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Rationale: The use of long-term noninvasive respiratory support is increasing in children along with an extension of indications, in particular in children with central nervous system (CNS) disorders., Objective: The aim of this study was to describe the characteristics of children with CNS disorders treated with long-term noninvasive respiratory support in France., Methods: Data were collected from 27 French pediatric university centers through an anonymous questionnaire filled for every child treated with noninvasive ventilatory support ≥3 months on 1st June 2019., Main Results: The data of 182 patients (55% boys, median age: 10.2 [5.4;14.8] years old [range: 0.3-25]) were collected: 35 (19%) patients had nontumoral spinal cord injury, 22 (12%) CNS tumors, 63 (35%) multiple disabilities, 26 (14%) central alveolar hypoventilation and 36 (20%) other CNS disorders. Seventy five percent of the patients were treated with noninvasive ventilation (NIV) and 25% with continuous positive airway pressure (CPAP). The main investigations performed before CPAP/NIV initiation were nocturnal gas exchange recordings, alone or coupled with poly(somno)graphy (in 29% and 34% of the patients, respectively). CPAP/NIV was started in an acute setting in 10% of the patients. Median adherence was 8 [6;10] hours/night, with 12% of patients using treatment <4 h/day. Nasal mask was the most common interface (70%). Airway clearance techniques were used by 31% of patients., Conclusion: CPAP/NIV may be a therapeutic option in children with CNS disorders. Future studies should assess treatment efficacy and patient reported outcome measures., (© 2023 Wiley Periodicals LLC.)

Serum amyloid A (SAA) is a good systemic marker of the exacerbations of chronic obstructive pulmonary disease (COPD), but the significance of SAA in stable patients with COPD has not been widely investigated. We aimed to evaluate the SAA level in peripheral blood from stable patients with COPD and to search for correlations between SAA and other inflammatory markers and clinical characteristics of the disease. Serum SAA, IL-6, IL-8, TNF-alpha, basic blood investigations, pulmonary function testing and a 6-min walk test were performed. The correlations between SAA and other inflammatory markers, functional performance and the number of disease exacerbations were evaluated. A total of 100 consecutive patients with COPD were analyzed. No correlations between SAA and inflammatory markers as well as pulmonary function were found. Hierarchical clustering identified two clusters incorporating SAA: one comprised SAA, PaO 2 and FEV 1 and the second was formed of SAA and nine other disease markers. The SAA level was higher in patients with blood eosinophils < 2% when compared to those with blood eosinophils ≥ 2% (41.8 (19.5-69.7) ng/mL vs. 18.9 (1.0-54.5) ng/mL, respectively, p = 0.04). We conclude that, in combination with other important disease features, SAA may be useful for patient evaluation in stable COPD.

The aim of the study was to describe the characteristics of children with neuromuscular diseases treated with long term noninvasive ventilation or continuous positive airway pressure in France. On June 1st 2019, 387 patients (63% boys, mean age 11.2 ± 5.5 years) were treated with long term noninvasive ventilation/continuous positive airway pressure. Thirty three percent of patients had spinal muscular atrophy, 30% congenital myopathy/dystrophy, 20% Duchenne muscular dystrophy, 7% Steinert myotonic dystrophy, and 9% other neuromuscular diseases. Ninety-four percent of patients were treated with long term noninvasive ventilation and 6% with continuous positive airway pressure. Treatment was initiated electively for 85% of patients, mainly on an abnormal overnight gas exchange recording (38% of patients). Noninvasive ventilation/continuous positive airway pressure was initiated during a respiratory exacerbation in 15% of patients. Mean duration of noninvasive ventilation/continuous positive airway pressure was 3.3 ± 3.1 years. Mean objective long term noninvasive ventilation/continuous positive airway pressure use was 8.0 ± 3.1 h/24. Spinal muscular atrophy, congenital myopathy/dystrophy, and Duchenne muscular dystrophy represented 83% of children with neuromuscular diseases treated with long term noninvasive ventilation in France. Screening for nocturnal hypoventilation was satisfactory as noninvasive ventilation /continuous positive airway pressure was predominantly initiated electively., Competing Interests: Declaration of Competing Interest None., (Copyright © 2022. Published by Elsevier B.V.)

Background: Obstructive sleep apnea is associated with an increased prevalence of cardiovascular disease. The mechanism of these associations is not completely understood. We aimed to investigate the association of the apnea hypopnea index and the degree of airflow limitation with endothelial dysfunction. Methods: This was a single-center prospective study of patients admitted for diagnostic coronary angiography (CAG). Endothelial function was assessed by the non-invasive EndoPAT system by reactive hyperemia index (RHI) and divided into two groups: endothelial dysfunction and normal endothelial function. Sleep apnea signs were detected by WatchPAT measuring the respiratory disturbance index (pRDI), the apnea and hypopnea index (pAHI), and the oxygen desaturation index (ODI). Patients underwent spirometry and body plethysmography. Based on CAG, the severity of coronary artery disease was assessed as follows: no significant coronary artery disease, single-, two- and three-vessel disease. Results: A total of 113 patients were included in the study. Breathing disorders measured by WatchPAT and spirometry were more severe in patients with endothelial dysfunction: pRDI (27.3 vs. 14.8, p = 0.001), pAHI (24.6 vs. 10.3, p < 0.001), ODI (13.7 vs. 5.2, p = 0.002), forced expiratory volume in one second (FEV1) (81.2 vs. 89, p = 0.05). In a multivariate regression analysis, pAHI and FEV1 were independent predictors of endothelial dysfunction assessed by RHI. There was no correlation between the severity of coronary artery disease and endothelial dysfunction. Conclusions: Obstructive sleep apnea signs and greater airflow limitation were associated with endothelial dysfunction regardless of the severity of the coronary artery disease.

Objective: To describe the characteristics of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) in France., Design: Cross-sectional national survey., Setting: Paediatric CPAP/NIV teams of 28 tertiary university hospitals in France., Patients: Children aged <20 years treated with CPAP/NIV since at least 3 months on June 1st, 2019., Intervention: An anonymous questionnaire was filled in for every patient., Results: The data of 1447 patients (60% boys), mean age 9.8 ± 5.8 years were analysed. The most frequent underlying disorders were: upper airway obstruction (46%), neuromuscular disease (28%), disorder of the central nervous system (13%), cardiorespiratory disorder (7%), and congenital bone disease (4%). Forty-five percent of the patients were treated with CPAP and 55% with NIV. Treatment was initiated electively for 92% of children, while 8% started during an acute illness. A poly(somno)graphy (P(S)G) was performed prior to treatment initiation in 26%, 36% had a P(S)G with transcutaneous carbon dioxide monitoring (PtcCO 2 ), while 23% had only a pulse oximetry (SpO 2 ) with PtcCO 2 recording. The decision of CPAP/NIV initiation during an elective setting was based on the apnea-hypopnea index (AHI) in 41% of patients, SpO 2 and PtcCO 2 in 25% of patients, and AHI with PtcCO 2 in 25% of patients. Objective adherence was excellent with a mean use of 7.6 ± 3.2 h/night. Duration of CPAP/NIV was 2.7 ± 2.9 years at the time of the survey., Conclusion: This survey shows the large number of children treated with long term CPAP/NIV in France with numerous children having disorders other than neuromuscular diseases., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Mandibuloacral dysplasia syndromes are mainly due to recessive LMNA or ZMPSTE24 mutations, with cardinal nuclear morphological abnormalities and dysfunction. We report five homozygous null mutations in MTX2, encoding Metaxin-2 (MTX2), an outer mitochondrial membrane protein, in patients presenting with a severe laminopathy-like mandibuloacral dysplasia characterized by growth retardation, bone resorption, arterial calcification, renal glomerulosclerosis and severe hypertension. Loss of MTX2 in patients' primary fibroblasts leads to loss of Metaxin-1 (MTX1) and mitochondrial dysfunction, including network fragmentation and oxidative phosphorylation impairment. Furthermore, patients' fibroblasts are resistant to induced apoptosis, leading to increased cell senescence and mitophagy and reduced proliferation. Interestingly, secondary nuclear morphological defects are observed in both MTX2-mutant fibroblasts and mtx-2-depleted C. elegans. We thus report the identification of a severe premature aging syndrome revealing an unsuspected link between mitochondrial composition and function and nuclear morphology, establishing a pathophysiological link with premature aging laminopathies and likely explaining common clinical features.

Aim: The aims of the study are to evaluate the impact of a 4% chlorhexidine (CHG4%) bathing on the occurrence of central-line-associated bloodstream infection (CLABSI) and to identify risk factors (RFs) for CLABSI in our population. This is a retrospective monocentric cohort study in the paediatric surgical intensive care unit at the Necker Enfants Malades Hospital, Paris, France., Methods: All hospitalised patients with central venous catheters (CVCs) in 2015 were included. CHG4% bathing was prescribed in CLABSI high-risk patients, defined by the presence of exposition factors (EFs): constitutive or acquired immunosuppression, presence of an invasive medical device (IMD) and the carriage of Staphylococcus aureus. The overall 2015 CLABSI incidence rate was compared with 2014 CLABSI incidence rate (before CHG4% bathing)., Results: In all, 775 patients were analysed. Some 182 had at least one EF, and 49 received CHG4%. The incidence rates of CLABSI in 2014 and 2015 were, respectively, 6.1 and 2.3/1000 days CVC (P < 0.01). The presence of at least one EF was associated with the CLABSI's occurrence: odds ratio = 15.13 (95% confidence interval: 4.26-53.71; P < 0.0001), particularly acquired immunosuppression, IMD and S. aureus colonisation. Other RFs were age <1 year and carrying duration >16 days., Conclusions: This study showed a significant reduction in incidence of CLABSI after introduction of a targeted CHG4% bathing protocol. Presence of IMD, S. aureus colonisation, immunosuppression, age <1 year and carrying duration >16 days were CLABSI RFs. Regarding the literature, the presence of IMD seems to be underestimated in CLABSI prevention., (© 2020 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Background: A significant percentage of patients with asthma and COPD do not use their inhalers properly. The aim of this study was to evaluate the impact of a single session of inhalation technique training on the frequency of inhalation errors and the course of asthma and COPD., Methods: This randomized controlled trial included adults who had been diagnosed with and treated for asthma or COPD with at least one inhaler use daily on a regular basis. All subjects were followed for 6 months, at which time their inhalation technique was assessed and an individual inhalation training (study group) or a sham procedure (control group) was applied. The number of mishandlings was calculated as the ratio of the number of errors to the number of inhalers used by an individual subject. The effect of training was evaluated after 3 months and again after 6 months., Results: 50 subjects with asthma and 50 subjects with COPD were enrolled. Only 20% of subjects made no critical errors before the intervention. Subjects who were trained in the proper inhalation technique made fewer errors after 3 months (32 of 50 vs 20 of 50). The relative risk was 1.63 (95% CI 1.1-2.4, P = .01) and the number needed to treat was 3.9 (95% CI 2.2-15). Despite the improvement in the inhalation technique, we found no reduction in the number of asthma/COPD exacerbations, symptom severity, or the quality of life. After 3 consecutive months, the efficacy of the intervention decreased, and only 66% of the former responders maintained the lower ratio of errors per inhaler. After 6 months, there was no difference in the number of subjects with better inhalation technique between intervention (24 of 50) and control group (27 of 50) ( P = .62)., Conclusions: Although a single inhalation training leads to a reduction in the number of errors made during inhalation, it does not influence the course of asthma and COPD. The positive effect of a single inhalation technique training is temporary. (ClinicalTrials.gov registration NCT02131454.)., Competing Interests: The authors have disclosed no conflicts of interest., (Copyright © 2019 by Daedalus Enterprises.)

Background: Testosterone has been recognized for its anabolic properties. It has been documented that in patients with chronic obstructive pulmonary disease (COPD), chronic hypoxia, disease severity, smoking, and corticosteroid treatment may contribute to low testosterone levels., Objectives: The aim of the study was to evaluate the incidence of decreased serum testosterone concentration in male COPD patients and its influence on their condition., Material and Methods: The study group consisted of 90 male patients, aged 67.2 ±8.8 years in all stages of airflow limitation severity (mild n = 6, moderate n = 43, severe n = 28 and very severe n = 13) Serum testosterone concentration was evaluated using ELISA method (Testosterone ELISE LDN). Decreased serum testosterone level was defined as a value of less than 3 ng/mL. Testosterone levels were related clinical features of COPD., Results: Serum testosterone concentration did not differ in patients with different stages of airflow limitation severity (3.8 ±0.7 ng/mL for mild: 3.6 ±2.1 ng/mL for moderate; 3.4 ±1.2 ng/mL for severe and 3.7 ±1.7 ng/mL for very severe, respectively). Decreased serum testosterone was found in 30 patients (group A). There were no differences in age, the number of exacerbations or CRP concentration between patients with decreased and the normal serum testosterone group (group B). Group A was characterized by a lower FEV1, shorter 6-minute walking distance, longer smoking history and higher BMI, but no differences in body composition and densitometry results were found., Conclusions: Serum testosterone depression may occur in as much as 30% of male COPD patients in all COPD stages of severity. The relationship between serum testosterone and negative COPD prognostic factors indicates its influence on the natural history of the disease.

Background: Chronic obstructive pulmonary disease (COPD) is associated with various comorbidities, which influence the course of COPD and worsen prognosis., Objectives: The aim of this study was to analyze the comorbidities in a cohort of COPD patients in Poland during 12 months of observation., Material and Methods: A total of 444 COPD patients (median age: 66.1 years) in all stages of airflow limitation severity were enrolled. Medical histories and a questionnaire concerning comorbidities were analyzed at baseline and after 12 months (data of 267 patients available). Anthropometric data, pulmonary function, and body mass index, airflow obstruction, dyspnea, and exercise capacity (BODE index) were assessed., Results: No comorbidities were reported in 9 patients (2.0%), 101 patients (22.7%) had 1-2 comorbidities, 243 (54.7%) had 3-5, and 91 (20.6%) had more than 5 comorbidities. Cardiovascular diseases (CVDs) were the most frequent ones, followed by peptic ulcer, obstructive sleep apnea (OSA), diabetes, gastroesophageal reflux disease (GERD), and osteoporosis; 11 patients had a history of lung cancer. Cachexia was observed in 11 cases, overweight in 136 cases and obesity in 139 cases. The incidence of CVDs increased with time. The number of comorbidities correlated with the body mass index (BMI) and the number of hospitalizations for extra-pulmonary causes, but not with airflow limitation. The BODE index score increased with the number of comorbidities., Conclusions: In a cohort of Polish COPD patients, the most frequent comorbidities were CVDs. The number of comorbidities affected the BODE index, but not airflow limitation. The BODE index is better than forced expiratory volume in 1 s (FEV1) in the rating of COPD patients' condition. The BMI correlated with the number of comorbidities as well as the number of hospitalizations for extra-pulmonary causes.

The prevalence of chronic obstructive pulmonary disease (COPD) has increased more rapidly in women than in men during the past two decades. Clinical presentation, comorbidities and prognosis may differ between genders and may influence management decisions. The influence of gender on COPD expression has not been clearly explained to date. Thus, the aim of this study was to evaluate significant differences between women and men suffering from COPD, regarding clinical presentation, pulmonary function test results, comorbidities, and prognosis. We prospectively recruited 470 patients with stable COPD with a history of smoking (152 women, 318 men, mean age 65.5 ± 8.8 vs. 66.6 ± 9.4 years, respectively). Comorbidities and exacerbations were recorded. Spirometry, body plethysmography, carbon monoxide diffusing capacity and 6-min walk tests were performed. The BODE prognostic score was also calculated. We found that women smoked less in comparison to men (30.4 vs. 41.9 pack-years, p < 0.05), showed more exacerbations (2.5 vs. 1.7, p = 0.01), higher forced expiratory volume in 1 s (FEV1%predicted), and increased residual volume/total lung capacity (RV/%TLC), but they had the same intensity of dyspnea. Women showed fewer comorbidities, on average, per patient (5.4 vs. 6.4, p = 0.002), but had a higher prevalence of at least seven comorbidities per patient (48.7% of women vs. 33.0% of men, p < 0.05). Women also had a significantly worse prognosis (4.6 vs. 3.1 BODE score, p < 0.05) that correlated with the number of comorbidities (r = 0.33, p < 0.01). In conclusion, this study strongly supports the existence of different gender phenotypes in COPD, especially regarding exacerbations, comorbidities, and prognosis. The gender difference may indicate a need for a targeted assessment and management of COPD in women and men.

Introduction: Chlamydia pneumoniae is an obligatory human pathogen involved in lower and upper airway infections, including pneumonia, bronchitis. Asymptomatic C. pneumoniae carriage is also relatively common. The association of C. pneumoniae infections with the chronic obstructive pulmonary disease (COPD) course is unclear., Objectives: The aim of the study was to investigate the association between chronic C. pneumoniae infection and clinical features of COPD, markers of inflammation and metabolic dysfunction., Patients and Methods: The study included 59 patients with stable COPD who had no, or had ≥2 acute exacerbations during last year. The level of IgA and IgG antibody against C. pneumoniae, IL-6, IL-8, resistin, insulin, adiponectin and acyl ghrelin was measured in serum by enzyme-linked immunosorbent assay (ELISA)., Results: No differences in clinical and functional data were observed between COPD patients without serological features of C. pneumoniae infection and chronic C. pneumoniae infection. The level of anti C. pneumoniae IgA significantly correlated with IL-8, IL-6, resistin concentration in group of frequent exacerbators. IgG level correlated negatively with acetyl ghrelin and body mass index (BMI) in patients without frequent exacerbations, in contrast to frequent COPD exacerbation group where significant correlations between IgG level and BMI was demonstrated. Serum IL-6 correlated positively with resistin and insulin and negatively with adiponectin in group of patients with serological features of chronic C. pneumoniae infection only., Conclusions: Our study showed that chronic C. pneumoniae infection does not influence the clinical course of COPD in the both study groups. Chronic C. pneumoniae infections might be associated with a distinct COPD phenotype that affects metabolic dysfunction.

Benign metastasising leiomyoma (BML) is characterised by extrauterine smooth muscle tumours in women after surgical treatment for uterine leiomyoma. Usually manifested as solitary or multiple focal lesions in various organs, it imposes a scrutinous diagnostic work-up to exclude a malignant disease and requires confirmation in microscopic examination of the extrauterine focus. The authors present a case of a 56-year-old woman with BML manifesting as bilateral multiple pulmonary nodules, with a tentative diagnosis of a disseminated malignant disease of mesenchymal origin. The patient underwent multiple diagnostic tests, which excluded malignancy. The definite diagnosis was established after the microscopic reevaluation of an excised pulmonary nodule. The patient is monitored with chest magnetic resonance. BML should be considered in the differential diagnosis of multiple pulmonary nodules in asymptomatic women. Patients with BML require long-term monitoring, therefore the selected imaging method should not carry the risk of cumulative side effects.

Body composition disorders are observed in chronic obstructive pulmonary disease (COPD) patients and have a significant impact the general condition and outcome in this disease., Aim: The aim of the study was to assess body composition in relation to airway obstruction severity in patients with COPD., Materials and Methods: The study group consisted of 143 patients (58F, 85M) in the middle age The group was divided to two groups: group A FEV1<50% predicted and group B FEV1 ≥50% predicted; both groups were compared. The following anthropometric parameters were assessed: body mass index (BMI), waist/hip ratio and shoulder circumference of the dominant upper limb. Body composition analysis was performed by bioimpedance (Tanita T5896, TANITA Corporation of America, Inc, Arlington Heights, USA)., Results: The mean BMI for the whole group was 27.5±5.1 kg/m2. None of the patients was underweight, 47 (32.9%) had normal BMI, 55 (38.5%) overweight and 41 (28.6%) were obese. Patients in group A had lower BMI, FFMI and muscle mass index (MMI) than patients in group B. We found the correlation between BMI, FFMI, MMI and FEV1 in the studied group., Conclusions: Our results confirm the relationship between airflow limitation and body compositions in COPD patients. We suggest that anthropometric measurements should be a part routine COPD management.

Introduction: The role of bacterial infections in acute exacerbations of chronic obstructive pulmonary disease (COPD) is widely examined. Denture plaque in patients with COPD is an example of bacterial and fungal biofilm, which is a reservoir of potentially pathogenic respiratory tract microorganisms. Poor denture hygiene might cause acute exacerbations of COPD., Objective: Assessment of prevalence of respiratory tract pathogens in denture plaque in stable patients with COPD and it influence on oral ontocenoses depending upon the therapy., Materials and Methods: The study was based on the clinical assessment of oral mucosa and denture hygiene in 53 patients with COPD with mean age of 70 ± 18 years and 14 generally healthy participants with mean age of 65 ± 14 years. Microbiological and mycological tests were performed by culturing direct denture swabs., Results: The study showcased the presence of potential pathogenic micro-organisms in denture plaque of 48 patients with COPD (90%) and nine healthy subjects (64.3%). Yeast-like fungi prevailed in denture surface swabs of 40 (75%) in patients with COPD and 8 (57%) in cases of control group. In 66% of patients, various degree of oral mucosa inflammation prevailed., Conclusions: Denture plaque could be a potential source of bacterial and fungal infections in patients with COPD., (© 2014 John Wiley & Sons A/S and The Gerodontology Association. Published by John Wiley & Sons Ltd.)

Introduction: Chronic obstructive pulmonary disease (COPD) affects more than 10% of the world's population over 40 years of age. The main exogenous risk factor is cigarette smoking; however, only 20% of smokers develop COPD, indicating that some other factors, e.g. genetic, may play an important role in the disease pathogenesis. Recent research indicates that ACE (angiotensin-converting enzyme) may be a susceptibility gene for asthma or COPD. The aim of our study was to determine the influence of I/D (insertion/deletion) polymorphism of the ACE gene (AluYa5, rs4646994) on the risk and course of COPD., Material and Methods: We investigated ACE I/D polymorphism in 206 COPD and 165 healthy Caucasian subjects., Results: In the generalized linear model (GLZ) analysis of the influence of selected factors on presence of COPD we found a significant independent effect for male sex (repeatedly increases the risk of COPD, OR = 7.7, p = 0.049), as well as smoking or lower body mass index, but only in combination with older age (OR = 0.96, p = 0.003 and OR = 1.005, p = 0.04 respectively). Interestingly, analysis of factors which may influence the risk of a higher number of exacerbations demonstrated that occurrence of DD genotype, but only in men, is associated with a lower risk (OR = 0.7, p = 0.03) of this complication., Conclusions: We suggest that ACE may not be a susceptibility gene for the origin of COPD but a disease-modifying gene. Since the impact of I/D polymorphism of the ACE gene on COPD risk is moderate or negligible, other molecular changes, that will help predict the development of this disease, should still be sought.

Asthma is a heterogeneous inflammatory disease. Most patients respond to current standard of care, i.e., bronchodilators, inhaled glucocorticosteroids and other anti-inflammatory drugs, but in some adequate asthma control cannot be achieved with standard treatments. These difficult-to-treat patients would be the target population for new biological therapies. At present, omalizumab is the only biological agent approved for the treatment of early-onset, severe IgE-dependent asthma. It is safe, effective, and well tolerated. Also, discovery of asthma subtypes suggests new treatments. Half of patients with severe asthma have T-helper type 2 (Th-2) inflammation and they are expected to benefit from monoclonal antibody-based treatments. The efficacy of the investigational monoclonal antibody mepolizumab which targets IL-5 has been well documented in late onset non-atopic asthma with persistent eosinophilic airway inflammation. Anti-IL-4 and IL-13 agents (dupilumab, lebrikizumab, and tralokinumab) which block different Th-2 inflammatory pathways and agents targeting the Th-17 inflammatory pathway in severe refractory asthma are under development. In clinical trials, these drugs reduce disease activity and improve lung function, asthma symptoms, and quality of life. However, studies on larger groups of patients are needed to confirm their safety and efficacy.

Introduction: Taking into account important role of apoptosis in COPD pathogenesis, we wanted to asses the serum levels of markers involved in apoptosis regulation, including apoptosis inducers such as TNF-a, sFasL or p53 protein and apoptosis inhibitor bcl-2 and, in addition, to compare these markers with selected COPD parameters., Material and Methods: In 181 patients (60 women) with COPD (age was 62.2+ 9.37 years; FEV1% 55.2 + 19.98 %) and in 29 controls (11 women), serum levels of TNF-a, sFasL, p53 and bcl-2 were evaluated by the enzyme-linked immunosorbent assay (ELISA) method., Results: In COPD patients the mean sFasL level was 0.092 ± 0.077 ng/ml and mean TNF-a level was 2.911 ± 3.239 pg/ml. There were no differences in serum sFasL and TNF-a in COPD patients and control group. TNF-a and sFasL did not correlate with COPD parameters such as FEV₁%, BMI, RV% (percentage of predicted value of residual volume) or BODE. Although we tried to evaluate bcl-2 and p53 protein serum levels with two different tests, measurable levels of bcl-2 were only detected in 15 patients and p53 in only 3 patients. Bcl-2 values were from 0.418 to 11.423 ng/ml and p53 from 90.772 to 994.749 pg/ml., Conclusions: We didn't observe any differences in serum levels of pro- and antiapoptotic markers in COPD patients and the control group or correlations between the markers studied and COPD parameters.

Unlabelled: Chronic obstructive pulmonary disease (COPD) is a significant clinical problem wich is dependent on many environmental factors., The Aim: of the study was to present a characteristic of examined group in the moment of including into the project., Materials and Methods: Based on data obtained from studies conducted in six medical universities in Poland we present the characteristics of 445 patients (M-69%), median age 66.2 years, suffering from COPD with median disease duration 7.7 years. The analysis included: age, education, risk factors, exacerbations and hospitalizations, comorbidities, severity of the disease, drug use and the results of selected tests and the quality of life of patients in relation with their place of residence., Results: Some differences were found among the participating centers. The youngest patients came from Wrocław and the oldest from Katowice. The largest number of patients with higher education were from Warsaw, while the lowest number was noted in Poznań; patients with primary education were most numerous in Lublin. Patients from Warsaw had the highest number of pack-years, smoking history was least relevant in patients from Wrocław. The highest values of spirometrical parameters were observed in Gdańsk, while the lowest--in Poznań. COPD treatment mainly comprised of long-acting beta2 agonists, followed by anticholinergic agents, more than 50% of patients were treated with inhaled glucocorticosteroids. The most common comorbidities were cardiovascular diseases., Conclusions: The study group showed characteristics similar to those of other cohorts of patients with COPD described in the literature, but we found some differences between patients from different centers which participated in the study., (© 2015 MEDPRESS.)

Introduction: Body composition is an important prognostic factor in patients with COPD. The decrease in fat free mass (FFM), muscle mass (MM) and increase in visceral fat is associated with an elevated secretion of cytokines which promote systemic inflammation. The aim of the study was to evaluate body composition and the cytokine profile in patients with COPD in relation with the presence of hyperinflation., Material and Methods: The study group consisted of 149 patients (61F, 88M) with stable COPD in all stages of severity aged 68 ± 8.8 yrs. All the patients underwent spirometry and bodypletysmography with bronchial reversibility testing. Hyperinflation was defined as RV%TLC > 48% and > 126% predicted. Body composition was analyzed by bioimpedance. The following serum inflammatory markers were evaluated: C-reactive protein, IL-6, IL-8, TNF-a, CC16, adiponectin and resistin., Results: Hyperinflation was found in 96 patients (group A) and it was more frequent in women than men (49/61 vs. 47/88, p < 0.001). BMI and age in this group were comparable to those in patients without hyperinflation (group B). Patients with hyperinflation have lover FFM, FFM index, MM and MM index and total body water and higher fat mass and fat mass index. We found significantly higher serum concentrations of inflammatory markers in group A: IL-6 - 6.4 ± 10.9 vs. 3.6 ± 4.2 pg/ml, resistin - 9.3 ± 4.2 vs. 7.6 ± 2.4 ng/ml, CRP 4.1 ± 2.3 vs. 2.9±2.1 mg/l, respectively., Conclusions: Patients with hyperinflation have a lower FFMI, TBW and MMI and a higher proportion of fat tissue. Hyperinflation is associated with elevated concentrations of inflammatory markers what may be associated with more severe disease. Body compositions abnormality and higher activity of systemic inflammation could therefore be a negative prognostic factor in COPD patients.

Unlabelled: In the course of various diseases, there is an accumulation of fluid in the pleural cavities. Pleural fluid accumulation causes thoracic volume expansion and reduction of volume lungs, leading to formation of restrictive disorders. The aim of the study was to estimate the volume of pleural fluid by ultrasonography and to search for the relationship between pleural fluid volume and spirometrical parameters., Material and Methods: The study involved 46 patients (26 men, 20 women) aged 65.7 +/- 14 years with pleural effusions who underwent thoracentesis. Thoracentesis was preceded by ultrasonography of the pleura, spirometry test and plethysmography. The volume of the pleural fluid was calculated with the Goecke' and Schwerk' (GS) or Padykuła (P) equations., Results: The obtained values were compared with the actual evacuated volume. The median volume of the removed pleural fluid was 950 ml. Both underestimated the evacuated volume (the median volume 539 ml for GS and 648 ml for P, respectively). Pleural fluid removal resulted in a statistically significant improvement in VC (increase 0.20 +/- 0.35 ; p < 0.05), FEV1 (increase 0.16 +/- 0.32 l; p < 0.05), TLC (increase 0.30 +/- 0.58 l; p < 0.05) and PEF (0.37 +/- 1 l/s; p < 0.05) CONCLUSIONS: Pleural fluid removal causes a significant improvement in lung function parameters. The analyzed equations for fluid volume calculation do not correlate with the actual volume.

Unlabelled: INTRODUCTION Exacerbations affect the natural history of chronic obstructive pulmonary disease (COPD)., Objectives: The aim of the study was to evaluate the effect of exacerbation frequency in COPD on body composition and systemic inflammation assessed by the measurement of serum adiponectin and ghrelin concentrations., Patients and Methods: The study group included 152 patients with COPD. Body composition was assessed by bioimpedance. Fasting serum adiponectin and ghrelin concentrations were evaluated by enzyme-linked immunosorbent assays., Results: Of the 152 patients, 60 did not report any exacerbation in the last 12 months, 53 had 1 exacerbation, and 39 had more than 1 exacerbation. The mean number of exacerbations in the whole group was 1.04 ±1.3 per patient per year and increased with the increasing degree of airway obstruction. Patients with exacerbations had a lower fat-free mass (FFM) index, lower total body water, and lower resting metabolic rate compared with patients without exacerbations (18.3 ±2.5 kg/m2 vs. 19.3 ±2.7 kg/m2; 36.9 ±8.1 kg vs. 40.2 ±8.0 kg; and 1482.8 ±301.1 kcal vs. 1616.0 ±322.0 kcal; respectively). The adiponectin concentration was higher in patients with more than 1 exacerbation than in those with 1 or no exacerbations (14.5 ±8.6 mg/l vs. 11.2 ±7.6 mg/l, P <0.05). We observed significant negative correlations between the number of exacerbations and the muscle mass index, FFM index, total body water, resting metabolic rate, and adiponectin concentration in the whole group., Conclusions: Exacerbations affect body composition in patients with COPD. Patients with frequent exacerbations have more enhanced systemic inflammation. Assessment of the body composition and systemic inflammation should be part of the routine management of patients with COPD.

Unlabelled: The GOLD 2011 recommendations for chronic obstructive pulmonary disease (COPD) introduce a new classification system to optimize treatment in individual patients. Except for FEV,, this classification incorporates breathlessness measurement using modified medical research council questionnaire (mMRC) or the COPD assessment Test (CAT) and the number of exacerbations. The aim of our study was to compare the GOLD 2010 and GOLD 2011 COPD., Material and Methods: The study group consisted of 143 patients. Based on the post-bronchodilator FEV, only, as recommended in the GOLD 2010 report, there were 24 patients in stage I, 57patients in II, 43 in Ill and 19 in IV, respectively. In all patients, the number of exacerbations per year was noted and dyspnea was assessed with the modified MRC scale. The patients were subsequently graded to group A,B,C,D as proposed in the combined COPD assessment in GOLD 2011., Results: Grading of 51 (35,7%) patients according to the GOLD 2011 criteria was difficult; there were 22 patients in GOLD stage I/II with > or =2 exacerbations per year and 29 patients in GOLD stage Ill/IV with < 2 exacerbations per year. They were grading to more risk group., Conclusion: The new classification according to GOLD 2011 lets on optimizations of the treatment, in most cases of COPD patients but in clinical practice, there may be problems with the classification of the patients with severe airway obstruction without frequent exacerbations and especially those with mild/moderate airflow limitation and frequent exacerbations.

Introduction: Cardiovascular diseases often coexist with chronic obstructive pulmonary disease (COPD), and in some cases it is difficult to differentiate between cardiac and pulmonary cause of dyspnoea. It is well known that the serum concentration of NT-proBNP in patients with cardiovascular diseases, especially with congestive heart failure, is elevated. The aim of this study was to estimate the usefulness of NT-proBNP serum level measurement in patients with COPD complaining of chronic dyspnoea., Material and Methods: The study group consisted of 81 stable COPD patients in middle age, 65 ± 7 years, (57 of them with concomitant cardiovascular disease). Serum concentration of NT-proBNP was measured using VITROS laboratory test., Results: There were no statistical differences in serum NT-proBNP between patients stratified according to the GOLD staging system for COPD severity or BODE index and mMRC breathlessness scale. The concentration of NT-proBNP was statistically significantly higher in the patients with coexisting cardiovascular diseases (220.8 ± 258.1 vs. 95.4±56.1 pg/ml). The group of patients with NT-proBNP concentration 〉 125 pg/ml (n = 36) was statistically significantly older (67.5 ± 6 years old vs. 63.2 ± 7.1 years old; p = 0.009) and had statistically significantly lower PaO2 (67.4 ± 11.8 mm Hg vs. 73.0 ± 11.6 mm Hg; p = 0.04)., Conclusions: 1. In the group of stable COPD patients there were no differences between NT-proBNP serum concentration according to GOLD staging, BODE index, and mMRC breathlessness scale. 2. The NT-proBNP serum concentration was statistically significantly higher in the group of COPD patients with the concomitant cardiovascular disease. 3. In patients with chronic dyspnoea testing of serum NT-proBNP may be useful in the detection of patients with cardiovascular problems, who require more intensive therapy.

Background: Asthma and COPD are non-infectious inflammatory diseases of the respiratory tract. Allergic rhinitis can be assumed as an intermediate condition between healthy and asthmatic state. Eotaxins are important indicators of allergic reaction. They are strong chemoattractants mainly for eosinophils but also for other cells., Objective: We measured the level of eotaxin expression and inflammatory cell count in the material from nasal brushing in healthy controls and in patients with allergic rhinitis, asthma, and COPD. We studied the correlation between the eotaxin gene expression level in the material from nasal brushing and respiratory tests in asthma and COPD patients., Methods: Expression of eotaxins was measured using quantitative RT-PCR. Number of eotaxin transcript copies was evaluated using real time PCR standard curve method., Results: Of all eotaxins CCL24 had the highest expression in the material from nasal brushing, and its level was increased in allergic asthma. CCL11 was significantly increased in the material from nasal brushing of COPD patients. Increased levels of all three eotaxins were observed in the material from nasal brushing of patients with allergic rhinitis in season. The levels of CCL26 expression and FEV1/FVC factor were correlated negatively in the asthma group and positively in the COPD group., Conclusions: Eotaxins are crucial factors of allergic, asthmatic and also COPD inflammatory reactions. Our results suggest a dual role of CCL26 - it can act as a negative regulator for neutrophils in COPD, while in asthma it may act as a chemoatractant of eosinophils and other cells into the lung., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Introduction: Chronic obstructive pulmonary disease (COPD) is a fourth cause of death in USA and is expected to be the third cause of death by year 2020. It is believed that some diseases like cardiovascular diseases, osteoporosis, cachexia, anaemia are systemic consequences of COPD. Some data show that the co-morbidities are more frequent in severe COPD patients than in control groups and are associated with the serious health outcomes. It is debated whether extra pulmonary comorbidities or pulmonary complications are the main reason of death in COPD. The aim of the study was to analyze the mortality and comorbidity in COPD patients who died during hospitalization in the Department of Pneumonology and Allergology in WMU from 2004 to 2008., Material and Methods: We analyzed 266 records of patients who had diagnosis of COPD and died during hospitalization (179 [67%] M and 87 [33%] F). The mean age at death was 73 ± 8 years (M 73 ± 8 years, F 74 ± 9 years)., Results: The most frequent cause of death were: COPD exacerbation (n = 81 [30%], F = 32, M = 49), pneumonia (n = 67 [25%], F = 17, M = 50), lung cancer (n = 50 [19%], F = 18, M = 32), ischemic heart disease (n = 20 [7%], F = 5, M = 15), cardiovascular insufficiency (n = 14 [5%], F = 6, M = 8) and other (n = 34 [14%]). The lung cancer was more frequent cause of death in younger patients (p = 0.002), and pneumonia in older (p = 0.02). In COPD patients the following comorbidities were diagnosed: chronic heart failure (n = 169), hypertension (n = 103), ischaemic heart disease (n = 102), diabetes mellitus type 2 (n = 55), renal insufficiency (n = 43), hypertrophy of prostate (n = 36), arteriosclerosis obliterans (n = 28), osteoporosis (n = 19), anaemia (n = 14) and others., Conclusions: 1. The most frequent reasons of death were related to respiratory system: COPD exacerbation, pneumonia and lung cancer. 2. The most frequent diseases coexisting with COPD were cardiovascular disease, respiratory insufficiency and diabetes mellitus.

Introduction: In OSAS patients CPAP therapy decreases cardiovascular morbidity and mortality. Homocysteine and leptin may play a role in development of ischaemic heart disease (IHD) in patients with OSAS. The aim of the study was to assess the influence of 3 month CPAP therapy on cardiovascular risk factors in patients with OSAS without IHD (pure OSAS) and with OSAS and IHD., Material and Methods: Therapy with CPAP was started in 42 OSAS without IHD (pure OSAS) and 23 OSAS and IHD patients. Plasma concentration of homocysteine, serum concentration of leptin, C-reactive protein (CRP), fibrinogen, lipids, and markers of visceral adiposity (MVA) were measured before and after treatment., Results: There were no significant changes in homocysteine, leptin, fibrinogen and CRP concentrations in neither group. In OSAS and IHD no change in serum lipids and MVA were found. In pure OSAS group total cholesterol and LDL cholesterol concentrations significantly decreased (202.5 ± 38.5 mg/dl v. 186.7 ± 33.5 mg/dl, p = 0.001 and 127.3 ± 32.9 mg/dl v. 116.4 ± 26.9 mg/dl, p = 0.02, respectively). Triglycerides did not significantly change (p = 0.09). There were no significant changes in BMI (30.4 ± 3.8 v. 30.6 ± 3.6, p = 0.5), waist circumference (108.5 ± 8.0 cm v. 107.0 ± 7.5 cm, p = 0.09) and waist to hip ratio (1.03 ± 0.04 v. 1.01 ± 0.03, p = 0.07)., Conclusions: Three month CPAP therapy did not change homocysteine and leptin concentration in neither group. However, it significantly decreased serum lipids concentration in patients with pure OSAS, but not in patients with OSAS and IHD, suggesting beneficial effects of CPAP therapy on cardiovascular risk factors.

Extraction and purification of an acid β-glucosidase from human placenta (alglucerase) for the treatment of Gaucher disease, replaced a few years later by a recombinant enzyme (imiglucérase, Cerezyme(®)), has paved the way to the development of enzyme replacement therapies (ERT) for the treatment of lysosomal storage diseases (LSD) among which Fabry disease for which the long-term efficacy of the two currently available preparations (agalsidase alfa, Replagal(®) and Fabrazyme(®)) is still being investigated. Mucopolysaccharidosis (MPS) type I (Hurler and Scheie diseases), II (Hunter syndrome) and VI (Maroteaux-Lamy disease) also benefit from ERT using laronidase (Aldurazyme(®)), idursulfase (Elaprase(®)) and galsulfase (Naglazyme(®)), respectively. ERT reduces the hepatosplenomegaly and improves the physical and respiratory capacities of MPS patients with a globally acceptable safety profile although the possibility of infusion-associated should always be kept in mind. Alglucosidase alpha (Myozyme(®)) improves the cardiomyopathy and life expectancy of infants suffering from Pompe disease and is under evaluation for the treatment of the juvenile and adult forms of the disease. CNS involvement remains a major challenge for many LSD and innovative research and approaches are needed to address the fact that recombinant enzymes do not cross the blood-brain barrier and therefore are not expected to lead to any improvement in CNS damages, except if alternative routes such as intrathecal administration would be developed. Molecular chaperones (e.g. migalastat for Fabry disease) and inhibitors of glucosylceramide synthesis (e.g. eliglustat tartrate for Gaucher disease) are currently under investigation in various clinical trials., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)