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Endophytic actinomycetes encompass bacterial groups that are well known for the production of a diverse range of secondary metabolites, including various antibiotics, antitumor, immunosuppressive agents, plant growth hormones, and have capacity of survive inside of plants tissues. Vochysia divergens is a Brazilian medicinal plant common isolated in the Pantanal region, and was focus of many researches, but the community endophytic remains unknown. Therefore, the goals of the present work were to carry out an initial assessment of antimicrobial, antitumor and antioxidant activities of crude extract produced by endophytic actinomycetes isolated from Vochysia divergens. Using 16S sequences, 10 isolates were classified as Microbispora sp. and two isolates were classified as Streptomyces sampsonii. The other two isolates were identified as Micromonospora sp. and are apparently undescribed species. The isolates were able to produce secondary metabolites with antioxidant activity, antitumor activity against of Glioblastoma cell and antimicrobial activity against bacteria Staphylococcus aureus, Escherichia coli, Pseudomonas aeruginosa, Methicillin-Resistant Staphylococcus aureus and the yeast Candida albicans. Taking into consideration the lack of effective medicaments for the treatment of Glioblastoma multiforme, and the increasing number of bacterial strains expressing resistance, the basic research using microorganisms from unexplored environmental showed can be an alternative to discover new secondary metabolites to treat these diseases. [ABSTRACT FROM AUTHOR]

Penelitian ini bertujuan untuk mengetahui hubungan antara pola asuh otoriter dengan bullying di sekolah pada siswa SMP. Hipotesis yang diajukan dalam penelitian ini adalah terdapat hubungan positif antara pola asuh otoriter dengan bullying di sekolah pada siswa SMP. Semakin tinggi pola asuh otoriter, maka bullying di sekolah akan semakin tinggi, demikian sebaliknya semakin rendah pola asuh otoriter, maka bullying di sekolah akan semakin rendah pula. Subjek penelitian ini adalah siswa Sekolah Menengah Pertama sebanyak 50 orang siswa, dengan usia 12-15 tahun. Alat pengumpul data menggunakan skala yaitu Skala Pola Asuh Otoriter dan Skala Bullying di sekolah. Hasil analisis dengan uji korelasi product moment diperoleh r sebesar 0,428 (p < 0,01). Hal ini menunjukkan adanya hubungan positif yang sangat signifikan antara pola asuh otoriter yang diberikan orang tua dengan bullying anak di sekolah, hipotesis yang diajukan dalam penelitian ini dapat diterima.

Background: The combination of cystic fibrosis transmembrane conductance regulator (CFTR) modulators elexacaftor, tezacaftor and ivacaftor (ELX/TEZ/IVA) has been approved for treatment of cystic fibrosis (CF) patients (pwCF) homozygous and heterozygous for Phe508del. We aim to assess the long-term effects of ELX/TEZ/IVA therapy on clinical outcomes in severe pwCF., Methods: Lung function, pulmonary exacerbation (PEx), sweat chloride concentration, body mass index (BMI) and the respiratory domain of the cystic fibrosis questionnaire-revised (CFQ-R RD) were prospectively evaluated in a cohort of pwCF who were candidates for inclusion in a compassionate program of ELX/TEZ/IVA therapy. All procedures were performed at baseline and then at 12 and 24 months after initiation of modulator therapy. The number of PExs in the year before the study enrollment was collected from our records., Results: Thirty-six adult pwCF (median age 36.7 years; BMI 19.8 kg/m 2 ; FEV1 36.5% predicted) were recruited from 2019. At 12 and 24 months after initiation, the absolute change in ppFEV1 (percent predicted forced expiratory volume in 1 s) from baseline was +12.5% (p < 0.0001) and +13% (p < 0.0001), respectively. A median of 4.0 exacerbations per patient was reported in the preceding year, while the median number of PExs was 0.0 and 1.0 after 12 and 24 months, respectively, of modulator therapy (both p < 0.0001). After 12 and 24 months of ELX/TEZ/IVA therapy, the CFQ-R RD score improved by 22.4 points (p < 0.0001) and 16.7 points (p < 0.0001), and sweat chloride levels decreased by 65.5 mmol/L (p < 0.0001) and 60 mmol/L (p < 0.0001), respectively. BMI significantly increased., Conclusions: Long-term ELX/TEZ/IVA combination therapy markedly impacts the clinical status of patients with severe CF, showing a sustained improvement in lung function and PEx rate., Competing Interests: Declaration of competing interest There is no conflict of interest for the authors., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

The need for energy rationalizing in farming operations require research that optimize grain crop conduction. The operations used in the processing and production of silage have limitations in energy optimization due to the lack of studies. This paper evaluated energy efficiency of whole-plant silage operations with the objective of favor the decision making. The adopted design of the experiment was in parcels (with seven replications), consisting of three harvesting modalities: single-line forage harvester, total area forage harvester, and total area forage harvester with support transshipment. The tractors were instrumented with sensors that measured engine rotation, travel speed, and hourly fuel consumption which were used to calculate field capacity, fuel consumption per area and per harvested mass, and production capacity of the harvester-tractor set. The results went to analysis of variance and subsequently to Tukey's test. The single had a faster speed and lower hourly fuel consumption, but smaller field capacity and greater energy expenditure for the mass. The use of support transshipment set with the front harvester allowed an improvement in the operation, with an increase in the worked area, and material processing (18%), and speed (13%), without differing in fuel expenditure. The total-area forage harvester modality showed smaller costs (USD 6.7), followed by the total-area forage harvester with support transshipment set (USD 7.7) and the single-line forage harvester (USD 9.38), respectively. The use of forage harvesters with a wider working width proved to be more efficient in terms of production costs per harvested hectare, validating it's reccomendation.

Background: The aim of this study was to assess the long-term clinical impact of the application of e-health as part of a virtual model of care in patients with Cystic Fibrosis (CF)., Methods: Digital care group (DCG) were deemed suitable for using the NuvoAir Home platform to monitor their disease at home as part of a virtual model of care project. The usual care group (UCG) remained on usual care. NuvoAir Home platform consists of a smartphone application, Bluetooth spirometer and a clinician portal. Data on pulmonary function, Cystic Fibrosis Questionnaire-Revised (CFQR) and pulmonary exacerbations were collected at baseline and after twelve months. A survey for the digital care group was emailed to evaluate their experience using the technology., Results: Between February 2020 and May 2020 a cohort of 43 CF patients were recruited for the DCG (26 females; mean age 31.6 ± 6.8; 16 homozygous for delta F508; FEV 1 48.4 ± 16.3% predicted) and 36 CF patients for UCG (18 females; mean age 29.1 ± 9.4; 6 homozygous for delta F508; FEV 1 77.0 ± 25.0% predicted). CFQ-R score improved significantly through 12 months in the DCG with a mean change of 13.8 points, p < 0.0001, and no changes for the UCG (p = 0.73). When we analyzed the subgroup of CF patients on digital and usual care who did not receive CFTR modulator therapy, we found a change in CFQ-R score which was significantly associated with the use of digital technology while adjusting for baseline differences (p = 0.020). There was no significant difference in the change in lung function and number of exacerbations. 90% of patients reported they understood their CF better using the NuvoAir Home platform. No changes in medical treatment were reported during that time., Conclusions: The application of digital technologies in the management of adults with CF showed an improvement in patients' quality of life. Using a virtual model of care was well accepted by CF patients and improved their understanding of their medical condition., Competing Interests: Declaration of competing interest The authors declare that they have no financial and personal relationships with other people or organizations that could inappropriately influence their work., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

A timely, confirmed diagnosis of Idiopathic Pulmonary Fibrosis (IPF) has a significant impact on the evolution of the disease. The current model of care in the Lazio region (in Italy) was assessed on the basis of real-world data provided by the four reference centers responsible for diagnosing and treating IPF. The 5-year, population-based, retrospective longitudinal study provided the data that is at the basis of the current proposal for a new clinical and therapeutic pathway (DTCP) and has been shared with regional decision makers. A DTCP must be defined and based on four pillars: GPs, pulmonologists, IPF centers, and telemedicine. Each must play a role within a sort of hub-and-spoke model. IPF centers remain the hubs, while spokes are identified in trained GPs and pulmonologists., Competing Interests: All authors has received a research grants from ALTEMS to participate to the study. For other studies, LR has received consulting fees from Boehringer Ingelheim, Roche, FibroGen, Celgene, RespiVant, Nitto, Bristol Myers Squibb, Pliant Therapeutics, Veracyte, CSL Behring, Novartis, and speaker honorarium from Boehringer Ingelheim, Roche, Zambon, CSL Behring and participated in Data Safety Monitoring Board or Advisory Boards for Boehringer Ingelheim, Roche, FibroGen, Veracyte. Departement of Experimental Medicine - Respiratory Medicine (Università di Roma “Tor Vergata”) was funded by Almirall, Boehringer Ingelheim, Chiesi Farmaceutici, Novartis, and Zambon. PR participated as a lecturer in scientific meetings and courses under the sponsorship of Almirall, AstraZeneca, Biofutura, Boehringer Ingelheim, Chiesi Farmaceutici, GlaxoSmithKline, Menarini Group, Mundipharma, and Novartis and as an advisor in scientific meetings and courses under the sponsorship of Almirall, AstraZeneca, Biofutura, Boehringer Ingelheim, Chiesi Farmaceutici, GlaxoSmithKline, Menarini Group, Mundipharma, and Novartis. AS has received consulting fee from Boehringer Ingelheim, Roche and participated in Data Safety Monitoring Board or Advisory Boards for Roche. Then received support for attending meetings and/or travel from Roche. FV has received a speaker honorarium from from Boehringer Ingelheim, Roche. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Di Bidino, Rogliani, Sebastiani, Ricci, Varone, Sgalla, Iovene, Bruni, Flore, D'Ascanio, Cavalli, Savi, Di Michele, Cicchetti and Richeldi.)

Background: Low compliance with drug therapy in patients with chronic respiratory diseases was a well-known issue even before the coronavirus pandemic, but its causes are not yet fully defined., Objective: To verify the adherence to drug therapy in patients with respiratory disease during the COVID-19 pandemic., Methods: From June to September 2020, about 700 patients of the Forlanini Hospital who had been unmonitored during the March-May 2020 lockdown in Italy received a questionnaire during the pneumological check-up based on self-reported information on compliance with therapy during lockdown., Results: 284 out of the 418 returned questionnaires could be used in this study: 179 patients (63.0%) responded positively to the continuation of therapy, 18 (6.3%) reduced the dosage of their medication and 82 (28.9%) interrupted the therapy., Conclusions: The low percentage of patients that reduced their drug dosage may be due to an increased awareness of drug treatment benefits, and may also be ascribed to the Government healthcare strategy during lockdown.

Background: Over the last decades aggressive interventions have been successful to improve nutritional outcomes in people with cystic fibrosis (CF). As a result, with improvement of life expectancy and new CFTR modulators, overweight and obesity are progressively becoming a source of concern for adult population and in developed countries., Methods: This was a multicenter, observational, cross-sectional study of 321 adults with CF at three large CF centers in Italy. Patients were divided into three groups according to BMI classes, overweight and obesity (OW) group including patients with BMI ≥25 kg/m 2 , normal weight (NW) group with BMI 18.6-24.9 kg/m 2 and underweight (UW) group with BMI ≤18.5 kg/m 2 ., Results: We demonstrated that prevalence of OW in adults with CF in Italy is 22%. OW status is independently associated with male sex (OR 3.520, P = 0.001), pancreatic sufficiency (OR 2.873, P = 0.014) and older age at diagnosis (1.015, P = 0.042). BMI correlated with ppFEV1 (r = 0.337; P<0.0001) with median ppFEV1 significantly higher in patients with OW than comparisons. We also reported preliminary data on unfavorable cardiovascular risk factors in a subgroup of patients, where median blood levels [IQR] of cholesterol and systemic hypertension [%] were significantly higher in the OW group than in the NW and UW., Conclusions: People with CF and OW is a relevant patient group that might deserve better definition and proper clinical management., Competing Interests: Declaration of competing of interest The authors declare no conflict of interest., (Copyright © 2021. Published by Elsevier B.V.)

In the physiopathology of cystic fibrosis (CF), oxidative stress implications are recognized and widely accepted. The cystic fibrosis transmembrane conductance regulator (CFTR) defects disrupt the intracellular redox balance causing CF pathological hallmarks. Therefore, oxidative stress together with aberrant expression levels of detoxification genes and microRNAs (miRNAs/miRs) may be associated with clinical outcome. Using total RNA extracted from epithelial nasal cells, the present study analyzed the expression levels of oxidative stress genes and one miRNA using quantitative PCR in a representative number of patients with CF compared with in healthy individuals. The present pilot study revealed the existence of an association among CFTR, genes involved in the oxidative stress response and miR-125b. The observed downregulation of CFTR gene expression was accompanied by increased expression levels of Nuclear factor erythroid derived-2 like2 and its targets NAD(P)H:Quinone Oxidoreductase and glutathione S-transferase 1. Moreover, the expression levels of heme oxygenase-1 (HO-1) and miR-125b were positively correlated with a forced expiratory volume in 1 sec (FEV1) >60% in patients with CF with chronic Pseudomonas aeruginosa lung infection (r=0.74; P<0.001 and r=0.57; P<0.001, respectively). The present study revealed the activation of an inducible, but not fully functional, oxidative stress response to protect airway cells against reactive oxygen species-dependent injury in CF disease. Additionally, the correlations of HO-1 and miR-125b expression with an improved FEV1 value suggested that these factors may synergistically protect the airway cells from oxidative stress damage, inflammation and apoptosis. Furthermore, HO-1 and miR-125b may be used as prognostic markers explaining the wide CF phenotypic variability as an additional control level over the CFTR gene mutations., Competing Interests: The authors declare that they have no competing interests., (Copyright © 2021, Spandidos Publications.)

Background: During the ongoing pandemic of coronavirus disease 2019 (COVID-19), lockdown periods have changed the way that people and communities live, work and interact., Case Presentation: This case report describes an uncommon but important presentation of allergic bronchopulmonary aspergillosis (ABPA) in a previously healthy male, who decided to live in the basement of his house when Italy entered a nationwide lockdown during the COVID-19 pandemic. As high resolution computed tomography (HRCT) of the chest on admission showed diffuse miliary nodules, a miliary tuberculosis was initially suspected. However, further investigations provided a diagnosis of unusual presentation of ABPA., Conclusions: This case highlights the importance of maintaining awareness of Aspergillus-associated respiratory disorders during the COVID-19 pandemic, especially because lifestyle changes associated with home isolation carry an increased risk of exposure to mold spores present in some indoor environments.

Background: Regular physical activity (PA) is a valued part of cystic fibrosis (CF) care. Although the accelerometer, SenseWear Armband (SWA), accurately measures habitual PA in CF, it is mostly used for research purposes. For the first time, we analyzed different methods of measuring PA in daily life by the use of smartphones and other electronic devices such as smartwatch and Fitbit., Methods: Twenty-four stable adults with CF (mean age 37.5 ± 11.5SD yrs.; FEV 1 58 ± 19% predicted, BMI 22.9 ± 3.2) were studied. Daily PA was monitored for seven consecutive days. All patients wore the accelerometer SWA and at the same time they monitored PA with the electronic device they used routinely. They were allocated into one of four arms according to their device: Smartwatch, Fitbit, Android smartphones and iOS smartphones. PA related measurements included: duration of PA, energy expenditure, number of steps., Results: There was a good agreement between SWA and Fitbit for number of steps (p = 0.605) and energy expenditure (p = 0.143). iOS smartphones were similar to SWA in monitoring the number of steps (p = 0.911). Significant differences were found between SWA and both Smartwatch and Android smartphones., Conclusions: Fitbit and iOS smartphones seem to be a valuable approach to monitor daily PA. They provide a good performance to measure step number compared to SWA.

Introduction: Malignant diseases are well-known complications after lung transplantation (LT). Among these, inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with a not well-known and often aggressive biological behavior., Material and Methods: We hereby describe 2 cases of cystic fibrosis patients who underwent bilateral sequential LT (BSLT) complicated by IMT., Results: A 26-year-old man presented a right endobronchial lesion 6 months after BSLT. Two consecutive fiber bronchoscopic biopsies showed granulation tissue. For the persistent lesion growth, the patient underwent a transthoracic biopsy showing histologic diagnosis of IMT. Therefore, he underwent to right pneumonectomy that was unfortunately complicated after 6 months with a late bronchopleural fistula and empyema with exitus 6 months later. A 31-year-old woman 1 year after BSLT presented with a left voluminous pleural-parenchymal lesion; the histologic examination after biopsy revealed an IMT. She underwent a removal of the lesion with a macroscopic R0 resection. Histologic, immunophenotypic, and cytogenetic examinations showed a strong overexpression of anaplastic lymphoma kinase requiring biological adjuvant therapies; however, the patient refused it. Four years later, she presented a recurrence treated with debulking procedure and adjuvant radiotherapy. At last follow-up, the patient was alive with stable disease and optimal graft function., Conclusions: Although IMT is a rare complication after lung transplant, to obtain a careful diagnosis, an early and aggressive treatment is mandatory., (Copyright © 2019 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Burkholderia contaminans is one of the 20 closely related bacterial of the Burkholderia cepacia complex, a group of bacteria that are ubiquitous in the environment and capable of infecting people with cystic fibrosis (CF). This species is an emerging pathogen and it has been widely isolated from CF patients in Argentina, Spain, Portugal, Australia, Canada, USA with a low prevalence in Ireland, France, Russia, Switzerland, Czech Republic, and Italy. This is the first report of B. contaminans affecting two Italian CF patients attending the same CF Centre. We correlate B. contaminans colonisation with lung function decline and co-infection with other clinically relevant CF pathogens., Case Presentation: B. contaminans was identified by Multi Locus Sequence Typing in routine sputum analysis of two Caucasian CF women homozygous for Phe508del CFTR mutation. Sequence Type 102 was detected in both strains. It is known that B. contaminans ST102 was isolated both from CF and non-CF patients, with an intercontinental spread across the world. Random Amplified Polymorphic DNA analysis revealed the genetic relatedness between the two strains. We examined their susceptibility to antimicrobial agents, comparing the latter with that recorded for other B. contaminans isolated from different countries. We also described key virulence factors possibly linked with a clinical outcome. Specifically, we attempted to correlate colonization with the incidence of acute exacerbation of symptoms and lung function decline., Conclusions: This case presentation suggests that acquisition of B. contaminans ST102 is not directly associated with a lung function decline. We retain that the presence of other CF pathogens (i.e. MRSA and Trichosporon) along with B. contaminans ST102 might have contributed to the worsening of clinical conditions in our CF patients. The circumstances leading to the establishment of B. contaminans ST102 infections are still unknown. We highlight the importance to proper detect and typing bacteria implicated in CF infection by using molecular techniques.

Despite being a hallmark and an independent prognostic factor in several cardiopulmonary diseases, ventilatory efficiency-i.e., minute ventilation/carbon dioxide output relationship (V̇e/V̇co 2 )-has never been systematically explored in cystic fibrosis (CF). To provide a comprehensive frame of reference regarding measures of ventilatory efficiency in CF adults with normal to moderately impaired lung function and to confirm the hypothesis that V̇e/V̇co 2 is a sensitive marker of early lung disease. CF patients were divided into three groups, according to their spirometry: normal (G 1 ), mild impairment (G 2 ), and moderate impairment (G 3 ) in lung function. All participants underwent incremental cardiopulmonary exercise testing on a cycle ergometer. Lowest V̇e/V̇co 2 ratio (nadir) and the slope and the intercept of the linear region of the V̇e/V̇co 2 relationship were contrasted in a two-center retrospective analysis, involving 72 CF patients and 36 healthy controls (HC). Compared with HC, CF patients had significantly higher V̇e/V̇co 2 nadir, slope, and intercept ( P < 0.001, P < 0.001, and P = 0.049, respectively). Subgroup analysis revealed significant differences in nadir ( P = 0.001) and slope ( P = 0.012) values even between HC and G 1 . Dynamic hyperinflation related negatively with slope ( P = 0.045) and positively with intercept ( P = 0.001), while no impact on nadir was observed. Ventilatory inefficiency is a clear feature of adults with CF, even among patients with normal spirometry. V̇e/V̇co 2 nadir seems to be the most reliable metric to describe ventilatory efficiency in CF adults. Further prospective studies are needed to clarify whether V̇e/V̇co 2 could represent a useful marker in the evaluation of early lung disease in CF. NEW & NOTEWORTHY This is the first study to investigate ventilatory efficiency in a cohort of adult cystic fibrosis (CF) patients with nonsevere lung disease. The finding of impaired ventilatory efficiency in patients with normal lung function confirms the higher sensitivity of exercise testing in detecting early lung disease compared with spirometry. Dynamic hyperinflation plays a significant role in determining the behavior of V̇e/V̇co 2 slope and intercept values with increasing lung function impairment. Apparently free from interference from mechanical constraints, V̇e/V̇co 2 nadir seems the most reliable parameter to evaluate ventilatory efficiency in CF adults.

The combination of the corrector lumacaftor with the potentiator ivacaftor has been approved for treatment of cystic fibrosis (CF) patients homozygous for the Phe508del CFTR mutation. There are no reports detailing the effect of lumacaftor-ivacaftor on physical activity (PA) and exercise tolerance. We performed incremental cardiopulmonary exercise testing (CPET) and we assessed PA pre- and post 2 years initiation of lumacaftor-ivacaftor in three CF adults. PA of mild intensity improved by +13% in patient 1, + 84% in patients 2 and + 89% in patient 3. Oxygen uptake increased both at anaerobic threshold and at peak exercise (patient 1 + 33%, patient 2 + 42% and patient 3 + 20%). Daily physical activities and exercise tolerance improved after two years of lumacaftor-ivacaftor therapy., (Copyright © 2019. Published by Elsevier B.V.)

Background: The aim of this study was to investigate the relationship between dynamic hyperinflation and daily physical activity (DPA) in adults with cystic fibrosis (CF)., Methods: Thirty-four clinically stable CF were studied. All patients undertook incremental cardiopulmonary exercise testing (CPET). CPET-related measurements included: oxygen uptake (V'O 2 ), carbon dioxide production (V'CO 2 ), ventilatory profile, work rate (W), inspiratory capacity (IC), end-expiratory lung volume (EELV). PA was assessed using the accelerometer SenseWear Pro3 Armband., Results: Exercise tolerance was reduced in most of patients and the mean V'O 2,peak value was 75.2% of predicted (28.5 ± 4.8 ml/min/kg). Seventy % of patients responded to CPET with dynamic hyperinflation. Higher incidence of dynamic hyperinflation was found in CF males compared to CF females (p = 0.026). Patients who developed dynamic hyperinflation during CPET had higher vigorous PA (p = 0.01) and more total energy expenditure (p = 0.006) than patients who did not. EELVΔ was related to activities requiring vigorous intensity and total energy expenditure (R = 0.46, p = 0.001; R = 0.57, p <  0.001)., Conclusions: In adults with CF and mild to moderate lung impairment, DPA might not be limited by dynamic hyperinflation.

Rationale: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells. Variations of CFTR dysfunction among patients with cystic fibrosis may be an important determinant of maximal exercise capacity in cystic fibrosis. Previous studies on the relationship between CFTR genotype and maximal exercise capacity are scarce and contradictory., Objectives: This study was designed to explore factors influencing maximal exercise capacity, expressed as peak oxygen uptake (V.O2peak), with a specific focus on CFTR genotype in children and adults with cystic fibrosis., Methods: In an international, multicenter, cross-sectional study, we collected data on CFTR genotype and cardiopulmonary exercise tests in patients with cystic fibrosis who were ages 8 years and older. CFTR mutations were classified into functional classes I–V., Results: The final analysis included 726 patients (45% females; age range, 8–61 yr; forced expiratory volume in 1 s, 16 to 123% predicted) from 17 cystic fibrosis centers in North America, Europe, Australia, and Asia, all of whom had both valid maximal cardiopulmonary exercise tests and complete CFTR genotype data. Overall, patients exhibited exercise intolerance (V.O2peak, 77.3 ± 19.1% predicted), but values were comparable among different CFTR classes. We did not detect an association between CFTR genotype functional classes I–III and either V.O2peak (percent predicted) (adjusted β = −0.95; 95% CI, −4.18 to 2.29; P = 0.57) or maximum work rate (Wattmax) (adjusted β = −1.38; 95% CI, −5.04 to 2.27; P = 0.46) compared with classes IV–V. Those with at least one copy of a F508del-CFTR mutation and one copy of a class V mutation had a significantly lower V.O2peak (β = −8.24%; 95% CI, −14.53 to −2.99; P = 0.003) and lower Wattmax (adjusted β = −7.59%; 95% CI, −14.21 to −0.95; P = 0.025) than those with two copies of a class II mutation. On the basis of linear regression analysis adjusted for relevant confounders, lung function and body mass index were associated with V.O2peak., Conclusions: CFTR functional genotype class was not associated with maximal exercise capacity in patients with cystic fibrosis overall, but those with at least one copy of a F508del-CFTR mutation and a single class V mutation had lower maximal exercise capacity.

We described physical activity measures and hourly patterns in patients with chronic obstructive pulmonary disease (COPD) after stratification for generic and COPD-specific characteristics and, based on multiple physical activity measures, we identified clusters of patients. In total, 1001 patients with COPD (65% men; age, 67 years; forced expiratory volume in the first second [FEV 1 ], 49% predicted) were studied cross-sectionally. Demographics, anthropometrics, lung function and clinical data were assessed. Daily physical activity measures and hourly patterns were analysed based on data from a multisensor armband. Principal component analysis (PCA) and cluster analysis were applied to physical activity measures to identify clusters. Age, body mass index (BMI), dyspnoea grade and ADO index (including age, dyspnoea and airflow obstruction) were associated with physical activity measures and hourly patterns. Five clusters were identified based on three PCA components, which accounted for 60% of variance of the data. Importantly, couch potatoes (i.e. the most inactive cluster) were characterised by higher BMI, lower FEV 1 , worse dyspnoea and higher ADO index compared to other clusters ( p < 0.05 for all). Daily physical activity measures and hourly patterns are heterogeneous in COPD. Clusters of patients were identified solely based on physical activity data. These findings may be useful to develop interventions aiming to promote physical activity in COPD.

Background: The aim of this study was to examine the relationship between pulmonary exacerbations and physical activity (PA) in adults with cystic fibrosis (CF)., Methods: We grouped adults with CF according to their exacerbation status in the year before study enrollment: (1) <1 exacerbation/year; (2) 1-2 exacerbations/year; and (3) >2 exacerbations/year. PA was assessed objectively by means of an accelerometer at the time of study enrollment., Results: Patients with >2 exacerbations/year spent less time in PA; specifically, fewer activities of mild intensity [>3 metabolic equivalents (METs)], and lower active energy expenditure (P = 0.01 and P = 0.03, respectively). After correcting for relevant confounders, PA levels were not related to the exacerbation frequency in the preceding year. PA at moderate intensity (4.8-7.2 METs) or greater (> 7.2 METs) was independently associated with gender and FEV1 % predicted (P = 0.007 and P = 0.04, respectively). Compared with men, women had reduced vigorous activities (P = 0.01) and active energy expenditure (P = 0.01)., Conclusions: Adult CF patients with more pulmonary exacerbations in the preceding year have more advanced disease and are less active than their peers. PA was independently associated with gender and airflow obstruction. Gender differences in PA are evident in CF adults.

Background: The best clinical practice to investigate aerobic fitness includes measurements obtained during cardiopulmonary exercise testing (CPET), however it remains an underutilised clinical measure in cystic fibrosis (CF). To investigate this further, different methods of quantifying exercise capacity in CF are required. The possibility that measuring physical activity (PA) by a portable accelerometer could be used to assess the CF aerobic state and could be added among the CPET surrogates has not been investigated. The aim of this study was to examine the relationship between PA and exercise fitness both at submaximal and maximal levels in clinically stable adults with CF., Methods: Thirty CF patients (FEV1 71 ± 19% predicted) and fifteen healthy controls undertook an incremental CPET on a cycle ergometer. CPET-related measurements included: oxygen uptake (V'O2), carbon dioxide production (V'CO2), ventilatory profile, heart rate (HR) and oxygen pulse (V'O2/HR) throughout exercise and at lactic threshold (LT) and peak. LT measures represent submaximal exercise related data. PA was assessed using the accelerometer SenseWear Pro3 Armband., Results: Moderate (>4.8 metabolic equivalents (METS)) and moderate + vigorous (>7.2 METS) PA was related to V'O2 (p = 0.005 and p = 0.009, respectively) and work rate (p = 0.004 and p = 0.002, respectively) at LT. Moderate PA or greater was positively related to peak V'O2 (p = 0.005 and p = 0.003, respectively). Daily PA levels were similar in CF and healthy controls. Except for peak values, V'O2 profile and the V'O2 at LT were comparable between CF and healthy controls., Conclusions: In adult CF patients daily PA positively correlated with aerobic capacity. PA measurements are a valuable tool in the assessment of exercise performance in an adult CF population and could be used for interventional exercise trials to optimize exercise performance and health status. PA levels and parameters obtained at submaximal exercise are similar in CF and in healthy controls.

The article compiles and analyses sample data for toxic components removed from waste electronic and electrical equipment (WEEE) from more than 30 recycling companies in Switzerland over the past ten years. According to European and Swiss legislation, toxic components like batteries, capacitors and printed wiring boards have to be removed from WEEE. The control bodies of the Swiss take back schemes have been monitoring the activities of WEEE recyclers in Switzerland for about 15 years. All recyclers have to provide annual mass balance data for every year of operation. From this data, percentage shares of removed batteries and capacitors are calculated in relation to the amount of each respective WEEE category treated. A rationale is developed, why such an indicator should not be calculated for printed wiring boards. The distributions of these de-pollution indicators are analysed and their suitability for defining lower threshold values and benchmarks for the depollution of WEEE is discussed. Recommendations for benchmarks and threshold values for the removal of capacitors and batteries are given., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Background: The aim of the present study was to determine whether different methods of recording physical activity (PA), i.e., accelerometers vs questionnaires, provided similar information in adults with cystic fibrosis (CF)., Methods: 20 CF (age 33 ± 8SD yrs, FEV1 68 ± 16% predicted) and 11 age-matched healthy controls completed the Habitual Activity Estimation Scale (HAES) questionnaire and wore a biaxial accelerometer (SenseWear Pro3 Armband). Exercise tolerance was measured in CF., Results: Patients had similar values in PA compared with controls. None of PA categories estimated by HAES questionnaire correlated with PA categories measured by the accelerometer; in CF the HAES questionnaire overestimated the effective levels of PA measured by the accelerometer. There were no differences between weekdays and weekend days PA levels provided by the accelerometer. In CF the questionnaire detected different time reported during the "somewhat inactive" and "somewhat active" categories (z = 2.651; p = 0.008; z = -2.651; p = 0.008), weekdays vs weekend; patients reported more time spent in activity (somewhat active & very active) during the weekend (z = -2.203; p = 0.02). Peak oxygen uptake correlated with accelerometer activities of "moderate" (>4.8 metabolic equivalents (METS)) and "vigorous" (>7.2 METS) intensity (r = 0.503, p = 0.02; r = 0.545, p = 0.01)., Conclusions: In adults with cystic fibrosis PA levels are better evaluated by the accelerometer and are similar to the controls. PA measured by the accelerometer is similar during the week and correlates with exercise tolerance., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Aim: Cystic fibrosis (CF) patients present an altered fatty acid (FA) metabolism characterized by imbalance in the arachidonic/docosohexasenoic acid (AA/DHA) ratio in favour of the former which can contribute to the increase in pulmonary inflammation. The present study aims to assess respiratory, nutritional, clinical and laboratory parameters, and inflammatory markers after six months of DHA supplementation in paediatric patients suffering from CF., Methods: A dose of 1 g/10 kg/die was administered to ten CF patients of paediatric age for the first month and 250 mg/10 kg/die for the remaining 5 months. We carried out follow-ups at T0 (baseline), T6 (after six months of the diet) and T12 (six months after supplementation was interrupted) during which respiratory, nutritional, clinical and laboratory parameters were assessed., Results: After six months of DHA supplementation inflammatory marker levels had diminished: interleukin 8 (IL-8) and Tumour Necrosis Factor Alfa (TNF-α) in serum, and calprotectin in stools. In addition, auxometric parameters were improved as was the clinical condition of patients, who tolerated DHA well., Conclusion: Dietetic integration with DHA seems to improve clinical condition and the inflammatory pulmonary and intestinal state of pediatric patients suffering from CF.

Chronic obstructive pulmonary disease (COPD) patients have reduced circulating hemopoietic progenitors. We hypothesized that severity of COPD parallels the decrease in progenitors and that the reduction in body mass index (BMI) could be associated with more severe bone marrow dysfunction. We studied 39 patients with moderate to very severe COPD (18 with low-BMI and 21 with normal-BMI) and 12 controls. Disease severity was associated to a greater reduction in circulating progenitors. Proangiogenetic and inflammatory markers correlated with disease severity parameters. Compared to normal-BMI patients, low-BMI patients showed: greater reduction in circulating progenitors; higher VEGF-A, VEGF-C, HGF, Ang-2, TNF-alpha, IL-6 and MCP-1 levels. Furthermore, among patients with similar pulmonary impairment, those who displayed low-BMI had a more markedly reduced number of CD34(+) cells and late endothelial progenitors. We show that the reduction in hematopoietic and endothelial progenitor cells correlates with COPD severity. Our findings also indicate that, in severe low-BMI COPD patients, bone marrow function seems to be further impaired and may lead to reduced reparative capacity., (Copyright 2009 Elsevier B.V. All rights reserved.)

Female genital schistosomiasis is not uncommon in endemic areas for schistosomiasis, but there are few reports in the Brazilian medical literature. Here, we describe the case of a 31-year-old woman with lower abdominal pain who was diagnosed as presenting a fallopian tube tumor caused by Manson's schistosomiasis. The diagnosis was delayed because her symptoms were considered nonspecific. Involvement of the parietal peritoneum of the ovarian fossa was observed during laparoscopy and confirmed by histological analysis. The left tube and the tumor were excised and schistosomiasis was treated with praziquantel. She presented a full recovery and options for future reproduction are under evaluation.

Purpose: to analyze interobserver variability in the histopathological diagnosis of premalignant breast lesions before and after training with diagnostic standardized criteria., Methods: Slides containing histological sections representative of three kinds of breast lesions (atypical ductal hyperplasia, ductal carcinoma in situ and ductal carcinoma in situ with microinvasion), revised by an international specialist in breast pathology whose diagnoses were considered as golden standard, have been used. The same slides have been evaluated at two different times by five pathologists from the community according to a specific protocol for classifying the lesions. In the first evaluation, the cases were analyzed and classified according to the specific criteria adopted in each service. At the second time, the pathologists were given a tutorial containing diagnostic criteria and representative images, and the lesions were classified again, employing the standardized criteria. Interobserver analysis using percent agreement and weighted Kappa index has been performed., Results: There has been a large diagnostic variation among the pathologists in the initial analysis without the use of standardized diagnostic criteria concerning the diagnostic, nuclear grade and histological grade (weighted Kappa indexes related to diagnosis varied from 0.15 to 0.40). In the second evaluation using standardized criteria, there has been a significant improvement in the diagnostic concordance among the five pathologists concerning the diagnosis, nuclear grade and histological grade (weighted Kappa indexes related to diagnosis have varied from 0.42 to 0.80)., Conclusions: interobserver concordance related to diagnosis and classification of breast premalignant lesions may be improved with specific training and the use of standardized histopathological criteria.

Heliox is a low density gas mixture of helium and oxygen commonly used in deep diving (> 6 ATM). This mixture has been also used for clinical purposes, particularly in the critical care setting. Due to of its physical proprieties, Heliox breathing reduces air flow resistances within the bronchial tree; in patients with obstructive lung diseases Heliox may also reduce the work of breathing and improve pulmonary gas exchange efficiency. Beneficial effects have been documented in severe asthma attacks and in patients with chronic obstructive pulmonary disease. A reduction in WOB during mechanical ventilation and an increase in exercise endurance capacity have also been described in COPD. Heliox has been also used in the treatment of upper airways obstruction, bronchiolitis and bronchopulmonary dysplasia. Despite the encouraging results, Heliox use in routine practice remains controversial because of technical implications and high costs.