Petr Pohunek

The European Cooperation in Science and Technology (COST) is a funding organisation for the creation of research networks, called COST Actions. COST Action 16125 was dedicated to European network for translational research in children's and adult interstitial lung disease. Working Group 5 of the COST Action CA16125 focussed on the problems of transition of children with interstitial lung diseases from paediatric to adult care. Results of this survey performed among members of the Working Group and some affiliated specialists showed highly variable and inadequate current system of transitions of these patients to adult care in most centres. In most centres there is no established and consistent procedure to guarantee appropriate transfer of information and prepare the patient for transition to a new specialist and a new team. Immediate action is therefore required. The Working Group has prepared a model procedure for the transition, based on protocols from several centres with es...

V clanku jsou uvedeny výsledky dotaznikoveho setřeni o poctu a typech bronchoskopických výkonů, personal¬niho a technickeho vybaveni bronchologických pracovisť v Ceske republice. Udaje se podařilo ziskat z 56 pracovisť provadějicich bronchoskopie u dospělých a z 9 provadějicich bronchoskopie u děti. V Ceske republice pracovalo 169 bronchoskopistů, kteři provedli během roku 2012 celkem 30 354 výkonů u dospělých pacientů. Z nich bylo l 767 provedeno s rigidnim instrumentariem, intervencnich výkonů bylo 665 (laserova intraluminalni resekce 217x, elektrokauterizace 133x, stent byl zaveden 120x). Ze specialnich diagnostických metod je nejoblibenějsi en-dobronchialni ultrazvuk. V roce 2012 bylo toto vysetřeni provedeno l 240x. Na 100 000 obyvatel připadlo 303 bronchoskopii. 12 bronchoskopistů pracujicich na dětských oddělenich provedlo 672 bronchoskopii. Výsledky soucasneho setřeni jsme mohli porovnat s výsledky podobných setřeni provedených u nas v letech 1980, 1996, 2009 i v zahranici. ...

A new pediatric fixed combination of beclometasone dipropionate (BDP) 50 μg and formoterol fumarate (FF) 6 μg via pressurized metered-dose inhaler (pMDI) (CHF1535, Chiesi, Italy) was investigated. In a double-blind, randomized, placebo-controlled, cross-over study, a single CHF1535 administration using AeroChamber Plus™ spacer device (2 actuations, total dose BDP 100 μg/FF 12 μg) was compared to the same pMDI free combination in 56 asthmatic children aged ≥ 5 and < 12 years. Primary efficacy variable was forced expiratory volume during the first second (FEV1) area under the curve corrected by time over 12 h following morning dose (AUC0-12h). Further CHF1535 doses (50 μg/6 μg, 100 μg/12 μg, and 200 μg/24 μg) were also explored. Adverse events, electrocardiogram, and vital signs were monitored for safety. CHF1535 was non-inferior to free combination [adjusted mean difference (95% CI) 0.004 L (− 0.050, 0.041] with lower confidence limit greater than the limit set at 0.1 L. FEV1 AUC0-12h of each CHF1535 dose vs placebo were 0.037 L (p = 0.160), 0.119 L (p < 0.001), and 0.094 (p < 0.001) for 50/6, 100/12, and 200/24, respectively. No safety signals were found. Conclusion: CHF1535 was as effective as free combination BDP/FF, with a trend towards a dose-related response. All treatments were safe. Trial registration: ClinicalTrials.gov ID: NCT01584492 What is Known: •Inhaled pressurized metered-dose solutions (pMDI) are the preferred treatment for pediatric asthma. •Combination therapy of inhaled corticosteroids and long-acting β2- agonists is a well-established approach to control airway inflammation and airway obstruction also in pediatric patients. What is New: •A novel pediatric pMDI fixed combination of beclomethasone dipropionate 50 μg and formoterol fumarate 6 μg (CHF 1535) was non-inferior to the free combination at the same dose in pulmonary function over the 12-h post-dose period in asthmatic children, with trend towards a dose-related response. What is Known: •Inhaled pressurized metered-dose solutions (pMDI) are the preferred treatment for pediatric asthma. •Combination therapy of inhaled corticosteroids and long-acting β2- agonists is a well-established approach to control airway inflammation and airway obstruction also in pediatric patients. What is New: •A novel pediatric pMDI fixed combination of beclomethasone dipropionate 50 μg and formoterol fumarate 6 μg (CHF 1535) was non-inferior to the free combination at the same dose in pulmonary function over the 12-h post-dose period in asthmatic children, with trend towards a dose-related response.

Introduction The number of patients with necrotizing pneumonia has increased in recent years. The aim of this study is to review the incidence, management, and outcome of pediatric necrotizing pneumonia requiring surgical therapy and to prove that lung resection results in favorable development of patients. We hypothesize that overall lung function in children after lung resection does not differ from that of the healthy population. Materials and Methods A retrospective tertiary referral center study with a prospective follow-up spirometric study of patients with necrotizing pneumonia managed between January 2010 and December 2019 was performed. Results The study cohort consisted of 1,295 patients admitted to the pediatric department for community-acquired pneumonia; 47 patients developed necrotizing pneumonia, 36 of whom underwent parenchymal lung resection. A 5-year rise in the occurrence of necrotizing pneumonia requiring resection was 77%, with a significant increase in the last...

In the current study we focused on changes in the immune parameters of patients with CF after lung transplantation (Tx), with particular emphasis on the interaction of the immune system, infection, the autoimmune phenomenon observed in some CF patients, and immunosuppression. Seven transplant patients with CF were investigated, 3 men and 4 women; the average age at Tx was 24.2 years (20.2-32.3). The parameters of both humoral immunity (immunoglobulins, complement, CRP, antinuclear and antineutrophil cytoplasmic antibodies) and cellular immunity (T and B lymphocytes, NK cells) were traced. We observed marked initial hyperimmunoglobulinemia, with a sharp drop in immunoglobulin levels within 1 month after Tx. Positivity for antineutrophil cytoplasmic antibodies (ANCA) was found in 3 patients before Tx. A strong ANCA positivity persisted 2 months after Tx despite deep introductory immunosuppression. In one patient ANCA positivity, after a transient negative result at months 2 and 12 aft...

Pulmonary exacerbations are a cause of significant morbidity in patients with primary ciliary dyskinesia (PCD) and are frequently used as an outcome measure in clinical research into chronic lung diseases. So far, there has been no consensus on the definition of pulmonary exacerbations in PCD.30 multidisciplinary experts and patients developed a consensus definition for children and adults with PCD. Following a systematic review, the panel used a modified Delphi process with a combination of face-to-face meetings and e-surveys to develop a definition that can be used in research settings for children and adults with PCD.A pulmonary exacerbation was defined by the presence of three or more of the following seven items: 1) increased cough, 2) change in sputum volume and/or colour, 3) increased shortness of breath perceived by the patient or parent, 4) decision to start or change antibiotic treatment because of perceived pulmonary symptoms, 5) malaise, tiredness, fatigue or lethargy, 6...

Background: In primary ciliary dyskinesia (PCD) there is no single diagnostic test. Different predictive tools have been proposed to guide referral of high-risk patients for further diagnostic workup. We aimed to test clinical index (CI) on a large unselected cohort and compare its characteristics with other widely used tools—PICADAR and NA-CDCF. Methods: CI, PICADAR, and NA-CDCF scores were calculated in 1401 patients with suspected PCD referred to our center. Their predictive characteristics were analyzed using receiver operating characteristics (ROC) curves and compared to each other. Nasal nitric oxide (nNO) was measured in 569 patients older than 3 years. Results: PCD was diagnosed in 67 (4.8%) patients. CI, PICADAR, and NA-CDCF scores were higher in PCD than in nonPCD group (all p < 0.001). The area under the ROC curve (AUC) for CI was larger than for NA-CDCF (p = 0.005); AUCPICADAR and AUCNA-CDCF did not differ (p = 0.093). An overlap in signs and symptoms among tools was ...

The ciliary ultrastructure can be damaged in various situations. Such changes include primary defects found in primary ciliary dyskinesia (PCD) and secondary defects developing in secondary ciliary dyskinesia (SCD). PCD is a genetic disease resulting from impaired ciliary motility causing chronic disease of the respiratory tract. SCD is an acquired condition that can be caused, for example, by respiratory infection or exposure to tobacco smoke. The diagnosis of these diseases is a complex process with many diagnostic methods, including the evaluation of ciliary ultrastructure using transmission electron microscopy (the golden standard of examination). Our goal was to create a program capable of automatic quantitative analysis of the ciliary ultrastructure, determining the ratio of primary and secondary defects, as well as analysis of the mutual orientation of cilia in the ciliary border. PCD Quant, a program developed for the automatic quantitative analysis of cilia, cannot yet be u...

Paediatric airway endoscopy is accepted as a diagnostic and therapeutic procedure, with an expanding number of indications and applications in children. The aim of this European Respiratory Society task force was to produce a statement on interventional bronchoscopy in children, describing the evidence available at present and current clinical practice, and identifying areas deserving further investigation. The multidisciplinary task force panel performed a systematic review of the literature, focusing on whole lung lavage, transbronchial and endobronchial biopsy, transbronchial needle aspiration with endobronchial ultrasound, foreign body extraction, balloon dilation and occlusion, laser-assisted procedures, usage of airway stents, microdebriders, cryotherapy, endoscopic intubation, application of drugs and other liquids, and caregiver perspectives. There is a scarcity of published evidence in this field, and in many cases the task force had to resort to the collective clinical exp...

Despite remarkable advances in our understanding of asthma, there are still several unmet needs associated with the management of pediatric asthma. A two-day, face-to-face, meeting was held in London, United Kingdom, on 28 and 29 October 2017 involving a group of international expert clinicians and scientists in asthma management to discuss the challenges and unmet needs that remain to be addressed in pediatric asthma. These unmet needs include a lack of clinical efficacy and safety evidence, and limited availability of non-steroid-based alternative therapies in patients <6 years of age. An increased focus on children is needed in the context of clinical practice guidelines for asthma; current pediatric practice relies mostly on extrapolations from adult recommendations. Furthermore, no uniform definition of pediatric asthma exists, which hampers timely and robust diagnosis of the condition in affected patients. There is a need for a uniform definition of pediatric asthma, clearl...

Primary ciliary dyskinesia (PCD) is a multigenic autosomal recessive condition affecting respiratory tract and other organs where ciliary motility is required. The extent of its genetic heterogeneity is remarkable. The aim of the study was to develop a cost-effective pipeline for genetic diagnostics using a combination of Sanger and next generation sequencing (NGS). Data and samples of 33 families with 38 affected subjects with PCD diagnosed in childhood were collected over the territory of the Czech Republic. A panel of 18 PCD causative or candidate genes was implemented into an Illumina TruSeq Custom Amplicon NGS assay, and three ancestral mutations in SPAG1 were screened by conventional Sanger sequencing, which was also used for the confirmation of the NGS results and for the analysis of familial segregation. The causative gene was DNAH5 in 11/33 (33%) probands, SPAG1 in 8/33 (24%), and DNAI1, CCDC40, LRRC6 in one family each. If the high proportion of subjects with bi-allelic ancestral mutations in SPAG1 is corroborated in other Caucasian populations, a simple Sanger sequencing test for these three mutations may serve as an effective pre-screening step, being followed by an NGS panel for other, much larger, PCD genes. We present a combination of Sanger sequencing with an NGS panel for known and candidate PCD genes, implemented in a moderate-size national collection of patients. This strategy has proven to be cost-effective, rapid and reliable, and was able to detect the causative gene in two thirds of our PCD patients. Pediatr Pulmonol. © 2015 Wiley Periodicals, Inc.

Monitoring of theophyllinaemia in the course of 24 h during treatment with theophylline anhydrate capsules (Euphyllin CR) was used in 20 children (9-15 years) and in 20 adults (18-61 years) with bronchial asthma. The doses used in children were 5.96 mg/kg at 7 a.m. and 10.2 mg/kg at 7 p.m., while in adults the morning and evening doses were similar (4.35 and 4.89 mg/kg). After the first dose in the morning the serum theophylline concentration (STC) did not differ in children and adults (10.4 and 10.5 mg/l). In adults the half-time of absorption was twice as high as in children (0.63 and 0.3 h), as well as the time when the peak theophylline concentration was reached (2.7 and 1.39 h). In children a double dose of the drug at night ensured a STC of 11.2 mg/l and a maximum theophyllinaemia (C max = 13.5 mg/l between 8 p.m. and 6 a.m. (measured by the EMIT method). In adults the values during the night were significantly lower than in day-time (STC: ANOVA test P less than 0.05, C max: T...

Background / Purpose: Idiopathic pulmonary fibrosis (IPF) is a serious disease in adults with a grim prognosis regardless of treatment. Childhood interstitial lung diseases (ILDs) are rare, however they have had a better prognosis than adult IPF. In some cases a common genetic background in childhood ILDs and adult IPF is supposed, namely in the genes for the surfactant proteins. Little is known about the immunogenetic background of both these diseases despite the possible influence of a cytokine genotype on the immunopathogenic pathways of lung fibrosis.The aim of our study was to investigate cytokine gene polymorphisms in adult IPF and in childhood ILDs and compare them with each other and with healthy controls. In 56 adult IPF patients , 19 children with ILDs and 145 controls we investigated polymorphisms of the IL-1alpha, IL-1beta, IL-1R, IL-1RA, IL-2, IL-4, IL-6, IL-10, IL-12, TNF-alpha, IFN-gamma, TGF-beta, IL-1 beta, IL-2, IL-4 and IL-4RA genes using a PCR- SSP methodology. M...