSH
• mladiství MeSH
• plíce patofyziologie   chirurgie   MeSH
• pružnost MeSH
• respirační funkční testy MeSH
• transpozice velkých cév patofyziologie   chirurgie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• Publikační typ
• časopisecké články MeSH

Functional and morphological examination of the lungs was performed in rats 48 hours after intratracheal injection of 0.5 mg/kg of the herbicide paraquat. Pronounced tachypnoea was observed (235+/-20 c/min), which also persisted under urethane anaesthesia (210+/-18 c/min). In control rats the mean breathing rate was 115+/-11 and 90+/-9 c/min in wake and anaesthetized rats respectively. The rate of breathing decreased to comparable values in experimental and control rats after bilateral cervical vagotomy. The functional residual lung capacity was significantly increased in experimental rats. After vagotomy also this increase of functional residual capacity became normalized. Histologically the disease was characterized by focal formation of hyaline membranes and oedema with occasional haemorrhages and signs of inflammation. The significant role fo vagal function in lung pathology is demonstrated.

• MeSH
• dýchání MeSH
• krysa rodu Rattus MeSH
• otrava patofyziologie   MeSH
• paraquat otrava   MeSH
• plíce patofyziologie   MeSH
• plicní fibróza chemicky indukované   patofyziologie   MeSH
• respirační funkční testy MeSH
• tělesná hmotnost MeSH
• zvířata MeSH
• Check Tag
• krysa rodu Rattus MeSH
• mužské pohlaví MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• paraquat 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

Farber disease (FD) is a debilitating lysosomal storage disorder (LSD) caused by a deficiency of acid ceramidase (ACDase) activity due to mutations in the gene ASAH1. Patients with ACDase deficiency may develop a spectrum of clinical phenotypes. Severe cases of FD are frequently associated with neurological involvement, failure to thrive, and respiratory complications. Mice homozygous ( Asah1P361R/P361R) for an orthologous patient mutation in Asah1 recapitulate human FD. In this study, we show significant impairment in lung function, including low compliance and increased airway resistance in a mouse model of ACDase deficiency. Impaired lung mechanics in Farber mice resulted in decreased blood oxygenation and increased red blood cell production. Inflammatory cells were recruited to both perivascular and peribronchial areas of the lung. We observed large vacuolated foamy histiocytes that were full of storage material. An increase in vascular permeability led to protein leakage, edema, and impacted surfactant homeostasis in the lungs of Asah1P361R/P361R mice. Bronchial alveolar lavage fluid (BALF) extraction and analysis revealed accumulation of a highly turbid lipoprotein-like substance that was composed in part of surfactants, phospholipids, and ceramides. The phospholipid composition of BALF from Asah1P361R/P361R mice was severely altered, with an increase in both phosphatidylethanolamine (PE) and sphingomyelin (SM). Ceramides were also found at significantly higher levels in both BALF and lung tissue from Asah1P361R/P361R mice when compared with levels from wild-type animals. We demonstrate that a deficiency in ACDase leads to sphingolipid and phospholipid imbalance, chronic lung injury caused by significant inflammation, and increased vascular permeability, leading to impaired lung function.

• MeSH
• bronchoalveolární lavážní tekutina MeSH
• ceramidy metabolismus   MeSH
• fenotyp MeSH
• fosfolipidy metabolismus   MeSH
• homozygot MeSH
• kyselá ceramidasa fyziologie   MeSH
• modely nemocí na zvířatech * MeSH
• myši knockoutované MeSH
• myši MeSH
• plíce metabolismus   patologie   MeSH
• poškození plic etiologie   metabolismus   patologie   MeSH
• respirační funkční testy MeSH
• zvířata MeSH
• Check Tag
• myši MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• Asah1 protein, mouse MeSH Prohlížeč
• ceramidy MeSH
• fosfolipidy MeSH
• kyselá ceramidasa MeSH

Static lung volumes, lung elasticity, and airway patency indices were measured in 47 children operated on for ventricular septal defect (VSD). Open-heart surgery was performed at the age of 0.6-12.0 years (median 4.1 years). In the first group (34 subjects), after primary repair of the VSD, there was an increase in lung recoil pressure at 100% of total lung capacity (TLC) (128% of the predicted value) and a reduction in specific airway conductance (sGaw) (75% of the predicted value). In the second group (13 patients), who had had previous pulmonary artery banding at 0.2-4.0 years (median 0.7 year) there was an increased functional residual capacity/TLC ratio (111% of predicted value), reduced sGaw (69% of predicted value), and reduced maximum expiratory flow at 25% of vital capacity (79% of predicted value). Lung volumes were insignificantly reduced in both groups. The frequency of lung function disturbances was similar in the two groups (71% of patients in the former group and 77% of patients in the latter group). A linear positive correlation between specific airway conductance and the mean pulmonary artery pressure (mPAP) (r = 0.793, p < 0.006) was observed in children with an mPAP <30 mmHg prior to open-heart surgery. A positive correlation between static recoil pressure at full inflation and mPAP (r = 0.545, p < 0.03) was found in children with an mPAP > 30 mmHg. The severity of congenital heart disease prior to surgery and the influence of the timing of the surgical procedures may cause the differences in lung function tests between the groups.

• MeSH
• arteria pulmonalis chirurgie   MeSH
• celková kapacita plic MeSH
• defekty komorového septa chirurgie   MeSH
• dítě MeSH
• hemodynamika MeSH
• kardiochirurgické výkony škodlivé účinky   metody   MeSH
• kojenec MeSH
• lidé MeSH
• mechanika dýchání * MeSH
• obstrukce dýchacích cest etiologie   MeSH
• poddajnost plic MeSH
• předškolní dítě MeSH
• respirační funkční testy MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• předškolní dítě MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

Lung function tests (i.e., spirometry, flow volume, and body plethysmography) were performed in 213 patients (age 6-21 years, mean 11.3 years) with hemodynamically significant congenital heart defects: atrial septal defect, ventricular septal defect (VSD), tetralogy of Fallot, aortic stenosis and coarctation of the aorta. We measured lung vital capacity, total lung capacity (TLC), residual volume (RV), the percentage ratio of the latter two measurements (%RV/TLC), maximal expiratory flow rates at 25% and 50% of vital capacity, and specific airway conductance. Pulmonary restriction dominated in patients with tetralogy of Fallot; pulmonary hyperinflation was more frequent in patients with VSD and coarctation of the aorta; and obstruction of the airways was observed most frequently in patients with tetralogy of Fallot. In conclusion, we found a range of pathologic lung function parameters in patients with hemodynamically significant congenital heart defects.

• MeSH
• dítě MeSH
• dospělí MeSH
• hemodynamika MeSH
• lidé MeSH
• mladiství MeSH
• plíce patofyziologie   MeSH
• respirační funkční testy MeSH
• vrozené srdeční vady patofyziologie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mladiství MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Breath detection, i.e. its precise delineation in time is a crucial step in lung function data analysis as obtaining any clinically relevant index is based on the proper localization of breath ends. Current threshold or smoothing algorithms suffer from severe inaccuracy in cases of suboptimal data quality. Especially in infants, the precise analysis is of utmost importance. The key objective of our work is to design an algorithm for accurate breath detection in severely distorted data. METHODS: Flow and gas concentration data from multiple breath washout test were the input information. Based on universal physiological characteristics of the respiratory tract we designed an algorithm for breath detection. Its accuracy was tested on severely distorted data from 19 patients with different types of breathing disorders. Its performance was compared to the performance of currently used algorithms and to the breath counts estimated by human experts. RESULTS: The novel algorithm outperformed the threshold algorithms with respect to their accuracy and had similar performance to human experts. It proved to be a highly robust and efficient approach in severely distorted data. This was demonstrated on patients with different pulmonary disorders. CONCLUSION: Our newly proposed algorithm is highly robust and universal. It works accurately even on severely distorted data, where the other tested algorithms failed. It does not require any pre-set thresholds or other patient-specific inputs. Consequently, it may be used with a broad spectrum of patients. It has the potential to replace current approaches to the breath detection in pulmonary function diagnostics.

• Klíčová slova
• Automated breath detection, Breath end, Lung function testing, Medical algorithm design, Multiple breath washout test, Tidal breathing,
• MeSH
• algoritmy * MeSH
• diagnóza počítačová metody   MeSH
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• počítačové zpracování signálu * MeSH
• předškolní dítě MeSH
• respirační funkční testy 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
• práce podpořená grantem MeSH

• MeSH
• cystická fibróza patologie   patofyziologie   terapie   MeSH
• dítě MeSH
• hodnotící studie jako téma MeSH
• lidé MeSH
• obstrukce dýchacích cest diagnóza   MeSH
• plíce patologie   patofyziologie   MeSH
• respirační funkční testy metody   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

Studies of the relationships between low socio-economic status and impaired lung function were conducted mainly in Western European countries and North America. East-West differences remain unexplored. Associations between parental education and lung function were explored using data on 24,010 school-children from eight cross-sectional studies conducted in North America, Western and Eastern Europe. Parental education was defined as low and high using country-specific classifications. Country-specific estimates of effects of low parental education on volume and flow parameters were obtained using linear and logistic regression, controlling for early life and other individual risk factors. Meta-regressions were used for assessment of heterogeneity between country-specific estimates. The association between low parental education and lung function was not consistent across the countries, but showed a more pronounced inverse gradient in the Western countries. The most consistent decrease associated with low parental education was found for peak expiratory flow (PEF), ranging from -2.80 to -1.14%, with statistically significant associations in five out of eight countries. The mean odds ratio for low PEF (<75% of predicted) was 1.34 (95% CI 1.06-1.70) after all adjustments. Although social gradients were attenuated after adjusting for known risk factors, these risk factors could not completely explain the social gradient in lung function.

• MeSH
• dítě MeSH
• lidé MeSH
• plíce fyziologie   MeSH
• průřezové studie MeSH
• regresní analýza MeSH
• respirační funkční testy MeSH
• rodiče * MeSH
• společenská třída * MeSH
• stupeň vzdělání MeSH
• ukazatele zdravotního stavu MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Geografické názvy
• Evropa MeSH
• Severní Amerika MeSH

Nebulization with saline solution, although commonly used to alleviate respiratory symptoms, particularly in children, is often questioned concerning its effectiveness. In this study, we investigated the effects of isotonic saline nebulization on lung function in 40 children (mean age of 14±1 years) suffering from different types of airway disorders. Measurements were carried out directly before and up to 15 min after nebulization, for six days in a row, always on the same day time in the morning. The children were divided into two study groups according to the baseline ratio of forced expired volume in one second/forced vital capacity (FEV1/FVC), below and above 80 %. We found significant improvements after saline nebulization in FEV1, mid-expiratory flow at 50 % and 75 % of FVC (MEF50 and MEF75), and peak expiratory flow (PEF) in the group with the baseline FEV1/FVC less than 80 %. In contradistinction, children with an index greater than 80 % displayed no appreciable changes in the lung function variables when compared with the baseline level before saline nebulization. We conclude that isotonic saline nebulization might mitigate the functional signs of threatening pulmonary obstruction and as such may be clinically useful in pediatric patients with mild respiratory problems.

• MeSH
• lidé MeSH
• mechanika dýchání účinky léků   fyziologie   MeSH
• mladiství MeSH
• nebulizátory a vaporizátory * MeSH
• plíce účinky léků   fyziologie   MeSH
• poruchy dýchání diagnóza   patofyziologie   MeSH
• respirační funkční testy metody   MeSH
• solný roztok aplikace a dávkování   MeSH
• usilovný výdechový objem účinky léků   fyziologie   MeSH
• Check Tag
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• solný roztok MeSH