Do middle-aged patients with onset of idiopathic scoliosis before the age of 10 years who have reduced pulmonary function have a risk for rapid decline - a comparative study.

BACK GROUND: Knowledge concerning pulmonary function in adult patients with onset of idiopathic scoliosis before age 10 is sparse. A long-term follow-up (FU, mean 26 years, > 12 years after treatment) of pulmonary function (PF) in patients treated with brace or surgery due to idiopathic scoliosis with onset before the age of 10 was earlier performed. To evaluate whether a more severe reduction in pulmonary function leads to more rapid deterioration within a four-year period, this study was performed. METHODS: Twenty patients with the most reduced pulmonary function and 19 out of those with normal PF found at the long-term FU were reexamined 4 years later to evaluate further changes in pulmonary function. Patients underwent spirometry and arterial blood gas analysis and answered pulmonary symptom questionnaires. RESULTS: 70% of the reduced pulmonary function group had undergone surgery vs. 26% of the normal group. The mean age (47 vs. 43 years) at this FU and curve size (37° vs. 35°) at the 26-year FU were similar. The decline in forced vital capacity (FVC) % of predicted was similar in both groups over the four-year period, from 67 to 65% in the reduced PF group vs. 96 to 94% in the normal PF group. The total lung capacity (TLC) % of predicted did not change over time in either group. No patient reported worsening dyspnea symptoms. Only one patient in the reduced PF group showed low arterial oxygen tension, 8.4 kPa, not signifying respiratory insufficiency. CONCLUSION: The age-related decline in FVC and TLC % of predicted did not differ between those with reduced and those with normal pulmonary function at the 26-year follow-up. Thus, these data do not infer increased rate of decline in the most deteriorated patients.

Inhomogeneity of ventilation in smokers and mild COPD assessed by the ratio of alveolar volume to total lung capacity.

BACKGROUND: Previous studies have shown that the ratio between alveolar volume (VA) and total lung capacity (TLC) reflects ventilation heterogeneity in subjects with chronic obstructive pulmonary disease (COPD). However, the ratio and its correlation to respiratory symptoms had to our knowledge not previously been investigated in subjects with mild chronic airflow obstruction or without airflow obstruction (normal ratio FEV1/VC). The purpose of this study was to investigate potential connection between VA/TLC and respiratory symptoms, smoking habits and lung function parameters in subjects with normal spirometry and with mild chronic airflow obstruction. METHODS: We examined 450 subjects (82 non-smokers with normal spirometry, 298 subjects who smoked or had smoked earlier but had a normal spirometry and 70 subjects with chronical airflow obstruction) with routine lung function tests and a questionnaire regarding respiratory symptoms. RESULTS: This study showed 31 out of 54 subjects with a low VA/TLC had a normal ratio FEV1/VC. Of these subjects, 58.1 % had respiratory symptoms, compared to the group with normal ratio for both VA/TLC and FEV1/VC where 35.8 % had respiratory symptoms (p-value 0.02). CONCLUSION: This study has shown that within the group of subjects with a normal ratio FEV1/VC, those with a decreased ratio VA/TLC had a higher prevalence of respiratory symptoms compared to subjects with a normal VA/TLC ratio. These findings indicate that including the ratio VA/TLC in the evaluation of a pulmonary function test assessment might increase the possibility to identify subjects with early or at risk of lung disease.

Structural and functional alterations of subjects with cement dust exposure: A longitudinal quantitative computed tomography-based study.

Cement dust exposure (CDE) can be a risk factor for pulmonary disease, causing changes in segmental airways and parenchymal lungs. This study investigates longitudinal alterations in quantitative computed tomography (CT)-based metrics due to CDE. We obtained CT-based airway structural and lung functional metrics from CDE subjects with baseline CT and follow-up CT scans performed three years later. From the CT, we extracted wall thickness (WT) and bifurcation angle (θ) at total lung capacity (TLC) and functional residual capacity (FRC), respectively. We also computed air volume (Vair), tissue volume (Vtissue), global lung shape, percentage of emphysema (Emph%), and more. Clinical measures were used to associate with CT-based metrics. Three years after their baseline, the pulmonary function tests of CDE subjects were similar or improved, but there were significant alterations in the CT-based structural and functional metrics. The follow-up CT scans showed changes in θ at most of the central airways; increased WT at the subgroup bronchi; smaller Vair at TLC at all except the right upper and lower lobes; smaller Vtissue at all lobes in TLC and FRC except for the upper lobes in FRC; smaller global lung shape; and greater Emph% at the right upper and lower lobes. CT-based structural and functional variables are more sensitive to the early identification of CDE subjects, while most clinical lung function changes were not noticeable. We speculate that the significant long-term changes in CT are uniquely observed in CDE subjects, different from smoking-induced structural changes.

Interventional bronchoscopy for chronic obstructive pulmonary disease: more than a pipe dream.

Endoscopic lung volume reduction (ELVR) is recognised in both national and international expert guidelines as one of the few additive treatments to benefit patients with advanced chronic obstructive pulmonary disease (COPD) who are otherwise receiving optimal medical and supportive care. Despite these recommendations and a growing evidence base, these procedures are not widely offered across Australia and New Zealand, and general practitioner and physician awareness of this therapy can be improved. ELVR aims to mitigate the impact of hyperinflation and gas trapping on dyspnoea and exercise intolerance in COPD. Effective ELVR is of proven benefit in improving symptoms, quality of life, lung function and survival. Several endoscopic techniques to achieve ELVR have been developed, with endobronchial valve placement to collapse a single lobe being the most widely studied and commonly practised. This review describes the physiological rationale underpinning lung volume reduction, highlights the challenges of patient selection, and provides an overview of the evidence for current and investigational endoscopic interventions for COPD.

Movement of the ribs in supine humans for small and large changes in lung volume.

An object-tracking algorithm was used on computed tomography (CT) images of the thorax from six healthy participants and nine participants with chronic obstructive pulmonary disease (COPD) to describe the movement of the ribs between the static lung volumes of functional residual capacity (FRC) and total lung capacity (TLC). The continuous motion of the ribs during tidal breathing was also described using four-dimensional CT datasets from seven participants with thoracic esophageal malignancies. Rib motion was defined relative to a local joint coordinate system where rotations about the axes that predominantly affected the anteroposterior and transverse diameters of the rib cage were referred to as pump-handle and bucket-handle movements, respectively. Between TLC and FRC, pump-handle movements were 1.8 times larger in healthy participants than in participants with COPD, in line with their 1.6 times larger inspiratory capacities. However, when rib motion was normalized to the change in lung volume, pump-handle movements were similar for healthy participants and participants with COPD. We found no differences in bucket-handle movements between participant groups before and after normalization. Pump-handle movement was the dominant rib motion between FRC and TLC, on average four times greater than bucket-handle movement in healthy participants. For expiratory tidal volume, pump-handle movements were 20% smaller than bucket-handle movements. When normalized to tidal volume and compared with inspiratory capacity, pump-handle movements were smaller and bucket-handle movements were larger during tidal breathing. The findings suggest that the pump-handle and bucket-handle components of rib motion vary for small and large changes in lung volume.NEW & NOTEWORTHY Rib movements over inspiratory capacity are comparable for healthy participants and participants with chronic obstructive pulmonary disease when normalized to the change in lung volume. The kinematics of the ribs during tidal breathing were described from four-dimensional computed tomography images. For large changes in lung volume with inspiratory capacity, pump-handle movements of the ribs are four times greater than bucket-handle movements, whereas at tidal volume, pump-handle movements are 20% smaller than bucket-handle movements.

Acute effect of inhaled iloprost on exercise dynamic hyperinflation in COPD patients: A randomized crossover study.

BACKGROUND AND OBJECTIVE: We tested whether the prostacyclin analog inhaled iloprost modulates dead space, dynamic hyperinflation (DH), and systemic inflammation/oxidative stress during maximal exercise in subjects with chronic obstructive pulmonary disease (COPD) who were not selected based on pulmonary hypertension (PH). METHODS: Twenty-four COPD patients with moderate-severe obstruction (age 59 ± 7 years, FEV1 53 ± 13% predicted) participated in a randomized, double-blind, placebo-controlled crossover trial. Each subject received a single nebulized dose of 5.0 µg iloprost or placebo on non-consecutive days followed by maximal cardiopulmonary exercise tests. The primary outcome was DH quantified by end-expiratory lung volume/total lung capacity ratio (EELV/TLC) at metabolic isotime. RESULTS: Inhaled iloprost was well-tolerated and reduced submaximal alveolar dead-space fraction but did not significantly reduce DH (0.70 ± 0.09 vs 0.69 ± 0.07 following placebo and iloprost, respectively, p = 0.38). Maximal exercise time (9.1 ± 2.3 vs 9.3 ± 2.2 min, p = 0.31) and peak oxygen uptake (17.4 ± 6.3 vs 17.9 ± 6.9 mL/kg/min, p = 0.30) were not significantly different following placebo versus iloprost. CONCLUSIONS: A single dose of inhaled iloprost was safe and reduced alveolar dead space fraction; however, it was not efficacious in modulating DH or improving exercise capacity in COPD patients who were not selected for the presence of PH.

Effects of Inspiratory Muscle Training Using an Electronic Device on Patients Undergoing Cardiac Surgery: A Randomized Controlled Trial

Abstract Background Cardiac surgery causes pathophysiological changes that favor the occurrence of pulmonary and functional complications. Objective To investigate the effects of inspiratory muscle training (IMT) with an electronic device on patients undergoing cardiac surgery. Methods A randomized controlled trial was conducted with 30 adult patients undergoing elective cardiac surgery. A control group (CG) received conventional physical therapy care, and an intervention group (IG) received IMT using the POWERbreathe K5® electronic device. Two daily sessions of physical therapy were performed at the intensive care unit and one daily session at the ward until the sixth postoperative day. The following variables were measured preoperatively and on the sixth postoperative day, in both groups: inspiratory muscle strength, dynamic inspiratory muscle strength, and peak inspiratory flow. Data distribution was evaluated by the Shapiro-Wilk test. Analysis of variance was used, and the results were considered statistically significant when p < 0.05. Results Maximal inspiratory pressure (71.7 ± 17.1 cmH2O vs 63.3 ± 21.3 cmH2O; p = 0.11], S-index (52.61 ± 18.61 vs 51.08 ± 20.71), and peak inspiratory flow [(2.94 ± 1.09 vs 2.79 ± 1.26)] were maintained in the IG but had a significant reduction in the CG. Conclusion IMT performed with an electronic device was effective at maintaining inspiratory muscle strength, dynamic inspiratory muscle strength, and peak inspiratory flow when compared to conventional physical therapy. Int J Cardiovasc Sci. 2020; [online].ahead print, PP.0-0

Assessing the applicability of the new Global Lung Function Initiative reference values for the diffusing capacity of the lung for carbon monoxide in a large population set.

BACKGROUND: The single-breath diffusing capacity of the lung for carbon monoxide (DLCO) interpretation needs the comparison of measured values to reference values. In 2017, the Global Lung Function Initiative published new reference values (GLI-2017) for DLCO, alveolar volume (VA) and transfer coefficient of the lung for carbon monoxide (KCO). We aimed to assess the applicability of GLI-2017 reference values for DLCO on a large population by comparing them to the European Community of Steel and Coal equations of 1993 (ECSC-93) widely used. METHODS: In this retrospective study, spirometric indices, total lung capacity, DLCO, VA and KCO were measured in adults classified in 5 groups (controls, asthma, chronic bronchitis, cystic fibrosis, and interstitial lung diseases (ILD)). Statistical analysis comparing the 2 equations sets were stratified by sex. RESULTS: 4180 tests were included. GLI-2017 z-scores of the 3 DLCO indices of the controls (n = 150) are nearer to 0 (expected value in a normal population) than ECSC-93 z-scores. All groups combined, in both genders, DLCO GLI-2017 z-scores and %predicted are significantly higher than ECSC z-scores and %predicted. In the ILD group, differences between the 2 equation sets depend on the DLCO impairment severity: GLI-2017 z-scores are higher than ECSC z-scores in patients with no or "mild" decrease in DLCO, but are lower in "moderate" or "severe" decrease. CONCLUSION: GLI-2017 reference values for DLCO are more suitable to our population and influence the diagnostic criteria and severity definition of several lung diseases.

Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors.

BACKGROUND: Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare form of idiopathic interstitial pneumonia that is characterized by predominantly upper lobe pleural and subpleural lung parenchymal fibrosis. Pneumothorax is one of the major respiratory complications in PPFE patients; however, its clinical features are poorly understood. OBJECTIVE: We aimed to investigate the complication of pneumothorax in patients with idiopathic PPFE. METHODS: A retrospective multicenter study involving 89 patients who had been diagnosed with idiopathic PPFE was conducted. We investigated the cumulative incidence, clinical features, and risk factors of pneumothorax after the diagnosis of idiopathic PPFE. RESULTS: Pneumothorax developed in 53 patients (59.6%) with 120 events during the observation period (41.8 ± 35.0 months). The cumulative incidence of pneumothorax was 24.8, 44.9, and 53.9% at 1, 2, and 3 years, respectively. Most events of pneumothorax were asymptomatic (n = 85; 70.8%) and small in size (n = 92; 76.7%); 30 patients (56.6%) had recurrent pneumothorax. Chest drainage was required in 23 pneumothorax events (19.2%), and a persistent air leak was observed in 13 (56.5%). Patients with pneumothorax were predominantly male and frequently had pathological diagnoses of PPFE and prior history of pneumothorax and corticosteroid use; they also had significantly poorer survival than those without pneumothorax (log-rank test; p = 0.001). Multivariate analysis revealed that a higher residual volume/total lung capacity ratio was significantly associated with the development of pneumothorax after the diagnosis. CONCLUSION: Pneumothorax is often asymptomatic and recurrent in patients with idiopathic PPFE, leading to poor outcomes in some cases.

Patient-directed Volume Reduction for Emphysema: Sequential Surgical and Endobronchial Techniques.

BACKGROUND: Lung volume reduction (LVR) surgery has traditionally been performed as a 1-stage bilateral procedure or staged at a predetermined interval. However to maximize the overall benefit we have allowed the patient to determine the timing of further interventions and have added endobronchial LVR into the protocol. We have reviewed the long-term outcome. METHODS: Three hundred thirty-one LVR procedures were performed on 254 patients (median age, 61 years [range, 23-79]) with baseline predicted lung function of (mean ± SD) forced expiratory volume in 1 second 28% ± 11% and residual volume 253% ± 53%. The initial procedure was by video-assisted thoracoscopic surgery in 236 patients (unilateral, 227; bilateral, 9), by open surgery in 5, and by endobronchial valve insertion in 13. Sixty-four patients received a second and 13 a third LVR procedure. The median time interval between first and third stage was 5.8 years (range, 1.9-10) RESULTS: In the subgroup of patients who underwent staged procedures there was a significant improvement in predicted forced expiratory volume in 1 second from 28% at baseline to 34% up to 6 years. There was sustained reduction in static lung volumes up to 8 years: Predicted residual volume remained reduced from 259% to 189%. There were sustained improvements over baseline in health status: EuroQol-5D improved from 50 ± 26 to 62 ± 23 (P < .01) for up to 5 years and the Short Form 36-item questionnaire for up to 9 years. Overall 30-day mortality was 3%. Median survival was 5.6 years (95% confidence interval, 4.7-6.9). CONCLUSIONS: A program of staged unilateral procedures of LVR has resulted in sustained benefits for up to 9 years in physiology and health status.

Sintomas de Fadiga e Força Muscular Respiratória de Pacientes Onco-hematológicos em Quimioterapia / Fatigue Symptoms and Respiratory Muscle Strength of Onco-hematological Patients in Chemotherapy / Síntomas de Fatiga y Fuerza de los Músculos Respiratorios de Pacientes Onco-hematológicos en Quimioterapia

Introdução: Quando exposto à quimioterapia, o paciente onco-hematológico está suscetível a várias complicações físicas e respiratórias, associadas aos efeitos colaterais dessas substâncias. Objetivo: Avaliar o impacto de força muscular respiratória quando comparada com os níveis de normalidade e sintomatologia de fadiga, durante recebimento do tratamento quimioterapêutico de pacientes onco-hematológicos. Método: Pesquisa observacional do tipo transversal, realizada por meio de questionário referente aos dados sociodemográficos e de manovacuometria com dispositivo analógico. Resultados: A pesquisa foi constituída por uma população composta de 19 pessoas, 57,9% mulheres e 42,9% homens. A idade média foi de 51,3 anos. A predominância diagnóstica foi leucemia, seguida por linfoma e mieloma. Entre as queixas, a dispneia esteve presente em 31,6% dos casos, sendo a quimioterapia o protocolo escolhido para todos os participantes. Durante a avaliação, 52,6% relataram cansaço e, entre eles, 70% relataram sentir-se melhor quando em repouso, seguidos por 50% impedidos de realizar suas atividades diárias. Ex-fumantes representaram 70% da população pesquisada e 84,2% não praticavam atividades físicas. Na amostra, 62,4% apresentaram frequência respiratória normal, predominando o padrão respiratório apical e o tórax longilíneo. Foram observados resultados significativos na diminuição de Pimáx e Pemáx, com valores estatisticamente conclusivos de p<0,001 nas duas variáveis. Conclusão: O quadro da doença, os tratamentos utilizados e as internações a que essa população foi submetida provocaram a diminuição da força muscular respiratória e o aumento dos sintomas de fadiga.

Introduction: When exposed to chemotherapy, the onco-hematological patient is susceptible to several physical and respiratory complications, associated with side effects of these substances. Objective: Evaluate the impact on respiratory muscle strength when compared to the levels of normality and symptoms of fatigue of onco-hematological patients during chemotherapy treatment. Method: Observational cross-sectional study performed trough a social demographic questionnaire and manovacuometry with analogical device. Results: The study population consisted of 19 subjects, 57.9% women and 42.9% men. The average age was 51.3 years old. The predominant diagnoses were leukemia, followed by lymphoma and myeloma. Among the complaints, dyspnea was present in 31.6% of the cases, chemotherapy was the protocol of choice for all the participants. During the evaluation, 52.6% reported tiredness, and among them, 70%, claimed they feel better when at rest, followed by 50% precluded from performing their daily activities. Ex-smokers represented 70% of the study population and 84.2% did not practice physical activities. 62.4 % of the sample presented normal respiratory frequency, with the apical breathing pattern and predominant slender thorax. Significant results were observed in decreasing MIP and MEP with statistically conclusive values of p<0.001 for the two variables. Conclusion: The disease, the treatments and the hospitalizations this population was submitted provoked the reduction of the respiratory muscle strength and increase of the fatigue symptoms

Introducción: Cuando se expone a quimioterapia, el paciente oncohematológico es susceptible a diversas complicaciones físicas y respiratorias, asociadas a los efectos secundarios de estas sustancias. Objetivo: Evaluar el impacto de la fuerza de los músculos respiratorios en comparación con los niveles de normalidad y síntomas de fatiga, mientras reciben tratamiento de quimioterapia de pacientes oncohematológicos. Método: Investigación observacional transversal, realizada mediante un cuestionario referente a datos sociodemográficos y realizando manovacuometría con dispositivo analógico. Resultados: La investigación consistió en una población compuesta por 19 personas, 57,9% mujeres y 42,9% hombres. La edad media fue de 51,3 años. El predominio diagnóstico fue la leucemia, seguida del linfoma y el mieloma. Entre las quejas, la disnea estuvo presente en el 31,6% de los casos, siendo la quimioterapia el protocolo elegido para todos los participantes. Durante la evaluación, el 52,6% refirió cansancio y, entre ellos, el 70% refirió sentirse mejor en reposo, seguido del 50% incapaz de realizar sus actividades diarias. Los exfumadores representaron el 70% de la población encuestada y el 84,2% no practicaba actividad física. En la muestra, el 62,4% tenía frecuencia respiratoria normal, con predominio de patrón respiratorio apical y tórax longilineal. Se observaron resultados significativos en la disminución de Pimax y Pmax, con valores estadísticamente concluyentes de p<0,001 en ambas variables. Conclusión: Debido a la enfermedad, los tratamientos utilizados y las hospitalizaciones a las que esta población fueron sometidos provocaron la disminución de la fuerza de los músculos respiratorios y aumento de los síntomas de fatiga

The effects of lung volume reduction treatment on diffusing capacity and gas exchange.

Lung volume reduction (LVR) treatment in patients with severe emphysema has been shown to have a positive effect on hyperinflation, expiratory flow, exercise capacity and quality of life. However, the effects on diffusing capacity of the lungs and gas exchange are less clear. In this review, the possible mechanisms by which LVR treatment can affect diffusing capacity of the lung for carbon monoxide (D LCO) and arterial gas parameters are discussed, the use of D LCO in LVR treatment is evaluated and other diagnostic techniques reflecting diffusing capacity and regional ventilation (V')/perfusion (Q') mismatch are considered.A systematic review of the literature was performed for studies reporting on D LCO and arterial blood gas parameters before and after LVR surgery or endoscopic LVR with endobronchial valves (EBV). D LCO after these LVR treatments improved (40 studies, n=1855) and the mean absolute change from baseline in % predicted D LCO was +5.7% (range -4.6% to +29%), with no real change in blood gas parameters. Improvement in V' inhomogeneity and V'/Q' mismatch are plausible explanations for the improvement in D LCO after LVR treatment.

Roxadustat (FG-4592) accelerates pulmonary growth, development, and function in a compensatory lung growth model.

Children with hypoplastic lung disease associated with congenital diaphragmatic hernia (CDH) continue to suffer significant morbidity and mortality secondary to progressive pulmonary disease. Current management of CDH is primarily supportive and mortality rates of the most severely affected children have remained unchanged in the last few decades. Previous work in our lab has demonstrated the importance of vascular endothelial growth factor (VEGF)-mediated angiogenesis in accelerating compensatory lung growth. In this study, we evaluated the potential for Roxadustat (FG-4592), a prolyl hydroxylase inhibitor known to increase endogenous VEGF, in accelerating compensatory lung growth. Treatment with Roxadustat increased lung volume, total lung capacity, alveolarization, and exercise tolerance compared to controls following left pneumonectomy. However, this effect was likely modulated not only by increased VEGF, but rather also by decreased pigment epithelium-derived factor (PEDF), an anti-angiogenic factor. Furthermore, this mechanism of action may be specific to Roxadustat. Vadadustat (AKB-6548), a structurally similar prolyl hydroxylase inhibitor, did not demonstrate accelerated compensatory lung growth or decreased PEDF expression following left pneumonectomy. Given that Roxadustat is already in Phase III clinical studies for the treatment of chronic kidney disease-associated anemia with minimal side effects, its use for the treatment of pulmonary hypoplasia could potentially proceed expeditiously.

[A study on the end criteria of forced vital capacity curve in adults].

Objective: To explore the end criteria of forced vital capacity(FVC) curve in adults. Methods: A multicenter cross-sectional study was performed in Zhongshan Hospital Affiliated to Fudan University, the First Affiliated Hospital of Fujian Medical University, and the Third Affiliated Hospital of Inner Mongolia Medical University from January 2017 to August 2017. A consecutive sample of subjects who completed the spirometry test and FVC curves met end criteria of no volume change (<0.025 L) for ≥ 1 s were qualified in this study. Subjects were divided into a normal group (n=610), an obstructive group (n=536), and a restrictive group(n=306) according to pulmonary function test results. The FET values in different groups were compared. The side effects in the 3 groups and the diagnostic accuracy, specificity and security of different FET in the obstructive group were assessed. Results: The FET values of the normal group, the obstructive group, and the restricted group were (4.00±1.07) s, (8.08±1.56) s and (2.97±0.76) s respectively, and the 95% CI of FET in the 3 groups were between 3.88-4.12 s, 7.02-10.14 and 2.21- 3.73 s (F=2 263.80, P<0.01). When the exhalation platform was used as the standard of FVC curve, the adverse reaction rate in the normal group and the restricted group were 1.1% and 1.3% respectively, lower than the rate of 17.2% in the obstructive group (χ(2)=92.73, χ(2)=48.49 respectively; all P<0.05). In the obstructive group, 7 s as the ending criterion had similar incidence of adverse reactions to 6 s (χ(2)=0.01, P=0.93). With further extension of expiration time, the incidence of adverse reactions increased significantly. In the obstructive group, the sensitivity of FEV(1)/FEV(7) was 99.25%, higher than that at FEV(1)/FEV(6) (χ(2)=4.06, P=0.04), and the specificity of diagnosis was very similar and 100%. Conclusions: FET was variable in subjects with different lung function status. It is not appropriate to use a fixed FET≥ 6 s as the end criterion of spirometry for adults. For patients with normal lung function or restrictive lung function defect, exhalation platform should be used as the end of exhalation standard. For patients with obstructive lung function defect, an FET of up to 7 s is appropriate.

Determining Static Hyperinflation in Patients with Severe Emphysema: Relation Between Lung Function Parameters and Patient-Related Outcomes.

BACKGROUND: Bronchoscopic lung volume reduction techniques are minor invasive treatment modalities for severely hyperinflated emphysema patients. The severity of static lung hyperinflation determines eligibility and success rate for these treatments. However, it is not exactly known what parameter should be used to optimally reflect hyperinflation. Commonly used parameters are residual volume (RV) and the RV/Total lung capacity (TLC) ratio. Other parameters reflecting hyperinflation are Inspiratory Capacity/TLC and forced vital capacity. OBJECTIVES: To define which of these function parameters is the most optimal reflection of hyperinflationin in relation to patient-related outcomes. METHODS: In a retrospective cohort study, data from measurements during baseline visits of eight studies were pooled. Primary outcomes were RV/TLC ratio and RV as percentage of predicted (RV%pred), both measured by bodyplethysmography, compared to the patient-related outcome variables: 6-min walk distance (6MWD), the St. George's Respiratory Questionnaire (SGRQ), and the modified Medical Research Council (mMRC). RESULTS: Two hundred seventy-four COPD patients (mean age 59 years; 66% female), FEV1 0.74 ± 0.28 L, RV 4.94 ± 1.06 L, 6MWD of 339 ± 95 m, were included in the analysis. Significant correlations (all p < 0.01) were found between RV%pred and 6MWD (r = - 0.358), SGRQ (r = 0.184), and mMRC (r = 0.228). Also, there was a significant correlation between RV/TLC ratio and 6MWD (r = - 0.563), SGRQ (r = 0.289) and mMRC (r = 0.354). Linear regression analyses showed that RV/TLC ratio was a better predictor of patient outcomes than RV%pred. CONCLUSION: This study demonstrates that both RV/TLC ratio and RV%pred are relevant indicators of hyperinflation in patients with severe emphysema in relation to patient-related outcomes. RV/TLC ratio is more strongly related to the patient-related outcomes than RV%pred.

Application of autologous SOX9+ airway basal cells in patients with bronchiectasis.

INTRODUCTION: Bronchiectasis is a common condition and a leading cause of respiratory morbidity and mortality. The treatment method for bronchiectasis is mainly symptomatic treatment or surgery; however, this condition is extremely prone to recurrence. OBJECTIVES: To preliminarily evaluate the safety and efficacy of applying SOX9+ autologous airway basal cells (BCs) in patients with bronchiectasis. METHODS: SOX9+ BCs were isolated from microscale tissue of a grade 3-5 bronchus by bronchoscopic brushing and expanded in vitro for approximately 4 weeks. Subsequently, the autologous SOX9+ BCs were transplanted into the diseased bronchus to treat patients with bronchiectasis. RESULTS: The forced expiratory volume in1 second (FEV1)%, forced vital capacity (FVC)%, total lung capacity (TLC)%, residual volume (RV)% and RV/TLC ratio of predicted value in patients with bronchiectasis were improved at 4, 12, 24 and 48 weeks after cell transplantation, although the differences were not statistically significant (P > .05). Chest CT scans showed that the lesions in the pulmonary segment had not progressed at 4, 12 and 24 weeks after transplantation. No patients died during the follow-up. At 4, 12 and 24 weeks after transplantation, routine blood tests, liver function tests, renal function tests and myocardial enzymatic indexes were normal (P > .05). CONCLUSION: Transplantation of autologous SOX9+ BCs has positive effects and is safe for patients with bronchiectasis.

Quantitative CT-based structural alterations of segmental airways in cement dust-exposed subjects.

BACKGROUND: Dust exposure has been reported as a risk factor of pulmonary disease, leading to alterations of segmental airways and parenchymal lungs. This study aims to investigate alterations of quantitative computed tomography (QCT)-based airway structural and functional metrics due to cement-dust exposure. METHODS: To reduce confounding factors, subjects with normal spirometry without fibrosis, asthma and pneumonia histories were only selected, and a propensity score matching was applied to match age, sex, height, smoking status, and pack-years. Thus, from a larger data set (N = 609), only 41 cement dust-exposed subjects were compared with 164 non-cement dust-exposed subjects. QCT imaging metrics of airway hydraulic diameter (Dh), wall thickness (WT), and bifurcation angle (θ) were extracted at total lung capacity (TLC) and functional residual capacity (FRC), along with their deformation ratios between TLC and FRC. RESULTS: In TLC scan, dust-exposed subjects showed a decrease of Dh (airway narrowing) especially at lower-lobes (p < 0.05), an increase of WT (wall thickening) at all segmental airways (p < 0.05), and an alteration of θ at most of the central airways (p < 0.001) compared with non-dust-exposed subjects. Furthermore, dust-exposed subjects had smaller deformation ratios of WT at the segmental airways (p < 0.05) and θ at the right main bronchi and left main bronchi (p < 0.01), indicating airway stiffness. CONCLUSIONS: Dust-exposed subjects with normal spirometry demonstrated airway narrowing at lower-lobes, wall thickening at all segmental airways, a different bifurcation angle at central airways, and a loss of airway wall elasticity at lower-lobes. The airway structural alterations may indicate different airway pathophysiology due to cement dusts.

The Efficacy of Lung Volume Reduction Coil Treatment in Patients with Severe Chronic Obstructive Pulmonary Disease (COPD) Type II Respiratory Failure.

Purpose: Emphysema is a progressive and irreversible disease, proceeding with the decrease in elastic recoil which is connected to tissue damage caused by chronic inflammation. Lung volume reduction coil (LVRC) method in patients with an advanced level of emphysema and irresponsive to medical treatment is shown to provide increase in lung volumes and exercise capacity, decrease in dyspnea, and increase in quality of life. The purpose of this study is to reveal that LVRC treatment is also efficient in severe COPD patients with hypercapnic respiratory failure. Patients and Methods: Eleven cases with severe COPD and emphysema were included in the study. LVRC treatment method was applied in upper lobes of both lungs in patients with severe COPD (FEV1 < %45) and Type-2 respiratory insufficiency (PCO2 55-80 mmHg) who were having medical treatment and CPAP treatment. The patients were followed up for a period of twelve months using arterial blood gas analysis. Results: Beginning with the first month of the LVRC treatment, PCO2 levels were found to be significantly decreased in all patients using arterial blood gas analysis. Conclusion: LVRC method can provide physiological and functional recovery and progress in quality of life in severe COPD cases. It is demonstrated that LVRC treatment caused significant decreases in carbon dioxide levels as well as causing improvement in life quality and respiratory function tests in the patients with hypercapnic respiratory failure.

Assessment of early lung disease in young children with CF: A comparison between pressure-controlled and free-breathing chest computed tomography.

BACKGROUND: Chest computed tomography (CT) in children with cystic fibrosis (CF) is sensitive in detecting early airways disease. The pressure-controlled CT-protocol combines a total lung capacity scan (TLC PC-CT) with a near functional residual capacity scan (FRC PC-CT) under general anesthesia, while another CT-protocol is acquired during free breathing (FB-CT) near functional residual capacity. The aim of this study was to evaluate the sensitivity in detecting airways disease of both protocols in two cohorts. METHODS: Routine PC-CTs (Princess Margaret Children's Hospital) and FB-CTs (Erasmus MC-Sophia Children's Hospital) were retrospectively collected from CF children aged 2 to 6 years. Total airways disease (%disease), bronchiectasis (%Bx), and low attenuation regions (%LAR) were scored on CTs using the Perth-Rotterdam annotated grid morphometric analysis-CF method. The Wilcoxon signed-rank test was used for differences between TLC and FRC PC-CTs and the Wilcoxon rank-sum test for differences between FRC PC-CTs and FB-CTs. RESULTS: Fifty patients with PC-CTs (21 male, aged 2.5-5.5 years) and 42 patients with FB-CTs (26 male, aged 2.3-6.8 years) were included. %Disease was higher on TLC PC-CTs compared with FRC PC-CTs (median 4.51 vs 2.49; P < .001). %Disease and %Bx were not significantly different between TLC PC-CTs and FB-CTs (median 4.51% vs 3.75%; P = .143 and 0.52% vs 0.57%; P = .849). %Disease, %Bx, and %LAR were not significantly different between FRC PC-CTs and FB-CTs (median 2.49% vs 3.75%; P = .055, 0.54% vs 0.57%; P = .797, and 2.49% vs 1.53%; P = .448). CONCLUSIONS: Our data suggest that FRC PC-CTs are less sensitive than TLC PC-CTs and that FB-CTs have similar sensitivity to PC-CTs in detecting lung disease. FB-CTs seem to be a viable alternative for PC-CTs to track CF lung disease in young patients with CF.

Thoracic mobility and its relation to pulmonary function and rib-cage deformity in patients with early onset idiopathic scoliosis: a long-term follow-up.

STUDY DESIGN: A group of adult patients with idiopathic scoliosis, diagnosed before the age of ten, at a mean of 26.5 years after treatment with either brace or surgery during childhood and adolescence attended a clinical follow-up. OBJECTIVES: To evaluate the relation between thoracic mobility, rib-cage deformity, and pulmonary function. Long-term studies of pulmonary function in relation to thoracic mobility after treatment in this patient group have not been published. METHODS: A total of 106 patients, 57 braced and 49 operated patients, attended the follow-up. We examined thoracic mobility (range of motion of the thoracic spine, thorax expansion, and breathing movements) and rib-cage deformity (curve size and trunk deformity) as well as pulmonary function, especially total lung capacity (TLC). Respiratory muscle strength was evaluated in a subgroup. RESULTS: Thoracic range of motion was significantly less among the surgically treated patients compared with both the brace-treated and comparison group. Thorax expansion and breathing movements during maximal breathing were significantly reduced in the scoliotic patients compared with the reference values, with no significant differences between the treatment groups. The brace-treated group had better pulmonary function than the operated group, as measured by the TLC, forced vital capacity (FVC), and forced expiratory volume in one second (FEV1) (percentage of predicted values). The respiratory muscle strength was significantly lower only in the surgically treated patients when compared with reference values. The results of a multivariate analysis revealed that the strongest factors explaining TLC percentage of predicted were gender, brace model, and smoking habits. CONCLUSIONS: Thoracic mobility was significantly reduced at mean 26.5 years after completed treatment in both brace-treated and surgically treated patients with early onset scoliosis, compared with the reference values, which did not influence TLC as strongly as gender, brace model, and smoking habits. LEVELS OF EVIDENCE: Level III.