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Alternating or phasic ECG voltage changes are most commonly associated with intrinsic myocardial electrophysiological perturbations or mechanical oscillation within a pericardial effusion. Rare descriptions of electrical alternation have been reported with pneumothorax. We present a case of a 53-year-old woman with vanishing lung syndrome who presented with spontaneous left pneumothorax and phasic ECG voltage changes that resolved after re-expansion of the lung., (2015 BMJ Publishing Group Ltd.)

Combined pulmonary fibrosis and emphysema (CPFE) syndrome is a rare disease characterized with shortness of breath, upper lobe emphysema, lower lobe fibrosis and impairment of gas exchange. This syndrome is a disease usually seen in male smokers. Pulmonary hypertension is associated with mortality. Another important feature, spirometric volumes relatively protected and a reduction in carbon monoxide diffusion test. CPFE syndrome in the literature so far have been identified in only 70 patients. In this review CPFE syndrome is presented with literature.

Introduction: A heterogeneous emphysema is one of the most severe forms of chronic obstructive pulmonary disease (COPD). In some cases, besides the standard pharmacotherapy, a new treatment option of emphysema can be used - bronchoscopic lung volume reduction (BLVR) with the use of intrabronchial valves., Objectives: To examine the health-related quality of life (HRQoL) of patients with severe emphysema after intrabronchial valve (IBV) implantation for the treatment of one lung., Material and Methods: From 2011 to 2013 a single centre prospective observational study was performed. The study assessed the effect of the therapeutic BLVR intervention, measured by St. George Respiratory Questionnaire (SGRQ). A statistical analysis by use of Wilcoxon test for dependent variables was performed., Results: Twenty patients were enrolled to the study (mean age 63 ± 10 years), all ex-smokers with tobacco exposure 38 ± 11.3 packyears. After 3 months of IBV treatment the average SGRQ score improved significantly in total (-12.8; p < 0.001) and in domains and the differences were for: "symptoms" (-8.5; p < 0.001), "activity" (-13.9; p < 0.001) and "influence on life"(-13.5; p < 0.002)., Conclusions: The presented study revealed a significant improvement of the quality in the life measured by SGRQ after IBV treatment for heterogeneous emphysema. For the first time our study showed the significant improvement of all three domains of SGRQ after IBV treatment.

Objectives: To identify perinatal risk indicators for air leaks in preterm infants treated with a policy of restrictive use of endotracheal intubation based on sustained inflations followed by noninvasive ventilation in the delivery room., Methods: Perinatal variables and variables of respiratory support in the delivery room were analyzed retrospectively in a cohort of 297 inborn preterm infants with a gestational age <29 weeks born in 2005-2009 in a tertiary care center with respect to their associations with air leaks. Multivariate logistic regression analysis was performed to analyze independent risk indicators., Results: Gestational age was 26 weeks + 0 days (22+3 to 28+6), birth weight was 790 g (265-1,660) and 270/297 survived (91.0%). A total of 63 (21.2%) developed air leaks, 32 (10.8%) pneumothorax, 44 (14.8%) pulmonary interstitial emphysema, and 1 (0.3%) pneumopericardium. The infants with air leaks had a higher risk of death (p < 0.01) and of intraventricular hemorrhage grade 3/4 (p < 0.05). Air leaks were associated with less use of prenatal steroids (p < 0.01), more frequent use of cardiac compressions (p < 0.01), use of a pressure of 30 cm H2O for sustained inflations (p < 0.05), and intubation in the delivery room (p < 0.01). After multivariate logistic regression only prenatal steroids (OR 0.41, 0.20-0.85), epinephrine (OR 3.56, 1.55-8.15) and surfactant use (OR 12.03, 3.39-42.72) remained significant., Conclusions: Our approach resulted in a high survival rate but was associated with a substantial rate of air leaks, which were associated with death and severe intraventricular hemorrhage. Prenatal steroids were protective, and epinephrine and surfactant use were significant risk indicators, whereas the use of sustained inflations was not a risk factor., (© 2015 S. Karger AG, Basel.)

Background: Chronic obstructive pulmonary disease (COPD) is often underdiagnosed, which might be attributable to a lack of knowledge about the disease among the general population. The objective of this study was to evaluate COPD-related knowledge among primary care users in an urban area in Brazil., Methods: This study was carried out at primary care clinics (PCCs), including 12 general PCCs and 26 family health PCCs, in the city of Goiânia, Brazil. Between May 2013 and February 2014, we interviewed 674 PCC users, applying a questionnaire designed to assess COPD-related knowledge. Satisfactory knowledge of COPD was defined as knowing at least two of its symptoms and that smoking is a risk factor for the disease., Results: Of the 674 users interviewed, only 9.2% recognized the term "COPD", 75.1% recognized the term "emphysema", and 15.7% did not recognize either term. We found that recognizing either term was associated with a higher level of education (P<0.001). The prevalence of satisfactory knowledge of COPD was 16.2%, and having such knowledge was associated with being over 60 years of age. The COPD symptom known by the greatest proportion of users (70.6%) was dyspnea, and most (87.5%) knew that smoking is a risk factor, whereas only a few (4.9%) knew that exposure to wood smoke is also a risk factor. The most frequently cited sources of knowledge were the media (43.1%) and a relative with COPD (36.4%)., Conclusion: Most of the PCC users evaluated did not know the term "COPD" but were familiar with the term "emphysema". The level of basic knowledge about the disease was low in this population. These results should alert health care administrators to the need for interventions aimed at increasing the diagnosis rate and thus promoting the early treatment of COPD.

COPD is not a homogenous disease but consists of at least four different phenotypes: Emphysema, COPD with chronic bronchitis, asthma-COPD overlap syndrome (ACOS), and COPD with recurrent exacerbations. With differentiation, treatment can be designed phenotype-specific. Some modern drugs are not indicated in all phenotypes.

Background: An innovative approach to lung volume reduction (LVR) for emphysema is introduced in the design of the Sequential Segmental Treatment of Emphysema with Upper Lobe Predominance (STEP-UP) trial where vapour ablation is administered bilaterally over the course of two sessions and is used to target only the most diseased upper lobe segments. By dividing the procedure into two sessions, there is potential to increase the total volume treated per patient but reduce volume treated and energy delivered per session. This is expected to correlate with improvements in vapour ablation's safety and efficacy profiles., Methods: The STEP-UP trial is a randomized, controlled, open-label, 12 month study of patients with upper lobe predominant emphysema (ULPE). The trial compares patients receiving standard medical management alone against patients receiving bilateral vapour ablation in addition to standard medical management. An intended sixty nine subjects will be randomized at a 2:1 (treatment arm:control arm) ratio. Inclusion criteria include a forced expiratory volume in 1 second (FEV1) between 20% and 45% predicted, total lung capacity > 100% predicted, residual volume > 150% predicted, marked dyspnea scoring ≥ 2 on the modified Medical Research Council (mMRC) scale, and PaCO2 ≤ 50 mm Hg. The primary endpoints are the change in FEV1 %predicted and St. George Respiratory Questionnaire (SGRQ) score between the treatment and control arm at 12 months. Adverse events will be monitored as secondary endpoints along with other efficacy outcomes at 6 and 12 months., Discussion: Vapour ablation can reduce lung volume in the presence of collateral ventilation (CV). Due to this ability, it can be used to target specifically the more diseased segments of each upper lobe. Safety and efficacy outcomes are expected to improve by considering which segments to treat along with the volume treated per session and per patient., Trial Registration: ClinicalTrials.gov NCT01719263.

Congenital cystic lesions of the lung are present in 1 in 10,000-35,000 births and present as a spectrum of anomalies. Majority of these cystic lesions comprise congenital cystic adenomatoid malformations, pulmonary sequestrations, congenital lobar emphysema, and bronchogenic cysts. Most of these lesions are nowadays detected antenatally, however some will present either in the newborn or during later childhood. A review of the aetiology, classification, natural history, investigations, and treatment of congenital cystic lung lesions is discussed., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Background: The National Emphysema Treatment Trial (NETT) was a randomized clinical trial designed to compare lung volume reduction surgery (LVRS) with maximal medical care for patients with severe emphysema. The trial was halted early for a subgroup of patients with severe lung disease. We report longer term follow-up for this high-risk subgroup., Methods: In a randomized clinical trial, patients with moderate to severe emphysema were assigned to LVRS plus maximal medical care or to maximal medical care alone and followed prospectively for vital status over 15 years. We focus on 140 high-risk patients. Quality of life data were available through 6 years of follow-up and were assessed using the University of California, San Diego Shortness of Breath Questionnaire and the Self-Administered Quality of Well-Being Scale., Results: Through the first 3 years of follow-up, surgical patients in the high-risk subgroup had a significantly higher probability of death. However, the mortality curves crossed and there was a trend favoring surgical treatment through the remainder of the follow-up. The log-rank test suggested that the 2 groups were not significantly different (p=0.95) in survival. Quality of life data suggested an advantage of LVRS through the first 5 years of follow-up (p<0.01). The combined quality-adjusted survival model favored the medical group for the first few years of follow-up and favored the LVRS group after 4 years., Conclusions: The NETT was stopped early for high-risk patients with severe lung disease. Longer term follow-up suggests that surgical patients in this high-risk subgroup ultimately achieved comparable outcomes. The high risk of death within 30 days of the surgery may discourage use of the procedure for high-risk patients despite the potential for better long-term outcomes., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We sought to assess whether the effects of mesenchymal stromal cells (MSC) on lung inflammation and remodeling in experimental emphysema would differ according to MSC source and administration route. Emphysema was induced in C57BL/6 mice by intratracheal (IT) administration of porcine pancreatic elastase (0.1 UI) weekly for 1 month. After the last elastase instillation, saline or MSCs (1×105), isolated from either mouse bone marrow (BM), adipose tissue (AD) or lung tissue (L), were administered intravenously (IV) or IT. After 1 week, mice were euthanized. Regardless of administration route, MSCs from each source yielded: 1) decreased mean linear intercept, neutrophil infiltration, and cell apoptosis; 2) increased elastic fiber content; 3) reduced alveolar epithelial and endothelial cell damage; and 4) decreased keratinocyte-derived chemokine (KC, a mouse analog of interleukin-8) and transforming growth factor-β levels in lung tissue. In contrast with IV, IT MSC administration further reduced alveolar hyperinflation (BM-MSC) and collagen fiber content (BM-MSC and L-MSC). Intravenous administration of BM- and AD-MSCs reduced the number of M1 macrophages and pulmonary hypertension on echocardiography, while increasing vascular endothelial growth factor. Only BM-MSCs (IV > IT) increased the number of M2 macrophages. In conclusion, different MSC sources and administration routes variably reduced elastase-induced lung damage, but IV administration of BM-MSCs resulted in better cardiovascular function and change of the macrophage phenotype from M1 to M2.

Background: Lung volume reduction surgery improves quality of life, exercise capacity, and survival in selected patients but is accompanied by significant morbidity. Bronchoscopic approaches may provide similar benefits with less morbidity., Methods: In a randomized, sham procedure controlled, double-blind trial, 277 subjects were enrolled at 36 centers. Patients had emphysema, airflow obstruction, hyperinflation, and severe dyspnea. The primary effectiveness measure was a significant improvement in disease-related quality of life (St. George's Respiratory Questionnaire) and changes in lobar lung volumes. The primary safety measure was a comparison of serious adverse events., Results: There were 6/121 (5.0%) responders in the treatment group at 6 months, significantly >1/134 (0.7%) in the control group [Bayesian credible intervals (BCI), 0.05%, 9.21%]. Lobar volume changes were significantly different with an average decrease in the treated lobes of -224 mL compared with -17 mL for the control group (BCI, -272, -143). The proportion of responders in St. George's Respiratory Questionnaire was not greater in the treatment group. There were significantly more subjects with a serious adverse event in the treatment group (n=20 or 14.1%) compared with the control group (n=5 or 3.7%) (BCI, 4.0, 17.1), but most were neither procedure nor device related., Conclusions: This trial had technical and statistical success but partial-bilateral endobronchial valve occlusion did not obtain clinically meaningful results. Safety results were acceptable and compare favorably to lung volume reduction surgery and other bronchial valve studies. Further studies need to focus on improved patient selection and a different treatment algorithm., Trial Registry: ClinicalTrials.gov NCT00475007.

Unlabelled: Pulmonary interstitial emphysema (PIE) is a frequent complication in preterm-ventilated infants. These newborns have an increased risk of mortality and bronchopulmonary dysplasia. Various treatments for PIE have been proposed: high-frequency ventilation, postural therapy, selective bronchial intubation or surgery. We present an atypical case of a preterm infant with respiratory distress syndrome complicated by pneumothorax, who after recovery and discontinuation of mechanical ventilation, developed unilateral PIE which was treated with selective bronchial intubation., Conclusions: Selective bronchial intubation and lung ventilation are therapeutic strategies which can promote the regression of PIE. Conservative approach should remain the first choice treatment of unilateral PIE in order to limit surgical procedures, while effort should be made to prevent this iatrogenic complication.

Background: Right ventricular dysfunction in COPD is common, even in the absence of pulmonary hypertension. The aim of the present study was to examine the effects of high intensity interval training (HIIT) on right ventricular (RV) function, as well as pulmonary blood vessel remodeling in a mouse model of COPD., Methods: 42 female A/JOlaHsd mice were randomized to exposure to either cigarette smoke or air for 6 hours/day, 5 days/week for 14 weeks. Mice from both groups were further randomized to sedentariness or HIIT for 4 weeks. Cardiac function was evaluated by echocardiography and muscularization of pulmonary vessel walls by immunohistochemistry., Results: Smoke exposure induced RV systolic dysfunction demonstrated by reduced tricuspid annular plane systolic excursion. HIIT in smoke-exposed mice reversed RV dysfunction. There were no significant effects on the left ventricle of neither smoke exposure nor HIIT. Muscularization of the pulmonary vessels was reduced after exercise intervention, but no significant effects on muscularization were observed from smoke exposure., Conclusions: RV function was reduced in mice exposed to cigarette smoke. No Increase in pulmonary vessel muscularization was observed in these mice, implying that other mechanisms caused the RV dysfunction. HIIT attenuated the RV dysfunction in the smoke exposed mice. Reduced muscularization of the pulmonary vessels due to HIIT suggests that exercise training not only affects the heart muscle, but also has important effects on the pulmonary vasculature.

Chronic obstructive pulmonary disease (COPD) is a disease with a high incidence and extensive comorbidities that make COPD a key challenge for anesthesiologists. A new treatment strategy, such as endoscopic lung volume reduction (ELVR) with implantation of endobronchial valves is a rapidly developing area which is still unknown to many anesthesiologists. This article therefore describes first experiences in a patient with five endobronchial valves in the right upper lobe who needed urgent surgery due to lumbar disc herniation with neurological impairment. After preoperative evaluation of the patient's condition, the use of bronchodilating volatile anesthetics and adjusting the ventilatory settings with long expiration times and low peak pressure in a pressure controlled mode seems favorable in these patients. Intraoperatively, the patient should be assessed with modern physiological monitoring tools to titrate the administration of anesthetic agents, opioids and myorelaxant drugs. In conclusion the care of patients with implanted endobronchial valves after ELVR does not differ from COPD patients without ELVR. A close cooperation between surgeons, anesthesiologists and internists is mandatory in the care of these patients.

Background: Non-pharmacological respiratory physiotherapy in treatment of COPD with severe emphysema is achieving increasing importance. Ez-PAP, a compact CPAP- or flow-PEP system, supports inspiration by using the Coanda effect in addition to a PE(E)P-effect during expiration., Methods and Patients: 30 patients with severe COPD and emphysema and hypercapnic respiratory failure under non-(NIV) (n = 28) and invasive ventilation (n = 2) were treated in ventilator-free intervals with Ez-PAP and analyzed retrospectively. Clinical courses such as ventilator-dependency, dyspnea by visual CR-10 Borg scale, results of six-minute walk tests (6MWT), lung function tests and recordings of transcutaneous CO2 measurements were evaluated where possible., Results: Analyzed patients showed less ventilator dependancy (n = 9), reported a median decrease of shortness of breath by 3,3 points (n = 10) and improved by 50,4 m in the 6MWT (n = 5). A median increase of vital capacity by 544 ml (n = 5) was shown by spirometry. Combined with manual techniques for reducing air-trapping, treatment success was documented by a median reduction of pCO2 by 7,3 mmHg (n = 6) using transcutaneous pCO2 measurement., Conclusion: Ez-PAP is a simple mechanical method to reduce dynamic hyperinflation in severe COPD, thus achieving relief of respiratory muscles and more effective breathing. Prospective studies of this promising method are urgently required., (© Georg Thieme Verlag KG Stuttgart · New York.)

Objectives: To investigate the role of volume quantitative assessment using multidetector-row computed tomography to select patients scheduled for endobronchial one-way valves treatment., Methods: Twenty-five consecutive patients (15 with heterogeneous emphysema and 10 with giant emphysematous bulla) undergoing endobronchial valves treatment were enrolled. All patients were studied pre- and postoperatively with standard pulmonary functional tests and quantitative volume assessment of target lobe and entire lung. Emphysematous parenchyma was obtained applying density thresholds of -1.024/-950 Hounsfield units. Among different subtype of patients, we evaluated: (i) the differences between preoperative versus postoperative data; (ii) the correlation between functional and volumetric quantification changes and (iii) the critical threshold value of volumetric quantification of the target lobe in close association with clinical effects., Results: Among heterogeneous emphysematous and giant emphysematous bulla patients, a significant improvement of flow-expiratory volume in 1 s (from 36.9 ± 15.3 to 43.9 ± 10.4; P = 0.01; and from 35.8 ± 6.0 to 47.5 ± 7.9; P < 0.0001, respectively); and of forced vital capacity (from 41.9 ± 5.9 to 47.3 ± 9.3; P = 0.0009 and from 40.7 ± 5.9 to 48.8 ± 4.9; P = 0.0002, respectively); and a significant reduction of residual volume (from 185 ± 14 to 157 ± 14.7; P = 0.005; and from 196 ± 13.5 to 137 ± 21; P < 0.0001, respectively) and of total lung volume (from 166.7 ± 13 to 137 ± 18 ; P = 0.0003, and from 169 ± 15 to 134 ± 18; P < 0.0001, respectively) were seen after treatment. The volumetric measurements showed a reduction of volume of the treated lobe among heterogeneous emphysematous patients (from 1448 ± 204 to 1076 ± 364; P = 0.0008); and in those with giant emphysematous bulla (from 1668 ± 140 to 864 ± 199; P < 0.0001). The entire lung and target lobe volume changes were inversely correlated with change in forced expiratory volume in 1 s in patients with heterogeneous emphysematous (r = -0.7; P = 0.0006; and r = -0.7; P = 0.0009, respectively) and giant emphysematous bulla (r = -0.8; P = 0.001; and r = -0.7; P = 0.009, respectively). Among patients with heterogenous emphysematous and giant emphysematous bulla, the value of sensitivity and specificity were 66.6 and 83%, respectively (for a volumetric qunatification >1.5239), and of 60 and 100%, respectively (for a volumetric qunatification >1.762)., Conclusions: Our study showed that the volumetric quantification adds further informations to the routine evaluation for optimizing the selection of patients scheduled for endobronchial valve treatment., (© The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Congenital lobar emphysema used to be treated surgically. Congenital lobar hyperinflation is the currently recommended term, as it involves pathologically healthy lung tissue, which is why conservative management may be an option. Four cases of diagnosed congenital lobar hyperinflation are presented in which conservative treatment was chosen due to their clinical stability. Their outcome has been satisfactory with progressively normal radiology., (Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)

The aim of this study was to characterize the evolution of lung function and -structure in elastase-induced emphysema in adult mice and the effect of mesenchymal stromal cell (MSC) administration on these parameters. Adult mice were treated with intratracheal (4.8 units/100 g bodyweight) elastase to induce emphysema. MSCs were administered intratracheally or intravenously, before or after elastase injection. Lung function measurements, histological and morphometric analysis of lung tissue were performed at 3 weeks, 5 and 10 months after elastase and at 19, 20 and 21 days following MSC administration. Elastase-treated mice showed increased dynamic compliance and total lung capacity, and reduced tissue-specific elastance and forced expiratory flows at 3 weeks after elastase, which persisted during 10 months follow-up. Histology showed heterogeneous alveolar destruction which also persisted during long-term follow-up. Jugular vein injection of MSCs before elastase inhibited deterioration of lung function but had no effects on histology. Intratracheal MSC treatment did not modify lung function or histology. In conclusion, elastase-treated mice displayed persistent characteristics of pulmonary emphysema. Jugular vein injection of MSCs prior to elastase reduced deterioration of lung function. Intratracheal MSC treatment had no effect on lung function or histology.

Introduction: Chronic obstructive pulmonary disease (COPD) is a major cause of morbidity and mortality worldwide. Emphysema is a component of COPD characterised by hyperinflation resulting in reduced gas exchange and interference with breathing mechanics. Endoscopic lung volume reduction using one-way valves to induce atelectasis of the hyperinflated lobe has been developed and studied in clinical trials over the last decade., Methods: Searches for appropriate studies were undertaken on PubMed and Clinical Trials Databases using the search terms COPD, emphysema, lung volume reduction and endobronchial valves., Results: The evidence from the randomised clinical trials suggests that complete lobar occlusion in the absence of collateral ventilation or where there is an intact lobar fissure are the key predictors for clinical success. Other indicators are greater heterogeneity in disease distribution between upper and lower lobes. The proportion of patients that respond to treatment improves from 20% in the unselected population to 75% with appropriate patient selection. The safety profile for endobronchial valves in this severely affected group of patients with emphysema was acceptable and the main adverse events observed were an excess of pneumothoraces., Conclusion: Selected patients have the potential of significant benefit in terms of lung function, exercise capacity and possibly even survival. These considerations are essential in-order to maximise patient benefit in a resource-limited environment and also to ensure that beneficial treatments are available for the appropriate patient.

Endobronchial valve (EBV) therapy may be associated with improvements in chronic obstructive pulmonary disease-related outcomes and may therefore be linked to improvements in the body mass index, airflow obstruction, dyspnoea, exercise capacity (BODE) index. Data from 416 patients with advanced emphysema and hyperinflation across Europe and USA, who were randomised to EBV (n=284) or conservative therapy (n=132) were analysed. Quantitative image analysis was used to compare the volume of the targeted lobe at baseline and at 6 months to determine target lobe volume reduction (TLVR). 44% of patients receiving EBV therapy (versus 24.7% of controls) had clinically significant improvements in the BODE index (p<0.001). BODE index was significantly reduced by mean ± sd 1.4 ± 1.8, 0.2 ± 1.3 and 0.1 ± 1.3 points in patients with TLVR >50%, 20%-50% and <20%, respectively (intergroup differences p<0.001), but increased by 0.3 ± 1.2 points in controls. Changes in BODE were predicted by baseline BODE and correlated significantly with lobar exclusion and lung volumes at 6 months. A greater proportion of patients in the treatment group than in the control group achieved a clinically meaningful improvement in BODE index; however, the likelihood of benefit was less than half in both groups. Patients in whom TLVR was obtained had greater improvements in clinical outcomes.

Minimum clinically important change of 5 points in the University of California, San Diego Shortness of Breath Questionnaire (SOBQ) is established, but cutoff values between a small, a moderate, and a large change are still unknown. We used the data set of National Emphysema Treatment Trial consisting of severe and very severe chronic obstructive pulmonary disease patients, whose mean age was 64 years. Changes from baseline to post-surgical 6-month follow-up were evaluated. The St. George's Respiratory Questionnaire was used as anchor: |∆SGRQ| < 4, meaningless change; 4 ≤ |∆SGRQ| < 8, small change; 8 ≤ |∆SGRQ| < 13, moderate change; 13 ≤ |∆SGRQ|, large change. We decided the final cutoff values for the SOBQ as medians of the three anchor methods. We also decided the range of cutoff values as the range of three values. In a cohort of surgically treated patients (N = 484), we propose value of 5 (range 5-6), 11 (range 9-15), and 16 (range 14-20) for the cutoff values between a meaningless and a small change (minimum clinically important difference), a small and a moderate change, and a moderate and a large change, respectively. In a cohort of medically treated patients, numbers of patients categorized according to ∆SOBQ scores were similar to those of the patients categorizes according to the ∆SGRQ (N = 480) or ∆Forced expiratory volume in 1 second (N = 425). We propose group-level cutoff values and range between a small, a moderate, and a large changes.

In the treatment of emphysema with an endobronchial valve, entire lobar treatment is important in achieving adequate atelectasis. This case illustrates that without treatment of the entire lobe, it can fail to collapse even after several years, leading to treatment failure. Intralobar collateral ventilation through the pores of Kohn is demonstrated in this case, as endobronchial valve blockage of the remaining patent anterior segment resulted in the desired atelectasis and significant improvements in pulmonary function.

An infant with ventricular septal defect, atrial septal defect, and Down syndrome (trisomy 21) was referred to our institution. The patient gradually developed infantile lobar emphysema of the right upper lobe and became dependent on mechanical ventilator support. When the patient was five months of age, we successfully performed a total correction of cardiac anomalies combined with a right upper lobectomy. The resection of the right upper lobe was performed under cardiopulmonary bypass through a median sternotomy. There was no major airway bleeding or mediastinitis. The patient was extubated on postoperative day 12 and discharged on postoperative day 29.

Background: Chronic bronchitis in COPD has been associated with an increased exacerbation rate, more hospitalizations, and an accelerated decline in lung function. The clinical characteristics of patients with advanced emphysema and chronic bronchitis have not been well described., Methods: Patients randomized to medical therapy in the National Emphysema Treatment Trial were grouped based on their reports of cough and phlegm on the St. George's Respiratory Questionnaire(SGRQ) at baseline: chronic bronchitis(CB+) and no chronic bronchitis(CB-). The patients were similarly categorized into severe chronic bronchitis(SCB+) or no severe chronic bronchitis (SCB-) based on the above definition plus report of chest trouble. Kaplan-Meier survival analysis was used to determine the relationships between chronic bronchitis and severe chronic bronchitis and survival and time to hospitalization. Lung function and SGRQ scores over time were compared between groups., Results: The CB+(N = 234; 38%) and CB- groups(N = 376; 62%) had similar survival (median 60.8 versus 65.7 months, p = 0.19) and time to hospitalization (median 26.9 versus 24.9 months, p = 0.84). The SCB+ group(N = 74; 12%) had worse survival (median 47.7 versus 65.7 months, p = 0.02) and shorter time to hospitalization (median 18.5 versus 26.7 months, p = 0.02) than the SCB- group (N = 536; 88%). Mortality and hospitalization rates were not increased when chest trouble was analyzed by itself. The CB+ and CB-groups had similar lung function and SGRQ scores over time. The SCB+ and SCB-groups had similar lung function over time, but the SCB+ group had significantly worse SGRQ scores., Conclusions: Severe chronic bronchitis is associated with worse survival, shorter time to hospitalization, and worse health-related quality of life.

Introduction: Idiopathic pulmonary fibrosis (IPF) is one of the most frequent interstitial lung disease. Emphysema can be associated with IPF as described in the «Combined pulmonary fibrosis and emphysema» syndrome., Aim: The primary endpoint of this retrospective cohort study was to evaluate the impact of the association of IPF and emphysema on lung function tests parameters (FVC, TLC, FEV1, FEV1/FVC and DLCO). The secondary endpoint was to assess the impact of the associated radiological emphysema on lung function parameters used in the du Bois prognostic score recently developed by Ron du Bois et al., Method: We retrospectively reviewed the medical files of 98 patients with lung fibrosis who were followed in our University Hospital with access to pharmacological studies and lung transplantation from 1981 to 2011. Fifty six patients were considered for analysis. The collected data included gender, age, smoking history and respiratory hospitalizations. We also analysed their pulmonary functional parameters along with radiological characteristics, in particular the presence of emphysema which was assessed on thoracic high resolution CT scan. The du Bois score was retrospectively calculated from these data., Results: TLC and FVC at diagnosis were significantly higher in the IPF-E group compared to the IPF group (respectively 86.6 ± 17.2% pv versus 72.0 ± 15.0% pv; p: 0.004 and 86.8 ± 18.4% pv versus 72.6 ± 20.6% pv; p: 0.020). The [Formula: see text] used in the calculation of the du Bois prognostic score was significantly higher in the IPF-E group. By cons, [Formula: see text] was not statistically different between the two groups., Conclusion: Radiological emphysema associated with IPF had an impact on pulmonary function tests. Despite this difference, the du Bois score was not statistically different between these two groups. Nevertheless, after one year of follow up, the patients with emphysema were in a subclass with a lower mortality rate than those without emphysema., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Transpulmonary pressure and the mechanical stresses of breathing modulate many essential cell functions in the lung via mechanotransduction. We review how mechanical factors could influence the pathogenesis of emphysema. Although the progression of emphysema has been linked to mechanical rupture, little is known about how these stresses alter lung remodeling. We present possible new directions and an integrated multiscale view that may prove useful in finding solutions for this disease.

Accidental sand aspiration is an unusual but potentially life-threatening condition in children. Sand aspiration often occurs in cases of near drowning and accidental burial in sand. The authors report a case of accidental sand aspiration in a 14-mo-old girl while she was playing with sand. Though child was symptomatic and chest radiograph was showing obstructive emphysema initially, there was spontaneous resolution of radiographic findings and clinical condition within 48 h. Child was treated conservatively and was discharged. Follow up examination after 2 mo revealed persistent wheeze but no respiratory distress. Computed tomography (CT) of thorax was done which revealed a small sand particle measuring 0.2 × 0.4 × 0.3 cm in left main bronchus and was removed via rigid bronchoscopy. This report underscores the importance of performing bronchoscopy in all cases of suspected foreign body aspirations.

Pulmonary interstitial emphysema (PIE) is a known complication of mechanical ventilation of preterm infants. PIE is associated with high mortality and treatment is controversial as both surgery and conservative treatment are possibilities. We present a case with a 15-month-old previously healthy child, hospitalized with severe respiratory distress. A computed tomography (CT) of the chest revealed localized PIE. He was successfully treated.

Unidirectional endobronchial valves, originally studied for potential treatment of emphysema, have emerged as a useful intervention for patients with persistent air leak from the lung. The procedure is accomplished via bronchoscopy in a patient who already has a chest tube in place for management of the air leak. It uses an occluding balloon to determine the specific airway(s) leading to the leak by impact on airflow and subsequent placement of removable valve(s) in one or more segment or subsegments to decrease flow across the leak to allow for healing of the fistula. Specific US Food and Drug Administration-approved criteria for placement and removal of these valves via a Humanitarian Device Exemption are discussed along with reported outcomes. Current Procedural Terminology codes effective for 2013 that are specific to the procedure are reviewed.

A 68-year-old man presented to the emergency department with a small pneumothorax following aggressive use of an incentive spirometer. The patient had a baseline chest radiograph consistent with emphysema. He was initially treated with oxygen in the emergency department, with resolution of his symptoms. The pneumothorax resolved spontaneously over a period of 3 days. The development of the pneumothorax was likely due to the patient's repeated forceful inspiratory maneuvers in the setting of emphysema and lung hyperinflation. Inspiratory resistive breathing can cause large negative swings in intrathoracic pressure, which may result in mechanical stress of lung tissue. This is the first report of a secondary pneumothorax associated with use of an incentive spirometer. Patients with bullous emphysema should be counseled to avoid frequent high intensity maneuvers with an incentive spirometer if the potential benefits of the procedure are marginal.

α-1-antitrypsin deficiency (A1ATD) is an under-recognized hereditary disorder associated with the premature onset of chronic obstructive pulmonary disease. There is considerable heterogeneity in the phenotypic expression of lung disease in A1ATD and the pathophysiology is complex, involving the interaction of multiple pathways. Other genetic factors that may contribute to emphysema risk in A1AT-deficient individuals are beginning to be identified. Methods of monitoring disease progression have evolved, including the use of computed tomography densitometry and biomarkers of disease activity. Progress in the development of novel treatment strategies continues, including the hope for a potential cure through the use of gene therapies. In this article, the authors review the recent advances in this field and outline potential future directions of research in A1ATD.

Several groups have described a syndrome in which idiopathic pulmonary fibrosis (IPF) coexists with pulmonary emphysema. This comes as no surprise since both diseases are associated with a history of exposure to cigarette smoke. The syndrome of combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. Physiological testing of these patients reveals preserved lung volume indices contrasted by markedly impaired diffusion capacity. The incidence of CPFE remains unknown but several case series suggest that this subgroup may comprise up to 35% of patients with IPF. CPFE is a strong determinant of associated pulmonary hypertension (PH). In addition, CPFE has major effects on measures of physiological function, exercise capacity and prognosis, and may affect the results of pulmonary fibrosis trials. Further studies are needed to ascertain the aetiology, morbidity, mortality and management of the CPFE syndrome, with or without PH, and to evaluate novel therapeutic options in CPFE.

Emphysema is characterized by destruction of alveolar walls with loss of gas exchange surface and consequent progressive dyspnea. This study aimed to evaluate the efficiency of cell therapy with bone marrow mononuclear cells (BMMC) in an animal model of elastase-induced pulmonary emphysema. Emphysema was induced in C57Bl/J6 female mice by intranasal instillation of elastase. After 21 days, the mice received bone marrow mononuclear cells from EGFP male mice with C57Bl/J6 background. The groups were assessed by comparison and statistically significant differences (p < 0.05) were observed among the groups treated with BMMC and evaluated after 7, 14 and 21 days. Analysis of the mean linear intercept (Lm) values for the different groups allowed to observe that the group treated with BMMC and evaluated after 21 days showed the most significant result. The group that received no treatment showed a statistically significant difference when compared to other groups, except the group treated and evaluated after 21 days, evidencing the efficacy of cell therapy with BMMC in pulmonary emphysema.

Pulmonary emphysema is a major manifestation of chronic obstructive pulmonary disease (COPD), which is characterized by progressive destruction of alveolar parenchyma with persistent inflammation of the small airways. Such destruction in the distal respiratory tract is irreversible and irreparable. All-trans-retinoic acid was suggested as a novel therapy for regeneration of lost alveoli in emphysema. However, profound discrepancies were evident between studies. At present, no effective therapeutic options are available that allow for the regeneration of lost alveoli in emphysematous human lungs. Recently, some reports on rodent's models have suggested the beneficial effects of various growth factors toward alveolar maintenance and repair processes.

Background: Pulmonary interstitial emphysema (PIE) is a common respiratory illness in preterm infants associated with significant morbidity and mortality for which the ventilatory management is imperfect., Objectives: To evaluate the impact of high-frequency oscillatory ventilation (HFOV) with a low oscillatory frequency and thus prolonged expiratory time in preterm infants with severe PIE., Methods: In a retrospective cohort study, preterm infants ≤30 weeks' gestation with radiological findings of severe PIE, and either high FiO2 or persistent respiratory acidosis were studied if managed on HFOV with a low frequency (5-6 Hz, inspiratory time 30%) for >24 h. Trends in physiological and ventilatory parameters were examined over the first 72 h, radiological changes noted, and in-hospital outcomes ascertained., Results: 19 cases were identified and analysed in two groups: 14 with bilateral and 5 with predominantly unilateral disease. After transition to low-frequency HFOV, physiological responses were seen in both groups, in particular a rapid and sustained improvement in oxygenation in the bilateral group (mean (SD) alveolar-arterial oxygen difference at baseline: 404 ± 206 mm Hg; 4 h post-transition: 262 ± 181 mm Hg; 72 h: 155 ± 74 mm Hg; p = 0.0003). This occurred following a reduction in mean airway pressure (mean (SD) baseline: 14 ± 3.9 cm H2O; 72 h: 12 ± 2.9 cm H2O; p = 0.011). In the unilateral group, radiological resolution of PIE was observed on re-inflation following collapse of the affected lung. Overall, 15 infants survived, including 10 of the bilateral cases (71%), and all of the unilateral cases., Conclusion: HFOV with a low oscillatory frequency may afford benefit in preterm babies with severe PIE., (Copyright © 2013 S. Karger AG, Basel.)

A preterm infant with congenital cystic adenomatoid malformation (CCAM) who developed a right-sided pulmonary air leak syndrome (pulmonary interstitial emphysema and bronchopleural fistula) following CCAM resection is reported. The pulmonary air leak syndrome was successfully ameliorated by intubating the right mainstem bronchus using a modified endotracheal tube that allowed selective ventilation of the left lung. The procedure was used successfully as rescue treatment to control the pulmonary air leak and to confirm the functional adequacy of the left lung prior to definitive operative surgery., (© 2012 The Authors. Journal of Paediatrics and Child Health © 2012 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)