Restrictive Ventilatory Disorder Research Articles

Functional disorders of the respiratory system in patients with penetrating lung wounds: study prospective design

Background. Lung function tests should be performed to patients with penetrating wounds of the lungs to identify lung function disorders, and the data obtained should be taken into account when correcting medical treatment and prescribing individual medical rehabilitation programs.
 The aim was to study the impact of penetrating lung wounds on the lung function in the early recovery period.
 Materials and methods. 13 patients were enrolled in the study, 100% male, median age 26 [21; 31] years. Spirometry, body plethysmography, and a diffusion test were performed on an average of 14 days from the injury.
 Results. A prospective cross-sectional study was performed. A moderate decrease in the vital capacity (VC), the forced vital capacity (FVC), the volume of forced exhalation in the first second (FEV1) was revealed while the total lung capacity (TLCpl) and the FEV1/FVC ratio were within normal values. However, in 2 (15.4%) and 3 (23%) patients, a restrictive ventilation disorders (TLC lower limit of normal) was detected when using Global Lung Function Initiative (GLI) and European Community for Steel and Coal (ECSC) 1993 predicted value system, respectively. The residual lung (RV) and RV/TLCpl were increased. Functional residual capacity (FRC), FRCpl/TLCpl, airway resistance remained within normal values. The diffusion lung capacity impairment was detected in 92.3% and 61.5% cases according to the ECSC 1993 and the GLI system, respectively.
 Conclusion. In the early period of recovery after penetrating lung wounds, a nonspecific pattern of ventilation disorders was the most often abnormality namely a decrease in VC while TLC and FEV1/FVC were within normal values. Less frequently, the classic restrictive pattern of ventilation disorders was diagnosed. Besides, the diffusion lung capacity impairment was detected. The system of predicted values has a significant impact on the frequency of detection of the lung function disorders. The revealed functional disorders should be taken into account for adjusting medical and rehabilitation interventions.

Remimazolam enabled safe anesthetic management during tracheostomy in a patient with amyotrophic lateral sclerosis: a case report

BackgroundAmyotrophic lateral sclerosis (ALS) is known to cause generalized muscle atrophy and respiratory complications. Anesthetic agents and methods for patients with ALS are extremely important because they critically influence postoperative outcomes. In this clinical case, we mainly used remimazolam for safe anesthesia management.Case presentationA 66-year-old man had a gradual onset of numbness and weakness in his extremities over 2 years. He was diagnosed with ALS after the appearance of dysarthria and restrictive ventilation disorder. Due to the rapid progression of respiratory dysfunction, the patient was placed on artificial respiration, and a tracheostomy was planned. General anesthesia was induced with remimazolam (6 mg/kg/h) and remifentanil (0.5 μg/kg/min). Tracheal intubation was performed without muscle relaxants, followed by total intravenous anesthesia (TIVA) with continuous administration of remimazolam 0.8–1.2 mg/kg/h and remifentanil 0.3–0.5 μg/kg/min. At the end of the surgery, the anesthetic effect of remimazolam was reversed with 0.4 mg of flumazenil. The patient was discharged from the operating room with stable breathing, and changes to preoperative ventilator settings were not necessary.ConclusionsWe safely performed tracheostomy for a patient with ALS using remimazolam during general anesthesia.

The proteasome-dependent degradation of ALKBH5 regulates ECM deposition in PM2.5 exposure-induced pulmonary fibrosis of mice

Long-term inhalation of fine particulate matter (PM2.5) can cause serious effects on the respiratory system. It might be attributed to the fact that PM2.5 could directly enter and deposit in lung tissues. We established models of PM2.5 exposure in vivo and in vitro to explore the adverse effects of ambient PM2.5 on pulmonary and its potential pathogenic mechanisms. Our results showed that PM2.5 exposure promoted the deposition of ECM and the increased stiffness of the lungs, and then led to pulmonary fibrosis in time- and dose- dependent manners. Pulmonary function test showed restrictive ventilation function in mice after PM2.5 exposure. After PM2.5 exposure, ALKBH5 was recognized by TRIM11 and then degraded through the proteasome pathway. ALKBH5 deficiency (ALKBH5-/-) aggravated restrictive ventilatory disorder and promoted ECM deposition in lungs of mice induced by PM2.5. And the YAP1 signaling pathway was more activated in ALKBH5-/- than WT mice after PM2.5 exposure. In consequence, decreased ALKBH5 protein levels regulated miRNAs and then the miRNAs-targeted YAP1 signaling was activated to promote pulmonary fibrosis induced by PM2.5.

Respiratory Disorders Related to e-Waste Exposure among Workers in the Informal Sector in a Sub-Saharan African City: An Exposed Nonexposed Study.

Introduction Exposure to electrical and electronic equipment waste (e-waste) has become a growing health concern. The objective of this study was to measure the effect of exposure to e-waste on respiratory symptoms and on lung function parameters in workers involved in informal recycling activities in Cotonou city, Benin. Methods This was a cross-sectional study, in which exposed e-waste workers in Cotonou city were randomly selected. A matching nonexposed group based on age and sex was recruited from the general population. Respiratory symptoms were investigated using a questionnaire adapted from the British Medical Research Council's standardized respiratory questionnaire. Participants underwent lung function test using a portable spirometer (MIR SPIROBANK). Data were analyzed with STATA version 15 software. Results The overall prevalence of respiratory symptoms in e-waste workers was statistically higher in the exposed group (33.1% vs. 21.6%; p = 0.027). Chest tightness (11.8% vs. 2.1%; p = 0.003) and breathlessness (6.8% vs. 1.4%; p = 0.018) were the most reported symptoms by e-waste workers. Lung function testing showed a higher proportion of disorders among e-waste workers (25.0% vs. 14.9%, p = 0.029), with a higher proportion of probable restrictive (10.8% vs. 2.7, p = 0.005) and mixed (4.1% vs. 0%, p = 0.013) ventilatory disorders. Handling or working with e-waste was found associated with a significant reduction in forced expiratory volume in one second (FEV1) by 0.4 L (95% CI: 0.3-0.6) and forced vital capacity (FVC) by 0.75 L (95% CI: 0.6-0.9) after adjustment for age, BMI, smoking habits, asthma history, and daily income. Conclusion Work involving e-waste is associated with a higher prevalence of respiratory symptoms and with an increased risk of FEV1 and FVC decline, as well as of lung function impairment, particularly of restrictive disorders. Further studies to better clarify this association are needed. Awareness on this major public health threat should be raised in other sub-Saharan and Asian urban areas.

Association of Ventilatory Disorders with Respiratory Symptoms, Physical Activity, and Quality of Life in Subjects with Prior Tuberculosis: A National Database Study in Korea.

Tuberculosis (TB) survivors experience post-TB lung damage and ventilatory function disorders. However, the proportions of obstructive and restrictive ventilatory disorders as well as normal ventilation among subjects with prior TB are unknown. In addition, the impacts of ventilatory disorder and its severity on respiratory symptoms, physical activity limitations, and the quality of life in subjects with prior TB remain unclear. Subjects who participated in the Korean National Health and Nutritional Examination Survey 2007–2016 were enrolled in this study. We evaluated the impact of each ventilatory disorder and its severity on respiratory symptoms, physical activity limitations, and quality of life (measured by the EuroQoL five dimensions questionnaire [EQ-5D] index values) in subjects with prior TB. Among 1466 subjects with prior TB, 29% and 16% had obstructive ventilatory disorders and restrictive ventilatory disorders, respectively. Mild and moderate obstructive ventilatory disorders were not associated with respiratory symptoms, physical activity limitations, or EQ-5D index value compared with normal ventilation; however, severe obstructive ventilatory disorders were associated with more respiratory symptoms (adjusted odds ratio [aOR] = 13.62, 95% confidence interval [CI] = 4.64–39.99), more physical activity limitation (aOR = 218.58, 95% CI = 26.82–1781.12), and decreased EQ-5D index (adjusted coefficient = −0.06, 95% CI = (−0.12–−0.10) compared with normal ventilation. Mild restrictive ventilatory disorders were associated with more respiratory symptoms (aOR = 2.10, 95% CI = 1.07–4.14) compared with normal ventilation, while moderate (aOR = 5.71, 95% CI = 1.14–28.62) and severe restrictive ventilatory disorders (aOR = 9.17, 95% CI = 1.02–82.22) were associated with physical activity limitation compared with normal ventilation. In conclusion, among subjects with prior TB, 29% and 16% developed obstructive and restrictive ventilatory disorders, respectively. Severe obstructive ventilatory disorder was associated with more respiratory symptoms, more physical activity limitation, and poorer quality of life, while severe restrictive ventilatory disorder was associated with more physical activity limitations.

Stages of menopause and abnormal lung function: a cross-sectional study of middle-aged women.

Whether hormonal changes during menopausal transition postmenopause stages influence pulmonary function is not clearly understood. We evaluated the association between each stage of menopause and the prevalence of abnormal lung function in healthy middle-aged women. We hypothesized that the prevalence of abnormal lung function would increase from the late menopausal transition stage during menopausal transition postmenopause stages. The study population included women 40 to 65 years of age who underwent comprehensive health screening examination at the Kangbuk Samsung Hospital Total Healthcare Centers from January 2015 to December 2017. Stages of menopause (premenopausal, early menopausal transition, late menopausal transition, and postmenopausal) were defined using the 2011 Stages of Reproductive Aging Workshop (STRAW + 10) criteria. Abnormal lung function was defined based on spirometry as forced expiratory volume in 1 second/forced vital capacity less than70% or forced vital capacity less than 80% predicted. Prevalence ratios (PRs) for abnormal lung function by stages of menopause were estimated from logistic regression models adjusted for age, anthropometric measurements, lifestyle factors, medical history, lipid and inflammatory biomarkers, and reproductive factors. Among 43,822 participants, there were 4,615 participants (10.5%) with restrictive ventilatory disorder and 653 participants (1.5%) with obstructive ventilatory disorder. The fully adjusted PRs (95% confidence interval) for abnormal ventilatory disorder comparing early menopausal transition, late menopausal transition, and postmenopausal stage to premenopausal stage were 1.01 (0.94-1.08), 1.13 (1.03-1.22), and 1.10 (0.98-1.22), respectively. The fully adjusted PRs for restrictive ventilatory disorder comparing early menopausal transition, late menopausal transition, and postmenopausal stages to premenopausal stage were 1.02 (0.94-1.11), 1.18 (1.06-1.30), and 1.15 (1.00-1.31), respectively. There was, however, no clear association between stages of menopause and obstructive ventilatory disorder. In this large study of middle-aged women, the fully adjusted prevalence of abnormal lung function, particularly that of restrictive ventilatory disorder, was higher in women in late menopausal transition and menopausal stages compared to women in premenopausal stage.

Rheumatoid arthritis-associated lung disease in black Africans: Descriptive study of 28 cases in Lomé.

Background Several studies have shown that lung disease is a common extra-articular manifestation of rheumatoid arthritis (RA). Objectives To describe the lung manifestations in the RA population in Lomé, Togo. Methods The study was conducted from October 2018 to July 2019 at the pulmonology unit of the Sylvanus Olympio University teaching hospital, in collaboration with rheumatology centres in Lomé, Togo. Patients meeting the American College of Rheumatology criteria for RA were prospectively enrolled. They underwent clinical examination, spirometry, a 6-minute walk test (6MWT) and a chest X-ray (CXR). All information collected and surveys gathered were subjected to statistical analysis. Results Twenty-four out of 28 patients were women (85.7%). The mean (standard deviation (SD)) duration of illness was 4.1 (2.8) years. Thirteen patients out of 28 (46.4%) had respiratory symptoms. On CXR, interstitial lung disease was the only pleuropulmonary lesion (17.8%). Spirometry was abnormal in 25% of cases, with a predominance of restrictive ventilatory disorder (21.4%). The 6MWT was abnormal in 25% of patients. A total of 20 patients (71.4%) had at least one lung manifestation. We noted that there were significantly more patients with respiratory symptoms and no radiographical abnormalities than those with both respiratory symptoms and radiographical abnormalities (p=0.013). Conclusion Lung changes affect a significant proportion of RA patients in Lomé. Studies conducted with appropriate respiratory investigations and combining cardiovascular explorations will bring us closer to an understanding of the effects of RA-associated lung disease.

Heart Failure Results in Inspiratory Muscle Dysfunction Irrespective of Left Ventricular Ejection Fraction

Background: Exercise intolerance in heart failure with reduced ejection fraction (HFrEF) or heart failure with preserved ejection fraction (HFpEF) results from both cardiac dysfunction and skeletal muscle weakness. Respiratory muscle dysfunction with restrictive ventilation disorder may be present irrespective of left ventricular ejection fraction and might be mediated by circulating pro-inflammatory cytokines. Objective: To determine lung and respiratory muscle function in patients with HFrEF/HFpEF and to determine its associations with exercise intolerance and markers of systemic inflammation. Methods: Adult patients with HFrEF (n = 22, 19 male, 61 ± 14 years) and HFpEF (n = 8, 7 male, 68 ± 8 years) and 19 matched healthy control subjects underwent spirometry, measurement of maximum mouth occlusion pressures, diaphragm ultrasound, and recording of transdiaphragmatic and gastric pressures following magnetic stimulation of the phrenic nerves and the lower thoracic nerve roots. New York Heart Association (NYHA) class and 6-min walking distance (6MWD) were used to quantify exercise intolerance. Levels of circulating interleukin 6 (IL-6) and tumor necrosis factor-α (TNF-α) were measured using ELISAs. Results: Compared with controls, both patient groups showed lower forced vital capacity (FVC) (p < 0.05), maximum inspiratory pressure (PI_max), maximum expiratory pressure (PE_max) (p < 0.05), diaphragm thickening ratio (p = 0.01), and diaphragm strength (twitch transdiaphragmatic pressure in response to supramaximal cervical magnetic phrenic nerve stimulation) (p = 0.01). In patients with HFrEF, NYHA class and 6MWD were both inversely correlated with FVC, PI_max, and PE_max. In those with HFpEF, there was an inverse correlation between amino terminal pro B-type natriuretic peptide levels and FVC (r = −0.77, p = 0.04). In all HF patients, IL-6 and TNF-α were statistically related to FVC. Conclusions: Irrespective of left ventricular ejection fraction, HF is associated with respiratory muscle dysfunction, which is associated with increased levels of circulating IL-6 and TNF-α.

Cardiorespiratory performance capacity and airway microbiome in patients following primary repair of esophageal atresia

BackgroundPatients following repair of an esophageal atresia (EA) or tracheoesophageal fistula (TEF) carry an increased risk of long-term cardiopulmonary malaise. The role of the airway microbiome in EA/TEF patients remains unclear.MethodsAll EA/TEF patients treated between 1980 and 2010 were invited to a prospective clinical examination, spirometry, and spiroergometry. The airway microbiome was determined from deep induced sputum by 16 S rRNA gene sequencing. The results were compared to a healthy age- and sex-matched control group.ResultsNineteen EA/TEF patients with a mean age of 24.7 ± 7 years and 19 age- and sex-matched controls were included. EA/TEF patients showed a significantly lower muscle mass, lower maximum vital capacity (VCmax), and higher rates of restrictive ventilation disorders. Spiroergometry revealed a significantly lower relative performance capacity and lower peak VO2 in EA/TEF patients. Alpha- and beta-diversity of the airway microbiome did not differ significantly between the two groups. Linear discriminant effect size analysis revealed significantly enriched species of Prevotella_uncultured, Streptococcus_anginosus, Prevotella_7_Prevotella_enoeca, and Mogibacterium_timidum.ConclusionEA/TEF patients frequently suffer from restrictive ventilation disorders and impaired cardiopulmonary function associated with minor alterations of the airway microbiome. Long-term examinations of EA/TEF patients seem to be necessary in order to detect impaired cardiopulmonary function.ImpactThe key messages of the present study are a significantly decreased VCmax and exercise performance, as well as airway microbiome differences in EA/TEF patients.This study is the first to present parameters of lung function and exercise performance in combination with airway microbiome analysis with a mean follow-up of 24 years in EA/TEF patients.Prospective, long-term studies are needed to unravel possible interactions between alterations of the airway microbiome and impaired pulmonary function in EA/TEF patients.

Association between reproductive lifespan and lung function among postmenopausal women.

Correspondence to: Hye Yun Park, MD, PhD. Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea. Email: hyeyunpark@skku.edu; Juhee Cho, PhD. Department of Clinical Research Design and Evaluation, SAIHST, Sungkyunkwan University, 81 Irwon-ro, Gangnam-gu, Seoul, 06351, South Korea. Email: Jcho@skku.edu.BackgroundBoth age of menarche and menopause are associated with impaired lung function. However, there is limited data on the association between reproductive lifespan (RL) and lung function. This study aims to evaluate the association between RL and lung function among postmenopausal women.MethodsIn the Korean National Health and Nutrition Examination Survey (2010–2015), an annual cross-sectional survey of a national representative sample of non-institutionalized Korean population, the postmenopausal women aged 40–64 years who underwent spirometry were included. Spirometry was performed according to the recommendations of the American Thoracic Society and European Respiratory Society, and RL (years) was calculated by subtracting the age at menarche from age at menopause.ResultsAmong 3,410 postmenopausal women, the mean RL were 34.91 (standard error 0.07). In spline regression models, shorter RL was associated with reduced predicted forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1), and the associations were linear. In multivariable-adjusted models, the prevalence ratios (95% confidence interval) for abnormal lung function (restrictive or obstructive ventilatory disorder) comparing women with RL ≤30 years and >30 to <39 years to women with RL ≥39 years were 1.83 (1.15, 2.50) and 1.42 (1.01, 1.84), respectively. Women with RL ≤30 years showed a 2.13 (1.28, 3.54) times higher prevalence of restrictive ventilatory disorder than women with RL ≥39 years.ConclusionsIn postmenopausal women, a shorter RL was associated with abnormal lung function, particularly with a restrictive ventilatory disorder.

Inspiratory muscle dysfunction and restrictive lung function impairment in congenital heart disease: Association with immune inflammatory response and exercise intolerance

BackgroundIn adult patients with congenital heart disease (ACHD), both underlying disease and lung restriction contribute to exercise intolerance. In ACHD the yet incompletely understood mechanism underlying restricted ventilation may be inspiratory muscle weakness. Therefore, this study comprehensively evaluated inspiratory muscle function in ACHD and associations with systemic inflammation and the clinical severity of exercise intolerance. Methods30 ACHD patients (21 men, 35 ± 12 years) and 30 healthy controls matched for age, gender and body mass index underwent spirometry, measurement of mouth occlusion pressures, and diaphragm ultrasound. Six-minute walking distance (6MWD) and New York Heart Association functional class were used to quantify exercise intolerance. Interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α) levels were measured using enzyme-linked immunosorbent assays. ResultsACHD patients showed lower forced vital capacity (FVC), and maximum inspiratory (PImax) and expiratory (PEmax) pressures compared with controls (all p < 0.05). On ultrasound, ACHD patients showed a lower diaphragm thickening ratio (2.3 ± 0.5 vs. 2.8 ± 0.9, p < 0.01) and lower diaphragm excursion velocity during a voluntary sniff maneuver (5.7 ± 2.2 vs. 7.6 ± 2.0 cm/s, p < 0.01). Respiratory parameters, such as FVC (r = 0.53; p < 0.01) and PImax (r = 0.43; p = 0.02), correlated with 6MWD. Furthermore, amino terminal pro B-type natriuretic peptide levels were inversely correlated with FVC (r = −0.54; p < 0.01). Circulating pro-inflammatory cytokines were markedly increased, and IL-6 was correlated with 6MWD, dyspnea, and biomarkers of heart, lung and inspiratory muscle function (all p < 0.05). ConclusionsOur findings show that diaphragm dysfunction is present in ACHD and relates to restrictive ventilation disorder and exercise intolerance, possibly mediated by increased IL-6 levels.

ОТДАЛЕННЫЕ РЕСПИРАТОРНЫЕ ПОСЛЕДСТВИЯ ЗАБОЛЕВАНИЙ ОРГАНОВ ДЫХАНИЯ, ВОЗНИКШИХ В НЕОНАТАЛЬНОМ ПЕРИОДЕ, У НЕДОНОШЕННЫХ ДЕТЕЙ

The study aims to determine the long-term outcomes of respiratory diseases that occurred in children during the neonatal period and to evaluate the contribution to the development of the adverse effects of the SOD2 genetic polymorphism (rs4880). Material and methods. A survey of 60 children with a history of bronchopulmonary dysplasia (BPD) and 60 children who had neonatal respiratory distress syndrome (RDS) without the development of BPD was conducted. It included the examination by a pulmonologist, a genetic study of venous blood, a chest multispiral computed tomography (MSCT), and the spirometry for the children over six years old. Results. As the outcome of the past BPD in older children, the formation of chronic lung diseases that occurred with the chronic obstructive pulmonary disease was significantly more frequently observed. Among the children in the comparison group, the clinical recovery was observed in the vast majority of patients. The function of external respiration in the children born prematurely reflected the presence of obstructive and restrictive ventilation disorders. The unevenness of pneumatization of the lung tissue and the areas of pneumofibrosis were more often detected during the MSCT study of the patients in the main group. The allelic variant of rs4880 C47T was more often detected in children of the main group, who formed chronic lung diseases as the outcome of BPD.

P0058THE ROLE OF GENETICS IN INCOMPLETE DISTAL RENAL TUBULAR ACIDOSIS IN NEPHROLITHIASIS

Abstract Background and Aims Distal renal tubular acidosis (dRTA) is characterized by an impairment of urinary acidification resulting in a hyperchloremic non-anion gap metabolic acidosis, hypokalemia and inappropriately elevated urine pH. If not treated, this chronic condition eventually leads to nephrocalcinosis and recurrent nephrolithiasis, impaired renal function and bone demineralization due to reabsorption of bicarbonate and phosphate complexed with calcium from the bone as a buffer for metabolic acidosis. Distal RTA is therefore a well-defined entity that can be diagnosed by genetic testing of five genes known to be disease-causative (ATPV1B1 and ATPV0A4, FOXi1, SLC4A1 and WDR72). Incomplete distal renal tubular acidosis (idRTA) is defined as impaired urinary acidification that does not lead to overt metabolic acidosis and therefore can be diagnosed if patients fail to adequately acidify urine after an ammonium chloride (NH4Cl) challenge or other acid load tests. It is observed in 10-20% of calcium stone formers. It is still uncertain whether idRTA represents a distinct entity or it is part of the dRTA spectrum and whether it is caused by mutations in the same genes of overt dRTA. Heterozygous ATP6V1B1 pathogenic variants have been linked to idRTA, as well as mutations in SLC4A1. Method In this cross-sectional study we investigated a group of 23 stone formers whose clinical features were suspicious of idRTA: a history of nephrolithiasis or nephrocalcinosis with morning urinary pH &amp;gt; 5.8 in the absence of overt metabolic acidosis. They therefore underwent a simplified NH4Cl challenge and were found to have impaired urinary acidification ability. These patients were then analyzed by genetic testing with sequencing of 4 genes: SLC4A1, ATP6V1B1, ATP6V0A4 and FOXi1. Results Two unrelated individuals were found to have mutations in SLC4A1: 2 different variants in heterozygosis that had never been described before. The first patient was a 47-year old man, recurrent stone former (mainly apatite, but also some calcium oxalate), with hypercalciuria but normal bone mineral density (BMD). His family history revealed 1 uncle with kidney stones. The second patient was a 56-year old woman with a diagnosis of osteogenesis imperfecta, a history of reduced BMD and severe restrictive ventilation disorder and passed 1 stone (35% apatite, 65% CaOx). She was found to have bilateral nephrocalcinosis and hypocitraturia. Her family history revealed a sister with kidney stones. Conversely, 21 patients did not show any mutations for the genes sequenced, leading to a prevalence of genetic mutations of 8.7%. This is a much lower figure compared with overt dRTA, in which only 30% of patients with a clinical diagnosis of hereditary dRTA have no identified causative mutations in the currently known genes. Conclusion This suggests the involvement of other genes (WDR72 or others) or non-genetic tubular dysfunction in the pathogenesis of idRTA in stone formers.

Respiratory Muscle and Lung Function in Lung Allograft Recipients: Association with Exercise Intolerance

Background: In lung transplant recipients (LTRs), restrictive ventilation disorder may be present due to respiratory muscle dysfunction that may reduce exercise capacity. This might be mediated by pro-inflammatory cytokines such as tumor necrosis factor-α (TNF-α) and interleukin-6 (IL-6). Objective: We investigated lung respiratory muscle function as well as circulating pro-inflammatory cytokines and exercise capacity in LTRs. Methods: Fifteen LTRs (6 female, age 56 ± 14 years, 63 ± 45 months post-transplantation) and 15 healthy controls matched for age, sex, and body mass index underwent spirometry, measurement of mouth occlusion pressures, diaphragm ultrasound, and recording of twitch transdiaphragmatic (twPdi) and gastric pressures (twPgas) following magnetic stimulation of the phrenic nerves and the lower thoracic nerve roots. Exercise capacity was quantified using the 6-min walking distance (6MWD). Plasma IL-6 and TNF-α were measured using enzyme-linked immunosorbent assays. Results: Compared with controls, patients had lower values for forced vital capacity (FVC; 81 ± 30 vs.109 ± 18% predicted, p = 0.01), maximum expiratory pressure (100 ± 21 vs.127 ± 17 cm H₂O, p = 0.04), diaphragm thickening ratio (2.2 ± 0.4 vs. 3.0 ± 1.1, p = 0.01), and twPdi (10.4 ± 3.5 vs. 17.6 ± 6.7 cm H₂O, p = 0.01). In LTRs, elevation of TNF-α was related to lung function (13 ± 3 vs. 11 ± 2 pg/mL in patients with FVC ≤80 vs. >80% predicted; p < 0.05), and lung function (forced expiratory volume after 1 s) was closely associated with diaphragm thickening ratio (r = 0.81; p < 0.01) and 6MWD (r = 0.63; p = 0.02). Conclusion: There is marked restrictive ventilation disorder and respiratory muscle weakness in LTRs, especially inspiratory muscle weakness with diaphragm dysfunction. Lung function impairment relates to elevated levels of circulating TNF-α and diaphragm dysfunction and is associated with exercise intolerance.

Risk factors affecting postoperative pulmonary function in congenital diaphragmatic hernia.

PurposeIt is well known that congenital diaphragmatic hernia (CDH) in infants impacts pulmonary function rehabilitation after surgery. However, the risk factors of postoperative pulmonary function are still unclear. In this research, we analyzed the potential risk factors of postoperative pulmonary function in CDH patients in order to improve the clinical management of CDH patients.MethodsThirty-three cases CDH infants followed were enrolled from November 2016 to September 2018. Clinical data were reviewed. Tidal breathing pulmonary function testing was performed after surgery. Correlation between pulmonary function and clinical characteristics was evaluated using multivariate analysis of variance.ResultsPulmonary dysfunction was detected in 87.9% patients (29 of 33). The defect size was found to be significantly larger in patients with obstructed and mixed ventilatory disorders (P = 0.001). Diagnosis of gestational age (GA) was also significantly earlier compared to restrictive ventilatory disorders (P = 0.001). Larger defect size, and earlier prenatal diagnosis of GA were detected in severe obstructive ventilatory disorders (P = 0.007, P = 0.001, retrospectively).ConclusionMost patients had various degrees of pulmonary dysfunction after surgery. Patients with larger defect size and earlier diagnosis time might be vulnerable to severe obstructive and mixed ventilatory disorders.

Autopneumonektomie. Ein vergessenes Krankheitsbild

An 80-year old female was referred to our hospital with left internal carotid artery stenosis and a childhood history of hemoptysis. The ECG showed 2nd degree Mobitz atrio-ventricular block. The chest x-ray and computerized tomography identified a shift of the mediastinum and the heart to the left. The left lung was completely destroyed whilst the right lung was enlarged and crossed the midline. Pulmonary function tests revealed a moderate restrictive ventilation disorder. The diagnosis of autopneumonectomy was based on patient history together with radiological findings. A pacemaker was implanted with two stimulation electrodes via a left cephalic venous cutdown. Acarotid endarterectomy was also performed without any complication. After autopneumonectomy, postpneumonectomy like syndrome may occur in very rare cases, whereupon operative treatment is mandatory. Any respiratory infections should be treated with antibiotics. Pacemaker electrode placement via the subclavian vein is contraindicated due to the risk of a catastrophic pneumothorax.

Effects of PM2.5 on Cardio-Pulmonary Function Injury in Open Manganese Mine Workers.

Exposure to fine particulate matter 2.5 (PM2.5) is associated with adverse health effects, varying by its components. The health-related effects of PM2.5 exposure from ore mining may be different from those of environment pollution. The aim of this study was to investigate the effects of different concentrations of PM2.5 exposure on the cardio-pulmonary function of manganese mining workers. A total of 280 dust-exposed workers who were involved in different types of work in an open-pit manganese mine were randomly selected. According to the different concentrations of PM2.5 in the working environment, the workers were divided into an exposed group and a control group. The electrocardiogram, blood pressure, and multiple lung function parameters of the two groups were measured and analyzed. The PM2.5 exposed group had significantly lower values in the pulmonary function indexes of forced expiratory volume in one second (FEV1.0), maximum mid expiratory flow (MMEF), peak expiratory flow rate (PEFR), percentage of peak expiratory flow out of the overall expiratory flow volume (PEFR%), forced expiratory flow at 25% and 75% of forced vital capacity (FEF 25, FEF75), forced expiratory flow when 25%, 50%, and 75% of forced vital capacity has been exhaled (FEF25%,FEF50%, FEF75%), and FEV1.0/FVC% (the percentage of the predicted value of forced vital capacity) than the control group (all p < 0.05). Both groups had mild or moderate lung injury, most of which was restrictive ventilatory disorder, and there was significant difference in the prevalence rate of restrictive respiratory dysfunction between the two groups (41.4% vs. 23.6%, p = 0.016). Electrocardiogram (ECG) abnormalities, especially sinus bradycardia, were shown in both groups, but there was no statistical difference of the prevalence rate between the two groups (p > 0.05). Also, no significant difference of the prevalence rate of hypertension was observed between the PM2.5 exposure and control groups (p > 0.05). PM2.5 exposure was associated with pulmonary function damage of the workers in the open-pit manganese mine, and the major injury was restrictive ventilatory disorder. The early effect of PM2.5 exposure on the cardiovascular system was uncertain at current exposure levels and exposure time.

Ventilatory Support via Mouthpiece to Facilitate Ambulation.

The aim of the study was to evaluate the efficacy of noninvasive ventilatory assistance/support via mouthpiece to improve performance in 6-min walk tests for individuals with severe restrictive ventilatory disorders. Each subject performed the 6-min walk test breathing spontaneously and again while using mouthpiece noninvasive ventilatory assistance/support at full ventilatory support. Oxyhemoglobin saturation (O2 sat), heart rate, Borg scale, distance walked, and ambulation duration were recorded. Eighteen patients using nocturnal nasal noninvasive ventilatory assistance/support, median (interquartile range) age of 58.5 (20) yrs, were studied. Their median baseline forced vital capacity and PaCO2 were 940 (385) ml and 54.1 (6) mm Hg, respectively. All used noninvasive ventilatory assistance/support for at least overnight hours for 36 (111) mos. Because of the progression of ventilatory impairment and hypercapnia, daytime mouthpiece noninvasive ventilatory assistance/support was initiated. All the 6-min walk test parameters improved significantly by using noninvasive ventilatory assistance/support. Both initial and final values of O2 saturation significantly increased (92% [6] vs 96% [2], P < 0.001) and (80% [19] vs 85.50% [13], P = 0.001), respectively. The median distance walked increased by 43% with noninvasive ventilatory assistance/support (175 [218] m vs 250 [113] m, P = 0.017), and the total ambulation time also increased with noninvasive ventilatory assistance/support (5.45 [3] mins vs. 6 [0] mins, P = 0.008). The exercise tolerance of patients with severe ventilatory impairment can improve by using noninvasive ventilatory support.

An informative value of impulse oscillonetry for detecting restrictive abnormalities in idiopathic pulmonary fibrosis

The aim of this study was to investigate a role of impulse oscillometry (IO) for diagnosis of restrictive abnormalities in patients with idiopathic pulmonary fibrosis (IPF). Methods. Seventy two patients with restrictive ventilatory disorders diagnosed with spirometry and body plethysmography were involved in the study. The patients were divided into two groups: the group 1 (n = 34) comprised IPF patients, the group 2 (n = 38) comprised patients with respiratory diseases without extended pulmonary fibrosis. Data of spirometry, body plethysmography, lung diffusion test, and IO were analyzed. Results. IO was the most informative method for diagnosis of restrictive abnormalities in IPF patients: abnormal values, predominantly deltaXrs5, were found in 68% of the patients. IO was less useful in patients with non-fibrotic respiratory diseases where abnormal basic IO values were found in 39% of the patients. Decreased Xrs5, increased fres, unchanged Rrs5 and Rrs20, and abnormal absolute frequency dependence of Rrs were found in IPF patients with restrictive abnormalities; these changes could be seen in any respiratory disease with TLC ≤ 69%pred. Moreover, abnormal relative frequency dependence of Rrs (D(Rrs5–Rrs20)/Rrs20%) was detected. Conclusion. IO could be used as additional method for detecting restrictive abnormalities in patients with significant fibrotic lesions of the lungs.

Cardiopulmonary fitness in children with congenital heart diseases versus healthy children: A multicenter cross-sectional study

We aimed to compare the cardiopulmonary fitness of children with congenital heart diseases (CHD) to that of age- and gender-adjusted controls. We also intended to identify clinical characteristics associated with maximum oxygen uptake (VO2 max ) in this population. We included in a cross-sectional multicentre study a total of 798 children (496 CHD and 302 controls) who underwent a complete cardiopulmonary exercise test (CPET). The association of clinical characteristics with VO2 max was studied using a multivariate analysis. The study was approved by the South Mediterranean IV Ethics Committee (2009-A00423-54) and registered on ClinicalTrials.gov ( NCT01202916 ). Informed consent was obtained from all parents. Mean VO2 max in the CHD group and control represented 93 ± 20% and 107 ± 17% of predicted values, respectively. VO2 max was significantly lower in the CHD group, overall (37.8 ± 0.3 vs. 42.6 ± 0.4 mL/kg/min, P < 0.0001) and for each group ( P < 0.05) ( Table 1 ). The mean VO2 max decline per year was significantly higher in CHD than in the controls overall (−0.84 ± 0.10 vs −0.19 ± 0.14 mL/kg/min/year, P < 0.01), for males (−0.72 ± 0.14 vs. 0.11 ± 0.19 mL/kg/min/year, P < 0.01), and for females (−1.00 ± 0.13 vs. −0.55 ± 0.21 mL/kg/min/year, P = 0.05) ( Fig. 1 ). VO2 max was associated with body mass index, ventilatory anaerobic threshold, female gender, restrictive ventilatory disorder, right ventricle systolic hypertension, tricuspid regurgitation, the number of cardiac catheter or surgery procedures, and the presence of a genetic anomaly. This comparative CPET study provided, for the first time, relevant values of VO2 max and their clinical determinants in the largest reported cohort of CHD children compared to a control group. Although the magnitude of the difference was not large, VO2 max among children with CHD was significantly lower than in normal children. We suggest performing CPET in routine follow-up of these patients.