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Background: Current management practices and outcomes in weaning from invasive mechanical ventilation are poorly understood. We aimed to describe the epidemiology, management, timings, risk for failure, and outcomes of weaning in patients requiring at least 2 days of invasive mechanical ventilation. Methods: WEAN SAFE was an international, multicentre, prospective, observational cohort study done in 481 intensive care units in 50 countries. Eligible participants were older than 16 years, admitted to a participating intensive care unit, and receiving mechanical ventilation for 2 calendar days or longer. We defined weaning initiation as the first attempt to separate a patient from the ventilator, successful weaning as no reintubation or death within 7 days of extubation, and weaning eligibility criteria based on positive end-expiratory pressure, fractional concentration of oxygen in inspired air, and vasopressors. The primary outcome was the proportion of patients successfully weaned at 90 days. Key secondary outcomes included weaning duration, timing of weaning events, factors associated with weaning delay and weaning failure, and hospital outcomes. This study is registered with ClinicalTrials.gov, NCT03255109. Findings: Between Oct 4, 2017, and June 25, 2018, 10 232 patients were screened for eligibility, of whom 5869 were enrolled. 4523 (77·1%) patients underwent at least one separation attempt and 3817 (65·0%) patients were successfully weaned from ventilation at day 90. 237 (4·0%) patients were transferred before any separation attempt, 153 (2·6%) were transferred after at least one separation attempt and not successfully weaned, and 1662 (28·3%) died while invasively ventilated. The median time from fulfilling weaning eligibility criteria to first separation attempt was 1 day (IQR 0–4), and 1013 (22·4%) patients had a delay in initiating first separation of 5 or more days. Of the 4523 (77·1%) patients with separation attempts, 2927 (64·7%) had a short wean (≤1 day), 457 (10·1%) had intermediate weaning (2–6 days), 433 (9·6%) required prolonged weaning (≥7 days), and 706 (15·6%) had weaning failure. Higher sedation scores were independently associated with delayed initiation of weaning. Delayed initiation of weaning and higher sedation scores were independently associated with weaning failure. 1742 (31·8%) of 5479 patients died in the intensive care unit and 2095 (38·3%) of 5465 patients died in hospital. Interpretation: In critically ill patients receiving at least 2 days of invasive mechanical ventilation, only 65% were weaned at 90 days. A better understanding of factors that delay the weaning process, such as delays in weaning initiation or excessive sedation levels, might improve weaning success rates. Funding: European Society of Intensive Care Medicine, European Respiratory Society.

BackgroundA collaboration between the University of Michigan (UM) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated in MU through conducting Quality Improvement projects (QIP). The global aim was to improve nutritional status of children with CF (cwCF), with a specific aim to increase the mean BMI percentile (BMIp) for cwCF by 10 percentile points in 12 months. MethodsBody mass index (BMI) percentiles of cwCF were categorized as: nutritionally adequate (BMIp >= 50%); at risk (BMIp 25%-49%); urgently at risk (BMIp 10%-25%); critically at risk (BMIp < 10%). Appropriate interventions were made according to BMIp category every three months. Forced expiratory volume in one-second percent predicted (FEV1pp), and health-related quality of life (HRQoL) were evaluated. ResultsOne hundred and eight-two cwCF with a mean age of 9.1 +/- 4.3 years were included in the project. Baseline BMIp increased from 25.6 to 37.2 at the 12th month (p < 0.001). In the critically at-risk group BMIp increased from 3.6 to 20.5 (p < 0.001), in the urgently at risk group from 15.9 to 30.8 (p < 0.001), in the at risk group from 37.0 to 44.2 (p < 0.079) and in the nutritionally adequate group the increase was from 66.8 to 69.5 (p < 0.301). FEV1pp also improved significantly, from 81.3 +/- 20.6 to 85.9 +/- 20.8 (p < 0.001). Physical functioning, eating problems, and respiratory symptoms domains of the HRQoL evaluation improved (p < 0.05). ConclusionThis project has led to significant improvements in BMIp, FEV1pp and HRQoL of cwCF; similar projects could easily be implemented by centers in other developing countries.

© 2022 All right reserved by the Turkish Society of Cardiovascular Surgery. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes (http://creativecommons.org/licenses/by-nc/4.0/).Background: This study aims to investigate whether the coexistence of advanced renal cell carcinoma and inferior vena cava tumor thrombus could be treated with a multidisciplinary approach and teamwork and to evaluate early and mid-term results. Methods: Between January 2017 and December 2020, a total of 33 patients (28 males, 5 females; mean age: 55.8±13.2 years; range, 27 to 76 years) who underwent radical nephrectomy and thrombectomy of the inferior vena cava were retrospectively analyzed. Demographic characteristics of the patients, types of operations, postoperative data, mortality and morbidity rates were recorded. Results: Of the patients, 12% (n=4) had Stage 2 tumor thrombus, 60.6% (n=20) had Stage 3 tumor thrombus, and 27% (n=9) had Stage 4 tumor thrombus. A total of 55% (n=19) of the patients had right-sided renal cell carcinoma, while 45% (n=14) of them had a left-sided mass. Totally, 66% (n=22) of the patients underwent primary inferior vena cava repair. The thrombectomy procedure and a Dacron® patch was applied with patch plasty in 24% (n=8) of the patients, and Dacron® graft interposition was applied to the inferior vena cava in 9% (n=3) of the patients. The mean follow-up was 20.3±13.0 (range, 2 to 70) months. Deep vein thrombosis was detected in the follow-up of seven (21%) patients, and no pulmonary thromboembolism was observed during the postoperative follow-up period. The mean length of stay in the intensive care unit was 1.39±0.6 (range, 1 to 3) days. The 30-day mortality rate was 3%, due to the loss of one patient from massive pulmonary embolism intraoperatively. Conclusion: Vascular surgical procedures performed regardless of the stage of the tumor thrombus provide satisfactory mid-term results in patients with advanced renal cell cancer. Amaç: Bu çalışmada, renal hücreli karsinom ve inferior vena kava tümör trombüs birlikteliğinin multidisipliner bir yaklaşım ve ekip çalışması ile tedavi edilip edilemeyeceği araştırıldı ve erken ve orta dönem sonuçları değerlendirildi. Ça­lış­ma pla­nı: Ocak 2017 - Aralık 2020 tarihleri arasında, radikal nefrektomi ile birlikte inferior vena kavaya trombektomi uygulanan toplam 33 hasta (28 erkek, 5 kadın; ort. yaş: 55.8±13.2 yıl; dağılım, 27-76 yıl) retrospektif olarak incelendi. Hastaların demografik özellikleri, ameliyat türleri, ameliyat sonrası verileri, mortalite ve morbidite oranları kaydedildi. Bulgular: Hastaların %12’sinde (n=4) Evre 2 tümör trombüsü, %60.6’sında (n=20) Evre 3 tümör trombüsü, %27’sinde (n=9) Evre 4 tümör trombüsü vardı. Hastaların toplam %55’inde (n=19) sağ taraflı renal hücreli karsinom ve %45’inde (n=14) sol taraflı kitle mevcuttu. Hastaların toplam %66’sında (n=22) inferior vena kava primer olarak onarıldı. Hastaların %24’üne (n=8) trombektomi ve yama plasti ile Dacron® yama uygulandı ve %9’una (n=3) inferior vena kavaya Dacron® greft interpozisyonu yapıldı. Ortalama takip süresi 20.3±13.0 (dağılım, 2-70) ay idi. Yedi (%21) hastanın takibinde derin ven trombozu saptandı ve ameliyat sonrası takip döneminde pulmoner tromboemboli saptanmadı. Yoğun bakım ünitesinde ortalama kalış süresi 1.39±0.6 (dağılım, 1-3) gün idi. Bir hasta ameliyat sırası masif pulmoner tromboemboli nedeniyle kaybedildiği için 30 günlük mortalite oranı %3 idi. So­nuç: İleri evre renal hücreli kanser hastalarında tümör trombüsünün evresinden bağımsız olarak yapılan vasküler cerrahi işlemler tatmin edici orta vadeli sonuçlar sağlar.

Background and Aim Pulmonary embolism (PE) is a potentially life-threatening disease in children. The objective of the study is to evaluate the utility of adult-based pulmonary embolism rule-out criteria (PERC), Pediatric PE Model, and D-dimer in the diagnosis of PE in children. Material and Methods The study consisted of patients under 18 years of age who were consulted to the Pediatric Pulmonology Clinic for the evaluation of PE. Patients were divided into two groups based on the confirmation of PE. The group with the presence of PE (n = 20) consisted of children who were diagnosed with PE. The group with the absence of PE (n = 28) consisted of children with clinically suspected PE but negative diagnostic imaging. Adult validated clinical decision PERC rule and Pediatric PE Model were retrospectively applied to the patients. Results In the study, PERC demonstrated a sensitivity of 60% and a specificity of 46% for the diagnosis of PE in children. When PE Model was evaluated for the children, it was found a 50% sensitivity and 75% specificity. Combining PE Model and PERC rule with D-dimer did not increase the specificity and sensitivity. Smoking was found to be relevant for PE in the childhood. Twenty-five percent of the patients had a genetic tendency for PE. All of the patients had an underlying disease as well. Conclusion None of the current risk assessment tools (PE Model, PERC, D-dimer) were found to be accurate in predicting PE. Further larger population studies are still required to develop a better diagnostic approach.

European CF Society Neonatal Screening Working Group (ECFS NSWG): Sabine Renner , Max Zeyda , Elke de Wachter , Luc Regal , Felix Votava, Andrea Holubova, Marianne Skov , Tessa Morgan, Paul Bregeaut , Loretta O'Grady, Ines Bucci , Stefano Pantano , Simonetta Simonetti, Domenica De Venuto, Donatello Salvatore, Nicola Perrotti, Mimma Caloiero, Giuseppe Castaldo, Antonella Tosco, Francesca Righetti, Giovanna Pisi, Fiorella Battistini, Antonio Angeloni, Giuseppe Cimino, Giovanni Fiocchi, Antonella Angiolillo, Michela Cassanello, Luisella Alberti, Laura E Claut, Raffaele Badolato, Enza Pavanello, Benedetta Fabrizzi, Elisabetta Bignamini, Anna Cardillo, Mariangela Lombardo, Letizia Cocciadiferro, Lisa Termini, Daniela Dolce, Vito Terlizzi, Anna Tamanini, Francesca Pauro, Giancarlo la Marca, Elina Aleksejeva, Dita Gaidule-Logina, Stoika Fustik, Violeta Anastasovska, Marelle Bouva, Alastair Reid, Jennifer Cundick, Emma Lundman, Egil Bakkeheim, Katarzyna Zybert, Mariusz Oltarzewski, Laura Vilarinho, Victoria Sherman, Elena Kondratyeva, Sarah Smith, Gordana Vilotijevic Dautovic, Maria Knapkova, Zuzana Mydlova, Rosa Mª López, Valle Velasco, Felicitas Díaz Flores, Cristóbal Colón Mejeras, Eva Sl Pedersen, Ugur Ozcelik, Bulent Karadag, Halyna Makukh, Moat Stuart. European CF Society Neonatal Screening Working Group (ECFS NSWG): INSA - Laura Vilarinho Background: The aim of this study was to record the current status of newborn bloodspot screening (NBS) for CF across Europe and assess performance. Methods: Survey of representatives of NBS for CF programmes across Europe. Performance was assessed through a framework developed in a previous exercise. Results: In 2022, we identified 22 national and 34 regional programmes in Europe. Barriers to establishing NBS included cost and political inertia. Performance was assessed from 2019 data reported by 21 national and 21 regional programmes. All programmes employed different protocols, with IRT-DNA the most common strategy. Six national and 11 regional programmes did not use DNA analysis. Conclusions: Integrating DNA analysis into the NBS protocol improves PPV, but at the expense of increased carrier and CFSPID recognition. Some programmes employ strategies to mitigate these outcomes. Programmes should constantly strive to improve performance but large datasets are needed to assess outcomes reliably. Highlights: In 2022, newborn bloodspot screening (NBS) for CF is undertaken in 30 European countries, 26 of them are national programmes; Some programmes are still not achieving ECFS standards. Compared to 2014, there is an improvement in sensitivity but a deterioration in achieving a sufficient PPV; There continues to be a wide variety of approaches, but the majority of national programmes are now using DNA analysis as a 2nd tier; This survey demonstrates areas of good practice, but there is considerable scope for improvement in the quality of NBS for CF across Europe; The framework of the 20 parameters to calculate the 8 key outcomes should be part of any annual report of a CF NBS programme, and thus improve future surveys. The survey was funded by the European CF Society info:eu-repo/semantics/publishedVersion

Objective: The objective of this study was to evaluate and compare knowledge, attitude, and practice patterns between pulmonologists and allergists for adult asthma in Turkey. Methods: Questionnaire-based data were gathered from 236 pulmonologists and 62 allergists, who had been members of the Turkish Thoracic Society and Turkish National Society of Allergy and Clinical Immunology in January-March 2021. Univariate and multivariate statistics were used to determine the factors associated with primary reliever preferences. Results: Of the 298 physicians, 39% encountered at least five asthma patients daily. Spirometer was used frequently by both the allergists (82.3%) and pulmonologists (77.5%) for asthma diagnosis. Budesonide was the most preferred inhaler corticosteroid. Formoterol/budesonide was the most preferred ICS/LABA combination, followed by beclomethasone/formoterol and fluticasone/salmeterol for asthma treatment. For mild asthmatics, formoterol/ICS was the most preferred (72.6%) reliever among allergists, whereas salbutamol was the most preferred (66.1%) among pulmonologists (p < 0.001). Age and workplace were associated with salbutamol preference of doctors for mild asthmatics. Age, specialty, and patient examination time were significantly associated with salbutamol preference for severe asthmatics. Conclusions: The use of diagnostic tools, such as a spirometer, for asthma diagnosis was compatible with the guidelines. While recent updates of the guidelines indicate that salbutamol should not be used solely in mild asthmatics due to its harmful effects in long-term use, it still was the most preferred drug by pulmonologists. Postgraduate education programs are needed to improve compliance with the guidelines.

BackgroundEffectiveness studies with biological therapies for asthma lack standardised outcome measures. The COMSA (Core Outcome Measures sets for paediatric and adult Severe Asthma) Working Group sought to develop Core Outcome Measures (COM) sets to facilitate better synthesis of data and appraisal of biologics in paediatric and adult asthma clinical studies.MethodsCOMSA utilised a multi-stakeholder consensus process among patients with severe asthma, adult and paediatric clinicians, pharmaceutical representatives, and health regulators from across Europe. Evidence included a systematic review of development, validity and reliability of selected outcome measures plus a narrative review and a pan-European survey to better understand patients’ and carers’ views about outcome measures. It was discussed using a modified GRADE (Grading of Recommendations Assessment, Development and Evaluation) Evidence to Decision framework. Anonymous voting was conducted using predefined consensus criteria.ResultsBoth adult and paediatric COM sets include forced expiratory volume in 1 s (FEV1) as z-scores, annual frequency of severe exacerbations and maintenance oral corticosteroid use. Additionally, the paediatric COM set includes the Paediatric Asthma Quality of Life Questionnaire and Asthma Control Test or Childhood Asthma Control Test, while the adult COM set includes the Severe Asthma Questionnaire and Asthma Control Questionnaire-6 (symptoms and rescue medication use reported separately).ConclusionsThis patient-centred collaboration has produced two COM sets for paediatric and adult severe asthma. It is expected that they will inform the methodology of future clinical trials, enhance comparability of efficacy and effectiveness of biological therapies, and help assess their socioeconomic value. COMSA will inform definitions of non-response and response to biological therapy for severe asthma.

BackgroundPaediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome.MethodsA retrospective, descriptive multicentre follow-up study initiated from the European network for translational research in children's and adult interstitial lung disease (Cost Action CA16125) and chILD-EU CRC (the European Research Collaboration for Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before the age of 18 years.ResultsData of 124 patients from 26 centres (15 counties) were submitted, of whom 117 patients fulfilled the inclusion criteria. Diagnoses were idiopathic pulmonary haemosiderosis (n=35), DAH associated with autoimmune features (n=20), systemic and collagen disorders (n=18), immuno-allergic conditions (n=10), other childhood interstitial lung diseases (chILD) (n=5), autoinflammatory diseases (n=3), DAH secondary to other conditions (n=21) and nonspecified DAH (n=5). Median (IQR) age at onset was 5 (2.0–12.9) years. Most frequent clinical presentations were anaemia (87%), haemoptysis (42%), dyspnoea (35%) and cough (32%). Respiratory symptoms were absent in 23%. The most frequent medical treatment was systemic corticosteroids (93%), hydroxychloroquine (35%) and azathioprine (27%). Overall mortality was 13%. Long-term data demonstrated persistent abnormal radiology and a limited improvement in lung function.ConclusionsPaediatric DAH is highly heterogeneous regarding underlying causes and clinical presentation. The high mortality rate and number of patients with ongoing treatment years after onset of disease underline that DAH is a severe and often chronic condition. This large international study paves the way for further prospective clinical trials that will in the long term allow evidence-based treatment and follow-up recommendations to be determined.

OBJECTIVESSurgical management of pulmonary metastases in colorectal cancer patients is a debated topic. There is currently no consensus on this matter, which sparks considerable risk for international practice variation. The European Society of Thoracic Surgeons (ESTS) ran a survey to assess current clinical practices and to determine criteria for resection among ESTS members.METHODSAll ESTS members were invited to complete an online questionnaire of 38 questions on current practice and management of pulmonary metastases in colorectal cancer patients.RESULTSIn total, 308 complete responses were received (response rate: 22%) from 62 countries. Most respondents consider that pulmonary metastasectomy for colorectal pulmonary metastases improves disease control (97%) and improves patients’ survival (92%). Invasive mediastinal staging in case of suspicious hilar or mediastinal lymph nodes is indicated (82%). Wedge resection is the preferred type of resection for a peripheral metastasis (87%). Minimally invasive approach is the preferred approach (72%). For a centrally located colorectal pulmonary metastasis, the preferred form of treatment is a minimally invasive anatomical resection (56%). During metastasectomy, 67% of respondents perform mediastinal lymph node sampling or dissection. Routine chemotherapy is rarely or never given following metastasectomy (57% of respondents).CONCLUSIONSThis survey among the ESTS members underlines the change in practice of pulmonary metastasectomy with an increasing tendency in favour of minimally invasive metastasectomy and surgical resection is preferred over other types of local treatment. Criteria for resectability vary and controversy remains regarding lymph node assessment and the role of adjuvant treatment.

Background: A collaboration between the University of Michigan (U of M) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated to improve the health status of people with cystic fibrosis (pwCF) at MU through implementing Quality Improvement (QI) initiatives. The main aim was to improve lung function in children with FEV1pp

OBJECTIVES: The underlying mechanisms for the development of chronic thromboembolic pulmonary hypertension and prognostic biomarkers are not clear yet. Thus, our aim is to assess and identify new biomarkers for the expression of 84 key genes linked to angiogenesis. METHODS: Patients who had levels more than 1000 dynes·sec·cm(−5) were included in the test group, and the other patients were included in the control group. Twelve specimens were taken from the patients. RT(2) Profiler PCR Array (Qiagen) was used to quantify the expression of the 84 key genes. RESULTS: Eight patients (6 male, 2 female, median age 54.4 ± 13.1 years) who underwent pulmonary endarterectomy were included. Pulmonary vascular resistance improved significantly from 811 ± 390 dyn/s/cm(−5) to 413.3 ± 144.9 dyn/s/cm(−5) (P .005) after surgery. Median length of hospital stay was 11.62 ± 2.97 days. The test group had a distinct pattern of impaired angiogenic and antiangiogenic genes. The expression levels of TGFA, TGFB1, THBS2, THBS1, TGFBR1, SERPINE1, SERPINF1, TGFB2, TIMP2, VEGFC, IFNA1, TNF, CXCL10, NOS3, IGF1, and MMP14 were downregulated in the specimens from the patients who had higher pulmonary vascular resistance values, whereas some genes, including PDGFA, showed upregulation that was statistically nonsignificant in the same group. CONCLUSIONS: These results can lead to the development of new markers that could predict adverse outcomes of patients with CTEPH. Identification of new markers that are related to worse outcomes would enable screening patients for early diagnosis and treatment.

© 2022 Wiley Periodicals LLC.Objectives: Postoperative ventilatory strategies in patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) may have an impact on early postoperative complications. Our national Esophageal Atresia Registry was evaluated to define a possible relationship between the type and duration of respiratory support on postoperative complications and outcome. Study Design: Among the data registered by 31 centers between 2015 and 2021, patients with esophago-esophageal anastomosis (EEA)/tracheoesophageal fistula (TEF) were divided into two groups; invasive ventilatory support (IV) and noninvasive ventilatory support and/or oxygen support (NIV-OS). The demographic findings, gestational age, type of atresia, associated anomalies, and genetic malformations were evaluated. We compared the type of repair, gap length, chest tube insertion, follow-up times, tensioned anastomosis, postoperative complications, esophageal dilatations, respiratory problems requiring treatment after the operation, and mortality rates. Results: Among 650 registered patients, 502 patients with EEA/TEF repair included the study. Four hundred and seventy of patients require IV and 32 of them had NIV-OS treatment. The IV group had lower mean birth weights and higher incidence of respiratory problems when compared to NIV-OS group. Also, NIV-OS group had significantly higher incidence of associated anomalies than IV groups. The rates of postoperative complications and mortality were not different between the IV and NIV-OS groups. Conclusion: We demonstrated that patients who required invasive ventilation had a higher incidence of low birth weight and respiratory morbidity. We found no relation between mode of postoperative ventilation and surgical complications. Randomized controlled trials and clinical guidelines are needed to define the best type of ventilation strategy in children with EA/TEF.

SUMMARY – Background: The novel coronavirus disease 2019 (COVID-19) has a broad spectrum of clinical manifestations, the most common serious clinical manifestation of the coronavirus infection being pneumonia. Unfortunately, the optimal treatment approach is still uncertain. However, many studies have been conducted on the effectiveness of several medications in the treatment of COVID-19 infection. The aim of this study was to evaluate the effectiveness of the hydroxychloroquine (HCQ) + favipiravir (FAV) treatment regimen and HCQ alone by comparing the patient’s clinical response and laboratory results on the fifth day of treatment in patients hospitalized due to COVID-19 infection. Patients and methods: This retrospective cohort study was conducted in Malatya Training and Research Hospital between March 2020 and July 2020. The study included 69 patients with confirmed COVID-19 with pneumonia. The patients were divided into 2 groups, those receiving HCQ alone and those receiving the HCQ + FAV combination. Results: A total of 69 patients were included in the study, and the mean age was 60.09±15.56 years. A statistically significant decrease was observed in C-reactive protein (CRP) levels, at the end of the fifth day, in patients who received HCQ + FAV treatment (p=0.002), whereas there was no decrease in CRP levels in patients who received HCQ treatment alone. In addition, an increase in lymphocyte count and a better fever response was observed at the end of the fifth day in patients who received HCQ + FAV (p=0.008). However, there was no statistical difference between both treatment regimens in terms of hospital stay and treatment results (p=0.008, p=0.744, p=0.517). Conclusion: Although the combination of HCQ + FAV treatment was observed to be effective on CRP levels and fever response in patients with COVID-19 pneumonia, there was no difference in terms of hospital stay and discharge.

Copyright © The authors 2022.Bronchiectasis is being diagnosed increasingly in children and adolescents. Recurrent respiratory exacerbations are common in children and adolescents with this chronic pulmonary disorder. Respiratory exacerbations are associated with an impaired quality-of-life, poorer long-term clinical outcomes and substantial costs to the family and health systems. The European Respiratory Society (ERS) clinical practice guideline for the management of children and adolescents with bronchiectasis provided a definition of acute respiratory exacerbations for clinical use but to date there is no comparable universal definition for clinical research. Given the importance of exacerbations in the field, this ERS task force sought to obtain robust definitions of respiratory exacerbations for clinical research. The panel was a multidisciplinary team of specialists in paediatric and adult respiratory medicine, infectious disease, physiotherapy, primary care, nursing, radiology, methodology, patient advocacy and parents of children and adolescents with bronchiectasis. We used a standardised process that included a systematic literature review, parents\" survey and a Delphi involving 299 physicians (54 countries) caring for children and adolescents with bronchiectasis. Consensus was obtained for all four statements drafted by the panel as the disagreement rate was very low (range 3.6% to 6.4%). The panel unanimously endorsed the four consensus definitions for: non-severe and severe exacerbations as an outcome measure; non-severe exacerbation for studies initiating treatment and; resolution of a non-severe exacerbation; for clinical trials involving children and adolescents with bronchiectasis. This ERS task force proposes using these internationally derived, consensus-based definitions of respiratory exacerbations for future clinical paediatric bronchiectasis research.

Aim: Point of sales (POS) advertising bans had been legislated in many countries without adequate enforcement. The aim of this study is to identify new products and devices at POS that could facilitate tobacco or nicotine use. Method: Shadow reporting at POS methodology developed by Framework Convention Alliance was used to investigate the products sold in four commercially dense districts of Istanbul. POS in one kilometer radius were visited by walking tour at each district. The products their display and compliance with legislation were noted. Results: Out of 198 POS visited 31 sell a product called "menthol ball". These were tiny colorful balls of menthol mixed with other various aromas that are sold together with an instrument that stab the end of a standard cigarette and insert the ball into the cigarette. The POS closest to schools were more likely to stock metholl balls. Majority of the POS placed the menthol balls close to candies, mints, chewing gums at eye level of children, just below the cigarette shelf. Conclusion: Tobacco industry uses menthol to sustain addiction among adolescents. Menthol use in cigarettes was banned on 5th January 2020 in Turkey. Using menthol balls to "make your own cigarette" is a novel technique developed by the industry to appeal the youth. Public health community needs to be vigilant and take actions.

BackgroundThe aim of this study was to evaluate the prevalence of anxiety, depression, sleep, and associated factors in caregivers of children with spinal muscular atrophy (SMA). Materials and MethodsBeck Depression Inventory (BDI), the State-Trait Anxiety Inventory-State (STAI-S), the State-Trait Anxiety Inventory-Trait (STAI-T), and Pittsburgh Sleep Quality Index (PSQI) were used to assess the anxiety, depression, and sleep quality of the caregivers of children with SMA. Higher scores indicated worse outcome for all three questionnaires. ResultsFifty-six caregivers of children with SMA were included in the study. Median age of children was 6 (3.2-10) years and mean age of the caregivers was 37.0 +/- 6.5 years. Median scores of the BDI, STAI-S, STAI-T, and PSQI were 12 (7.2-17), 35.5 (31-44), 40.5 (35-48), and 7.0 (5.0-10.0), respectively. There was a positive correlation between BDI and PSQI scores (p < 0.05). There was a negative correlation between the age of the caregivers and PSQI, BDI, STAI-T scores (p = 0.01, r = -0.341; p = 0.006, r = -0.364; p = 0.003, r = -0.395, respectively). There was a negative correlation between the age of the patients and the PSQI scores of the caregivers (p = 0.01, r = -0.33). There was a negative correlation between BDI scores and household income (p = 0.01, r = -0.34). ConclusionCaregivers of children with SMA had elevated depression and anxiety levels and they also had decreased sleep quality. Economic and social support resources are needed to help caregivers of those children.

Background: Prolonged survival of patients with chronic respiratory failure increased the number of patients receiving long-term home ventilation (LTHV). This study aimed to describe the aetiology and patterns of the use of LTHV in Istanbul. Methods: This cross-sectional study included children on home invasive ventilation (IV) and non-invasive ventilation (NIV) from six tertiary hospitals. Data including underlying diagnosis and LTHV were collected from patients' charts. Results: Study included 416 patients. The most common diagnoses were neuromuscular (35.1%) and neurological diseases (25.7%). While 49.5% (n=206) received NIV, 50.5% (n=210) of the patients received IV. The median age at initiation of LTHV was lower in the IV group (10 vs. 41 months, p

In this article, Early Career Task Force Group members of the Turkish Thoracic Society summarize the European Respiratory Society 2020 virtual congress. Current developments in the field of respiratory diseases were compiled with the addition of sessions specific to coronavirus disease 2019 this year. Almost all of the congress sessions were examined, and the important and striking results of the congress were highlighted. Congress sessions were attended by expert researchers, and the prominent messages of each session were highlighted in short summaries. They were then grouped under relevant titles and ranked in order of meaning and relation. It was finalized by a team of researchers.

Background Postural and aerobic exercises are essential in rehabilitation in cystic fibrosis (CF). The aim of this study is to examine the effect of telerehabilitation on the quality of life, depression, and anxiety levels of children with CF and their caregivers\" mood and anxiety levels. Materials and Methods Patients between the ages of 6-13 with CF were randomized into two groups. Study group received an exercise program three times a week via Zoom for 12 weeks. Cystic Fibrosis Revised Questionnaire (CFQ-R), Anxiety and Depression Scale in Children-Revised (RCADS) were applied to the patients and State-Trait Anxiety Scale (STAI) and Beck Depression Inventory (BDI) were applied to the caregivers in the beginning and at the end of the program. Patients\" FEV1 levels and 6-min walk tests were also measured. Results Twenty-eight patient-caregiver dyads, 14 dyads in each group, completed the study. The initial mean RCADS-Major depressive disorder score of the patients in the exercise group was 6.21 +/- 3.11, and this value decreased to 3.92 +/- 3.79 at the end of the study and was significantly better (p < 0.02). A similar significant change was observed when the RCADS-generalized anxiety disorder score decreased from the initial mean level of 6.28 +/- 2.81 to 3.42 +/- 2.65 (p < 0.01). There were significant changes in improvement in the body image in telerehabilitation group. Similar significant changes were not observed in the control group. Caregivers\" anxiety and depression levels did not change significantly. Conclusion A short-term telerehabilitation program improved patients\" anxiety and depression levels, body image, and functional status. However, caregiver anxiety and depression levels did not change significantly.

Introduction: Investigations of muscle dysfunction in patients with idiopathic pulmonary fibrosis (IPF) are limited to peripheral muscles. However, decreased thoracic muscle mass is known and deterioration of chest wall muscle strength is not clear. Objective: The aims of the present study were to evaluate pectoralis muscle strength located on the chest wall and to investigate the relationship of spirometric measurements and respiratory muscle strength with pectoralis muscle strength. Methods: Elderly patient with IPF (mean disease duration 7.47 +/- 7.04 years) and the age-and sex-matched healthy volunteers were recruited in this cross-sectional study. The pulmonary function test was performed by a portable spirometer for spirometric variables and a gas analyzer for diffusing capacity for carbon monoxide (DLCO). Maximal inspiratory (MIP) and expiratory pressure (MEP) were measured with mouth pressure device. Modified Medical Research Council Dyspnea Scale (MMRC) was used to determined dyspnea severity. The pectoralis muscle strength was assessed isometrically during shoulder joint horizontal adduction movement with a hand-held dynamometer. Results: A total of 17 patients with IPF (9 males, mean age 69.06 +/- 3.94 years) and 19 healthy controls (10 males, mean age 70.95 +/- 4.99 years) were included. Patients with IPF had lower pectoralis muscle strength than healthy controls (p

Objective: Tobacco industry has been using the point of sales (POS) as a promotion outlet for their products. Previously, in Istanbul, the complience with the advertising ban has been found to be low. This study aims to investigate the effect of plaing packaging implementation on the compliance of the POS in 2020. Method: This observational study was carried out at the six trade-dense districts of Istanbul by using the "Framework Convention Alliance Shadow Reporting" Methodology in 2020. All the POS in 1 km diameter were visited. A form is filled about the display format and photos were taken. Results: Of the 177 POS visited ; at 100 % the display was at the eye-level of children, at 97 % the shelves contained tobacco advertising, at 92% the tobacco products were visible from outside the shop, at 85 % the stacking of the packs were against the legislation, at 80 % tax stamps were covering the pictorial warnings, at 73 % the display shelves were donations of the tobacco companies, at 52 % the pictorial warning were invisible due to perpendicular placement, at 31 % the shop sign indicated a tobacco brand. The same violation strategy was recorded in 93% of the sale points throughout various districts. A stacking design with various pack sizes was noted in all shops. Measurements and Main Results: A consistent pattern of stacking that made the pictorial warning invisible was noted in the majority of the POS in various districts of the city, indicating orchestrated industry interference. The findings of this study implies that plain packaging renders pictorial warnings prominent. The displays at POS must be made visible to the eye of the consumers by preventing industry interference.

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 (iPCD) and calculated z-scores for forced expiratory volume in the first second (FEV1) and forced vital capacity (FVC) 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 and 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 BMI 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 specialized centres. It is essential to develop strategies to reverse this tendency and improve prognosis.Take home messageLung function in children with PCD is reduced by age 6 years and further declines during the growth period. It is essential to develop strategies to improve prognosis.

Background/objective: Ultrasound-based diaphragmatic thickness fraction (DTF) is a reflection of the size and function of the diaphragm. This study aimed to examine the value of this measurement in identifying the high-risk chronic obstructive pulmonary disease patients for development of symptoms and exacerbations. Methods: This prospective observational study included 53 COPD patients. Respiratory function test results, USG-based diaphragmatic thickness, quality of life scores (mMRC), CAT symptom scores (COPD Assessment Test), and number of previous exacerbations and admissions were recorded. All study tests were performed on the same day for each patient. Results: Only age showed an inverse and weak relation with percent thickness fraction (r=-0.37, p=0.006). None of the other variables tested correlated significantly with percent thickness fraction. High and low risk/symptom groups for frequency of exacerbations, mMRC and CAT symptom scores, or ABCD risk/symptom groups did not differ with regard to percent thickness fraction. Conclusion: DTF measurements based on diaphragmatic US assessment in COPD patients seems to be unable to identify high-risk patients for symptoms and exacerbations as defined by GOLD ABCD composite disease index.

Amaç: Malign melanoma melanositlerin malign transfomayonu sonucu gelişir.Pulmoner metastazını pulmoner arterlere ulaşan tümör embolileri yoluyla yapar.Endobronşial yayılımlı malign melonom vakaları sınırlı sayıdadır. Gereç ve Yöntem: Nadir görülen endobronşial yayılım yapmış bronkoskopi ile tanı koyduğumuz vakamızı sunduk. Bulgular: 42 yaşında bayan hasta nefes darlığı şikaye ile başvurdu. 2006 da tanı konan sır a konjenital dev nevüs tanısı mevcu u.5 paket/yıl sigara hikayesi mevcu u. Fizik muayenede bel bölgesinde 25x15 cm boyularında yaklaşık tüm lumbar bölgeyi kaplayan gluteal bölgeye de yayılan dev nevüsü mevcu u. Vücu a özellikle sır a ve saçlı deride birden fazla çok sayıda nevüsler izlendi. Nevüslerde renk değişikliği tariflemiyordu. Sır aki dev nevüste kalınlaşma belir yordu. Solunum sistemi muayenesi doğaldı.Aksiller ele gelen 1x2 cm lik lenfadenopa si mevcu u. PA akciğer grafisinde sol hilar bölgede düzensizlik ve dansite ar şı mevcu u. Toraks BT’de sol üst lobda 40x30 mm kitlesel lezyon mevcu u. Bronkoskopide sol akciğer üst lob girişinde nevüs tarzında siyahımsı mukozadan kabarık endobronşial lezyon izlendi. Alınan bronkoskopik biyopsi, rça ve bronkoalveolar lavaj materyalleri malign melonom ile uyumlu geldi. Sonuç: Malign melanoma bağlı endobronşial metastaz nadirdir. Literatürde 1966-2002 yılları arası yapılmış geniş taramada 204 akciğer dışı kaynaklı endobronşial metastaz vakası saptanmış olup bunları sırasıyla meme(%35), böbrek(%17), kolon ve rektum(%15) oluşturmaktadır.. Deri kanseri vakaları sadece 9 vaka olup , 7’si malign melanom kökenli saptanmış r. Tanıda primer akciğer kanserinden ayırtetmek için bronkoskopi şar r. Malign melannomaya bağlı endobronşial metastazı olgumuzu literatürde nadir görülmEsi nedeniyle sunduk.

This article is also accessible online at: http://BioMedNet.com/karger Dear Sir, We have published a study documenting the effect of inhaled cyclosporin A (Cyc-A) on the rat airway inflammation in this issue [1]. Our approach to assess the effect of pure Cyc-A was not rigorous since we used a commercial Cyc-A ampoule (50 mg cyclosporin A, 278 mg ethanol and 650 mg castor oil in a 1-ml intravenous ampoule; Sandoz, Basel, Switzerland). We have recently studied 10 additional rats to evaluate the effect of the ingredients (ethanol, castor oil) on the airway inflammation of sensitized rats. Twenty-one days after the initial intraperitoneal ovalbumin injection (1 mg ovalbumin and 100 mg Al(OH)3 in 1 ml 0.9% NaCl), animals were administered a nebulized ethanol and castor oil solution (278 mg ethanol and 650 mg castor oil in 1 ml 0.9% NaCl, adjusted dose: 0.4 ml/kg diluted to 2 ml with 0.9% NaCl) 1 h prior to exposure to nebulized ovalbumin; the same procedure was repeated on the 2nd day. 18–24 h later, bronchoalveolar lavage (BAL), peripheral blood and lung tissue sampling were performed as previously described [2, 3]. There was a nonsignificant decrease in the percentage of neutrophils (26.3 B 26.8 vs. 7.4 B 2.1%; p ! 0.06), a significant decrease in macrophages (66.1 B 7.7 vs. 63.6 B 7.2%; p ! 0.02), a nonsignificant increase in lymphocytes (21.1 B 12.4 vs. 24.4 B 7.0%; p 1 0.05) and a significant increase in eosinophils (2.4 B 2.6 vs. 4.7 B 2.0%; p ! 0.02) in the BAL of the ingredient pretreated group as compared with the group pretreated with the commercial Cyc-A ampoule. On light microscopic examination of the lung tissue samples, a significantly higher eosinophil count per high-powered field (HPF) (!400) (0 B 0 vs. 2.6 B 3.9/HPF in trachea, p ! 0.05; 4.3 B 9.4 vs. 16.1 B 12.4 in bronchi, p ! 0.008; 19.4 B 38.4 vs. 35 B 2.2 in bronchioles, p ! 0.02 was obtained in the ingredient-pretreated group compared to the group pretreated with the commercial Cyc-A ampoule. The percentage of peripheral blood eosinophil was significantly decreased in the ingredient-treated group (6.9 B 4.7 vs. 2.2 B 2.7%; p ! 0.004) compared with the group treated with the commercial Cyc-A ampoule. Our former study published in this issue demonstrated that commercial Cyc-A ampoule inhalation inhibits eosinophilia nonsignificantly in BAL and significantly in lung tissue in sensitized rat airway walls, with an increase of neutrophils in BAL and increase of peripheral blood eosinophils. This second part of the study showed that ingredients have no effect on BAL and lung tissue eosinophilia. It is likely that the immunosuppressive effect of the commercial Cyc-A ampoule is not mediated by the ingredients. Interestingly, we were unable to find neutrophilia in BAL and lung tissue or eosinophilia in the peripheral blood in rats pretreated with the ingredients. We postulate that eosinophils migrate from the lung to the peripheral blood with pure Cyc-A; however, we are unable to explain the pulmonary neutrophilia due to pure Cyc-A and this should be tested prospectively. Nevertheless, the definitive suggestions as to the most appropriate Cyc-A therapy in asthma still await the results of pure Cyc-A solution studies.