Assessment of bronchodilator response in preschoolers: A systematic review.

BACKGROUND: Several techniques can be used to assess bronchodilator response (BDR) in preschool-aged children, including spirometry, respiratory oscillometry, the interrupter technique, and specific airway resistance. However, there has not been a systematic comparison of BDR thresholds across studies yet. METHODS: A systematic review was performed on all studies up to May 2023 measuring a bronchodilator effect in children 2-6 years old using one of these techniques (PROSPERO CRD42021264659). Studies were identified using MEDLINE, Cochrane, EMBASE, CINAHL via EBSCO, Web of Science databases, and reference lists of relevant manuscripts. RESULTS: Of 1224 screened studies, 43 were included. Over 85% were from predominantly European ancestry populations, and only 22 studies (51.2%) calculated a BDR cutoff based on a healthy control group. Five studies included triplicate testing with a placebo to account for the within-subject intrasession repeatability. A relative BDR was most consistently reported by the included studies (95%) but varied widely across all techniques. Various statistical methods were used to define a BDR, with six studies using receiver operating characteristic analyses to measure the discriminative power to distinguish healthy from wheezy and asthmatic children. CONCLUSION: A BDR in 2- to 6-year-olds cannot be universally defined based on the reviewed literature due to inconsistent methodology and cutoff calculations. Further studies incorporating robust methods using either distribution-based or clinical anchor-based approaches to define BDR are required.

Assessment of Treatment Response in Patients With Severe Asthma Using Visual and Quantitative Analysis of Chest CT.

OBJECTIVE: To evaluate the role of visual and quantitative chest CT parameters in assessing treatment response in patients with severe asthma. MATERIALS AND METHODS: Korean participants enrolled in a prospective multicenter study, named the Precision Medicine Intervention in Severe Asthma study, from May 2020 to August 2021, underwent baseline and follow-up chest CT scans (inspiration/expiration) 10-12 months apart, before and after biologic treatment. Two radiologists scored bronchiectasis severity and mucus plugging extent. Quantitative parameters were obtained from each CT scan as follows: normal lung area (normal), air trapping without emphysema (AT without emph), air trapping with emphysema (AT with emph), and airway (total branch count, Pi10). Clinical parameters, including pulmonary function tests (forced expiratory volume in 1 s [FEV1] and FEV1/forced vital capacity [FVC]), sputum and blood eosinophil count, were assessed at initial and follow-up stages. Changes in CT parameters were correlated with changes in clinical parameters using Pearson or Spearman correlation. RESULTS: Thirty-four participants (female:male, 20:14; median age, 50.5 years) diagnosed with severe asthma from three centers were included. Changes in the bronchiectasis and mucus plugging extent scores were negatively correlated with changes in FEV1 and FEV1/FVC (ρ = from -0.544 to -0.368, all P < 0.05). Changes in quantitative CT parameters were correlated with changes in FEV1 (normal, r = 0.373 [P = 0.030], AT without emph, r = -0.351 [P = 0.042]), FEV1/FVC (normal, r = 0.390 [P = 0.022], AT without emph, r = -0.370 [P = 0.031]). Changes in total branch count were positively correlated with changes in FEV1 (r = 0.349 [P = 0.043]). There was no correlation between changes in Pi10 and the clinical parameters (P > 0.05). CONCLUSION: Visual and quantitative CT parameters of normal, AT without emph, and total branch count may be effective for evaluating treatment response in patients with severe asthma.

Functional Assessment of Stable Chronic Obstructive Pulmonary Disease Patients Using Squat-to-stand Test.

Chronic obstructive pulmonary disease (COPD) is a preventable and treatable disease of airflow limitation that is not fully reversible. COPD affects the lungs and produces significant systemic consequences. In Bangladesh, the prevalence of COPD after 40 years of age is 21.24% and the general population is 4.3%. COPD leads to a sedentary life, which reduces the functional status of the individual. Functional status assessment is vital for appropriate therapy and rehabilitation programs in COPD patients. A Sit-to-stand test (STST) has been proposed as a better alternative to 6MWT, but a Squat-to-stand test (SqTST) to test their ability to stand from the squatting position will be more appropriate in rural patients. This study was conducted using a cross-sectional observational design from July 2020 and September 2021 in the Department of Respiratory Medicine at the National Institute of Diseases of the Chest and Hospital, Bangladesh. Sixty (60) diagnosed cases of COPD patients were enrolled in this study. Severities of airflow obstruction according to GOLD were categorized on the basis of post-bronchodilator FEV1 by spirometry. SqTST was performed on all patients, and functional status was recorded. All data were collected using a preformed questionnaire. Statistical analyses of the findings were carried out using SPSS version 23.0. In this study, the majority of 21(35.0%) patients had very severe COPD, and almost half (48.3%) of the patients had abnormal SqTST. A significant relation was found between the severity of COPD with SqTST (p=0.001). Based on the receiver-operator characteristic (ROC) curve, SqTST had an area under curve 0.901. SqTST had 82.1% sensitivity, 85.7% specificity, 83.3% accuracy, 91.4% positive predictive value, and 72.0% negative predictive value to find severe COPD cases in stable COPD patients. ROC was constructed using SqTST, which gave a cut-off value <7.0, with 82.1% sensitivity and 85.7% specificity for predicting severe COPD. From this study, it may be concluded that SqTST is a clinically useful tool to assess the functional status of stable COPD patients.

Implications of Race Adjustment in Lung-Function Equations.

BACKGROUND: Adjustment for race is discouraged in lung-function testing, but the implications of adopting race-neutral equations have not been comprehensively quantified. METHODS: We obtained longitudinal data from 369,077 participants in the National Health and Nutrition Examination Survey, U.K. Biobank, the Multi-Ethnic Study of Atherosclerosis, and the Organ Procurement and Transplantation Network. Using these data, we compared the race-based 2012 Global Lung Function Initiative (GLI-2012) equations with race-neutral equations introduced in 2022 (GLI-Global). Evaluated outcomes included national projections of clinical, occupational, and financial reclassifications; individual lung-allocation scores for transplantation priority; and concordance statistics (C statistics) for clinical prediction tasks. RESULTS: Among the 249 million persons in the United States between 6 and 79 years of age who are able to produce high-quality spirometric results, the use of GLI-Global equations may reclassify ventilatory impairment for 12.5 million persons, medical impairment ratings for 8.16 million, occupational eligibility for 2.28 million, grading of chronic obstructive pulmonary disease for 2.05 million, and military disability compensation for 413,000. These potential changes differed according to race; for example, classifications of nonobstructive ventilatory impairment may change dramatically, increasing 141% (95% confidence interval [CI], 113 to 169) among Black persons and decreasing 69% (95% CI, 63 to 74) among White persons. Annual disability payments may increase by more than $1 billion among Black veterans and decrease by $0.5 billion among White veterans. GLI-2012 and GLI-Global equations had similar discriminative accuracy with regard to respiratory symptoms, health care utilization, new-onset disease, death from any cause, death related to respiratory disease, and death among persons on a transplant waiting list, with differences in C statistics ranging from -0.008 to 0.011. CONCLUSIONS: The use of race-based and race-neutral equations generated similarly accurate predictions of respiratory outcomes but assigned different disease classifications, occupational eligibility, and disability compensation for millions of persons, with effects diverging according to race. (Funded by the National Heart Lung and Blood Institute and the National Institute of Environmental Health Sciences.).

Race Adjustment of Pulmonary Function Tests in the Diagnosis and Management of COPD: A Scoping Review.

Aim: Increasing evidence suggests that the inclusion of self-identified race in clinical decision algorithms may perpetuate longstanding inequities. Until recently, most pulmonary function tests utilized separate reference equations that are race/ethnicity based. Purpose: We assess the magnitude and scope of the available literature on the negative impact of race-based pulmonary function prediction equations on relevant outcomes in African Americans with COPD. Methods: We performed a scoping review utilizing an English language search on PubMed/Medline, Embase, Scopus, and Web of Science in September 2022 and updated it in December 2023. We searched for publications regarding the effect of race-specific vs race-neutral, race-free, or race-reversed lung function testing algorithms on the diagnosis of COPD and COPD-related physiologic and functional measures. Joanna Briggs Institute (JBI) guidelines were utilized for this scoping review. Eligibility criteria: The search was restricted to adults with COPD. We excluded publications on other lung disorders, non-English language publications, or studies that did not include African Americans. The search identified publications. Ultimately, six peer-reviewed publications and four conference abstracts were selected for this review. Results: Removal of race from lung function prediction equations often had opposite effects in African Americans and Whites, specifically regarding the severity of lung function impairment. Symptoms and objective findings were better aligned when race-specific reference values were not used. Race-neutral prediction algorithms uniformly resulted in reclassifying severity in the African Americans studied. Conclusion: The limited literature does not support the use of race-based lung function prediction equations. However, this assertion does not provide guidance for every specific clinical situation. For African Americans with COPD, the use of race-based prediction equations appears to fall short in enhancing diagnostic accuracy, classifying severity of impairment, or predicting subsequent clinical events. We do not have information comparing race-neutral vs race-based algorithms on prediction of progression of COPD. We conclude that the elimination of race-based reference values potentially reduces underestimation of disease severity in African Americans with COPD.

Pulmonary function assessment among conventional and organic cotton farmers exposed to pesticides in the Central-West region of Burkina Faso.

BACKGROUND: Respiratory diseases have been associated with the exposure of populations to some environmental pollutants such as pesticides. To assess effects of pesticides on farmers' respiratory health, this study aimed to evaluate the pulmonary function of cotton farmers exposed to synthetic and natural pesticides in the Central-West region of Burkina Faso. METHODS: A cross-sectional study was conducted from June to July 2022 among 281 conventional and 189 organic cotton farmers. After collecting information on pesticide use conditions, pulmonary function tests (PFTs) were performed on each farmer according to the American Thoracic Society/European Respiratory Society guidelines, in order to assess chronic respiratory effects among cotton producers. Binary logistic regression was used to assess factors associated with the occurrence of ventilatory changes. RESULTS: Both conventional and organic cotton farmers reported similar chronic respiratory symptoms in different proportions. The main reported were rhinitis (54.45% conventional vs. 34.92% organic), chest pains (41.28% conventional vs. 23.81% organic), cough (33.45% conventional, 24.34% organic), breathlessness (31.67% conventional, 4.23% organic) (p<0.05). 16.18% and 27.50% of conventional male and female cotton farmers, respectively, had a restrictive defect. Among organic cotton farmers, 15.85% and 18.69%, respectively, of males and females had a restrictive defect. Furthermore, a significant increase in the predicted average percentage of FEV1/FVC ratio was observed among organic cotton farmers after salbutamol's use (p = 0.039). The type of cultivated cotton was not associated with ventilatory changes neither in the univariate analysis, nor in the multivariate analysis. Other factors such as farmers' age, BMI and insecticides use frequency per year were also important. Farmers who used insecticides more than 6 times per season had an increased risk of developing an obstructive defect (OR = 1.603; 95%CI: 0.484-5.309) compared to those who used them 6 times or less. CONCLUSION: Chronic respiratory signs and ventilatory impairments were found among conventional and, to our knowledge, for the first time among organic cotton producers. However, these health effects were more prevalent among conventional cotton farmers than organic ones.

Respiratory muscle strength can improve the prognostic assessment in COPD.

Impaired lung function, respiratory muscle weakness and exercise intolerance are present in COPD and contribute to poor prognosis. However, the contribution of the combination of these manifestations to define prognosis in COPD is still unknown. This study aimed to define cut-off points for both inspiratory and expiratory muscle strength (MIP and MEP, respectively) for mortality prediction over 42-months in patients with COPD, and to investigate its combination with other noninvasive established prognostic measures (FEV1, V̇O2peak and 6MWD) to improve risk identification. Patients with COPD performed pulmonary function, respiratory muscle strength, six-minute walk and cardiopulmonary exercise tests, and were followed over 42 months to analyze all-cause mortality. A total of 79 patients were included. The sample was mostly (91.1%) comprised of severe (n = 37) and very severe (n = 34) COPD, and 43 (54%) patients died during the follow-up period. Cut-points of ≤ 55 and ≤ 80 cmH2O for MIP and MEP, respectively, were associated with increased risk of death (log-rank p = 0.0001 for both MIP and MEP) in 42 months. Furthermore, MIP and MEP substantially improved the mortality risk assessment when combined with FEV1 (log-ranks p = 0.006 for MIP and p < 0.001 for MEP), V̇O2peak (log-rank: p < 0.001 for both MIP and MEP) and 6MWD (log-ranks: p = 0.005 for MIP; p = 0.015 for MEP). Thus, patients severely affected by COPD presenting MIP ≤ 55 and/or MEP ≤ 80 cmH2O are at increased risk of mortality. Furthermore, MIP and MEP substantially improve the mortality risk assessment when combined with FEV1, V̇O2peak and 6MWD in patients with COPD.

Regional Pulmonary Ventilation Assessment Method and System Based on Impedance Sensing Information from the Pentapulmonary Lobes.

Regional lung ventilation assessment is a critical tool for the early detection of lung diseases and postoperative evaluation. Biosensor-based impedance measurements, known for their non-invasive nature, among other benefits, have garnered significant attention compared to traditional detection methods that utilize pressure sensors. However, solely utilizing overall thoracic impedance fails to accurately capture changes in regional lung air volume. This study introduces an assessment method for lung ventilation that utilizes impedance data from the five lobes, develops a nonlinear model correlating regional impedance with lung air volume, and formulates an approach to identify regional ventilation obstructions based on impedance variations in affected areas. The electrode configuration for the five lung lobes was established through numerical simulations, revealing a power-function nonlinear relationship between regional impedance and air volume changes. An analysis of 389 pulmonary function tests refined the equations for calculating pulmonary function parameters, taking into account individual differences. Validation tests on 30 cases indicated maximum relative errors of 0.82% for FVC and 0.98% for FEV1, all within the 95% confidence intervals. The index for assessing regional ventilation impairment was corroborated by CT scans in 50 critical care cases, with 10 validation trials showing agreement with CT lesion localization results.

Bronchodilator response assessment through impulse oscillometry system and spirometry in children and adolescents with cystic fibrosis.

OBJECTIVE: To investigate the effect of bronchodilator on the respiratory mechanics and pulmonary function of children and adolescents with cystic fibrosis. METHODS: Cross-sectional study on clinically stable children and adolescents with cystic fibrosis aged from six to 15 years. Participants underwent impulse oscillometry and spirometry evaluations before and 15 minutes after bronchodilator inhalation. The Kolmogorov-Smirnov test was applied to verify the sample distribution, and the Student's t-test and Wilcoxon test were used to compare the data before and after bronchodilator inhalation. RESULTS: The study included 54 individuals with a mean age of 9.7±2.8 years. The analysis showed a statistically significant improvement in impulse oscillometry and spirometry parameters after bronchodilator inhalation. However, according to the American Thoracic Society (ATS) and European Respiratory Society (ERS) recommendations (2020 and 2021), this improvement was not sufficient to classify it as a bronchodilator response. CONCLUSIONS: The use of bronchodilator medication improved respiratory mechanics and pulmonary function parameters of children and adolescents with cystic fibrosis; however, most patients did not show bronchodilator response according to ATS/ERS recommendations.

Office-based respiratory assessment in patients with generalized myasthenia gravis.

Patients with myasthenia gravis (MG) can present with respiratory dysfunction, ranging from exercise intolerance to overt respiratory failure, increased fatigue, or sleep-disordered breathing. To investigate the value of multiple respiratory tests in MG, we performed clinical and respiratory assessments in patients with mild to moderate generalized disease. One-hundred and thirty-six patients completed the myasthenia gravis quality-of-life score(MG-QOL-15), myasthenia gravis impairment index(MGII), Epworth sleepiness scale(ESS), University of California-San Diego Shortness of Breath Questionnaire(UCSD-SOB), Modified Medical Research Council Dyspnea Scales(MRC-DS), supine and upright forced vital capacity(FVC), maximal inspiratory pressures(MIPs) and sniff nasal inspiratory pressures(SNIP). Seventy-three (54 %) had respiratory and/or bulbar symptoms and 45 (33 %) had baseline abnormal FVC, with no significant postural changes (p = 0.89); 55 (40.4 %) had abnormal MIPs and 50 (37 %) had abnormal SNIPs. Overall, there were low scores on respiratory and disability scales. Females had increased odds of presenting with abnormal FVC (OR 2.89, p = 0.01) and MIPs (OR 2.48, p = 0.022). There were significant correlations between MIPs, FVC and SNIPs; between MGII/MG-QOL15 and UCSD-SOB/MRC-DS and between ESS and respiratory scales in the whole group. Our data suggests that office-based respiratory measurements are a useful screening method for stable MG patients, even when presenting with minimal respiratory symptoms and no significant disability.

The longitudinal association between adverse childhood experiences, childhood socioeconomic status, and lung function among middle-aged and older adults.

OBJECTIVES: This study aims to investigate the association between adverse childhood experiences (ACEs), childhood socioeconomic status (SES) with lung function among general Chinese middle-aged and older adults. METHODS: Participants at baseline were 9052 individuals aged 45 years old and above from the China Health and Retirement Longitudinal Study (CHARLS), a population-based cohort of Chinese adults. Analyses were conducted with data from three waves (2011, 2013, and 2015). The ACEs included five threat-related indicators (i.e., physical abuse, household substance abuse, domestic violence, unsafe neighborhood, and bullying) and five deprivation-related adversities (i.e., emotional neglect, household mental illness, incarcerated household member, parental separation or divorce, and parental death). The cumulative score of threat-related and deprivation-related ACEs was used for analysis. Lung function was assessed by peak expiratory flow (PEF). Two-level linear mixed growth models were used to evaluate the longitudinal association between and ACEs, childhood SES, and PEF. RESULTS: Participants with more than three deprivation-related ACEs were significantly associated with lower PEF (b = -11.45 L/min, 95%CI: -18.40, -4.49) after adjusting for multiple confounding factors. Threat-related ACEs were not associated with PFF. Father's illiterate education predicted lower lung function (b = -8.49 L/min, 95%CI: -11.68, -5.31) for all middle-aged and older adults while mother's illiterate education was only significantly associated with PEF among the men (b = -9.21 L/min, 95%CI: -18.20, -0.22), and middle-aged adults (b = -7.96 L/min, 95%CI: -14.35, -1.57). DISCUSSION: ACEs and disadvantaged childhood SES are important predictors of lower lung function during adulthood. Reducing ACEs and improving childhood SES may be beneficial for long-term health development.

Assessment of pulmonary function and respiratory symptoms among INDIAN textile sizing mill workers.

BACKGROUND: Textile-sizing mill workers are exposed to various hazards in the sizing units during their working hours and are at risk of acquiring lung impairments due to the usage of sizing chemicals in the sizing process. OBJECTIVE: The main aim of this study is to assess the influence of cotton dust and sizing agents on lung function and breathing difficulties among Indian textile sizing mill workers. METHODS: This cross-sectional study was carried out at a textile-sizing mill from August 2022 to September 2022. A modified questionnaire based American Thoracic Society's standard was used to assess respiratory symptoms among sizing mill workers and the pulmonary function test was conducted Spirometry. The chi-square test was used to find the difference between respiratory symptoms and the t-test was used to find the difference between spirometric parameters. RESULTS: Textile sizing mill workers showed significant (P < 0.0001) decline in peak expiratory flow rate, forced vital capacity (FVC), ratio of FEV1 and forced vital capacity, and forced expiratory volume in 1 s (FEV1). There was an association between symptoms and duration of exposure to pulmonary abnormality. Sizing mill workers showed a significant decline in lung functions and an increase in pulmonary symptoms. As the service duration of exposure in terms of years increased, respiratory symptoms increased and spirometric abnormality also increased. CONCLUSION: This study confirms that sizing agents such as polyvinyl alcohol (PVA), emulsifier, wax, carboxymethyl cellulose (CMC), and starch used in sizing mills are also responsible for respiratory illness and lung impairment among textile workers.

Novel Surface Topographic Assessment of Lung Volume and Pulmonary Function Tests in Idiopathic Scoliosis: A Preliminary Study.

OBJECTIVE: Severe spinal deformity results in restrictive pulmonary disease from thoracic distortions and lung-volume limitations. Though spirometry and body plethysmography are widely accepted tests for pulmonary function tests (PFTs), they are time-consuming and require patient compliance. This study investigates whether surface topographic [surface topography (ST)] measurements of body volume difference (BVD) and torso volume difference between maximum inhale and exhale correlate to values determined on PFTs. METHODS: This study included patients with idiopathic scoliosis and thoracic/thoracolumbar curves ≥40 degrees. Patients received ST scans, clinical examinations, and EOS biplanar radiographs on the same day. PFTs were performed within 3 months of ST/radiographic analysis. Univariate linear regression analysis was used to examine relationships between BVD, PFT values, and mean curves. RESULTS: Sixteen patients (14.6 ± 2.2 y, 69% females) with idiopathic scoliosis and mean thoracic/thoracolumbar curves of 62 degrees ± 15˚ degrees (45 degrees to 93 degrees) were assessed. BVD displayed statistically high-positive positive correlations with forced vital capacity ( R = 0.863, P < 0.0001), forced expiratory volume in 1 second ( R = 0.870, P < 0.001), vital capacity ( R = 0.802, P < 0.0001), and TLC ( R = 0.831, P < 0.0001. Torso volume difference showed similarly high positive correlations to forced vital capacity, forced expiratory volume in 1 second, vital capacity, and TLC, but not residual volume. No correlations emerged between the mean thoracic/thoracolumbar curve and BVD or PFT values. CONCLUSION: This study strongly endorses further investigation into ST scanning as an alternative to traditional PFTs for assessing pulmonary volumes. The noncontact and noninvasive nature of ST scanning presents a valuable alternative method for analyzing thoracic volume, particularly beneficial for patients unable to cooperate with standard PFTs. LEVEL OF EVIDENCE: Level II-prognostic.

Clinical application of computed tomographic volumetric imaging in postoperative lung function assessment in patients with lung cancer.

BACKGROUND: To evaluate the effectiveness of the computed tomographic (CT) volumetric analysis in postoperative lung function assessment and the predicting value for postoperative complications in patients who had segmentectomy for lung cancer. METHODS: CT scanning and pulmonary function examination were performed for 100 patients with lung cancer. CT volumetric analyses were performed by specific software, for the volume of the inspiratory phase (Vin), the mean inspiratory lung density (MLDin), the volume of expiratory phase (Vex), and the mean lung density at expiratory phase (MLDex). Pulmonary function examination results and CT volumetric analysis results were used to predict postoperative lung function. The concordance and correlations of these values were assessed by Bland-Altman analysis and Pearson correlation analysis, respectively. Multivariate binomial logistic regression analysis was executed to assess the associations of CT data with complication occurrence. RESULTS: Correlations between CT scanning data and pulmonary function examination results were significant in both pre- and post-operation (0.8083 ≤ r ≤ 0.9390). Forced vital capacity (FVC), forced expiratory volume in the first second (FEV1), and the ratio of FVC and FEV1 estimated by CT volumetric analyses showed high concordance with those detected by pulmonary function examination. Preoperative (Vin-Vex) and (MLDex- MLDin) values were identified as predictors for post-surgery complications, with hazard ratios of 5.378 and 6.524, respectively. CONCLUSIONS: CT volumetric imaging analysis has the potential to determine the pre- and post-operative lung function, as well as to predict post-surgery complication occurrence in lung cancer patients with pulmonary lobectomy.

Impulse oscillometry assessment of respiratory function in pediatric patients with a history of COVID-19.

OBJECTIVE: While coronavirus disease 2019 (COVID-19) is generally considered to exhibit a less severe clinical course in children than in adults, studies have demonstrated that respiratory symptoms can endure for more than 3 months following infection in at least one-third of pediatric cases. The present study evaluates the respiratory functions of children aged 3-15 years within 3-6 months of their recovery from COVID-19 using impulse oscillometry (IOS) and compares them with the values of healthy children. METHODS: Included in this prospective cross-sectional study were 63 patients (patient group) aged 3-15 years who contracted COVID-19 between December 2021 and May 2022, as well as 57 healthy children as a control group, matched for age and sex. The demographic, clinical, and laboratory data of the patients were recorded, and respiratory function was assessed based on airway resistance (zR5, zR20, R5-20) and reactance (zX5, zX20, reactance area [AX], resonant frequency [Fres]) using an IOS device. RESULTS: There were no significant differences in the age, weight, height, and body weight z score values of the two groups (p > .05). While the zR5 and R5-20 levels of the patient group were higher (p = .008 and p < .001, respectively) than those of the controls, the zR20, AX, and Fres values did not differ significantly between the groups (p > .05). The parameters indicating the reactance, including zX5 and zX20, were significantly lower in the patient group than in the control group (p = .028 and p < .001, respectively). CONCLUSION: Total and peripheral airway resistances were found to be elevated in children who had recovered from COVID-19 in the preceding 3-6 months.

Comparison of particles in exhaled air and multiple breath washout for assessment of small airway function in children with cystic fibrosis.

BACKGROUND: The introduction of modulator therapy for cystic fibrosis (CF) has led to an increased interest in the detection of small airway disease (SAD) as sensitive marker of treatment response. The particles in exhaled air (PExA) method, which records exhaled particle mass (PEx ng/L) and number (PExNR), detects SAD in adult patients. Our primary aim was to investigate if PExA outcomes in children with CF are different when compared to controls and associated with more severe disease. Secondary aims were to assess feasibility and repeatability of PExA in children with CF and to correlate PExA to multiple breath nitrogen washout (MBNW) as an established marker of SAD. METHODS: Thirteen healthy children (HC), 17 children with CF with normal lung function (CF-N) (FEV1 z-score ≥ -1.64) and six with airway obstruction (CF-AO) (FEV1 z-score < -1.64) between 8 and 18 years performed MBNW followed by PExA and spirometry. Children with CF repeated the measurements after 3 months. RESULTS: PEx ng/L and PExNR/L per liter of exhaled breath were similar between the three groups. The lung clearance index (LCI) was significantly higher in both CF-N and CF-AO compared to HC. All participants, except one, were able to perform PExA. Coefficient of variation for PEx ng/l was (median) 0.38, range 0-1.25 and PExNR/l 0.38, 0-1.09. Correlation between LCI and PEx ng/l was low, rs 0.32 (p = .07). CONCLUSION: PExA is feasible in children. In contrast to LCI, PExA did not differentiate healthy children from children with CF suggesting it to be a less sensitive tool to detect SAD.

Advanced and traditional chest MRI sequence for the clinical assessment of systemic sclerosis related interstitial lung disease, compared to CT: disease extent analysis and correlations with pulmonary function tests.

BACKGROUND: MRI is a radiation-free emerging alternative to CT in systemic sclerosis related interstitial lung disease (SSc-ILD) assessment. We aimed to compare a T2 radial TSE and a PD UTE MRI sequence with CT in SSc-ILD extent evaluation and correlations with pulmonary function tests (PFT). MATERIAL AND METHODS: 29 SSc-ILD patients underwent CT, MRI and PFT. ILD extent was visually assessed. Lin's concordance correlation coefficients (CCC) and Kruskal Wallis test (p-value < 0.05) were computed for inter-method comparison. Patients were divided in limited and extended disease, defining extended ILD with two methods: (A) ILD>30% or 10%20% or 20% with FVC%<70%. MRI Sensitivity, Specificity, Positive Predictive Value (PPV), Negative Predictive Value (NPV) and Accuracy were assessed. Pearson correlation coefficients r (p-value<0.025) were computed between ILD extents and PFT (FVC% and DLCO%). RESULTS: Median ILD extents were 11%, 11%, 10% on CT, radial TSE and UTE, respectively. CCC between CT and MRI was 0.95 for both sequences (Kruskal-Wallis p-value=0.64). Sensitivity, Specificity, PPV, NPV and Accuracy in identifying extended disease were: (A) 87.5 %, 100 %, 100 %, 95.5 and 96.6 % with radial TSE and 87.5 %, 95.2 %, 87.5 %, 95.2 and 93.1 % with UTE; (B) 86.7 %, 86.4 %, 66.7 %, 95.0 % and 86.2 % for both sequences. Pearson r of CT, radial TSE and UTE ILD extents with FVC were -0.66, -0.60 and -0.68 with FVC, -0.59, -0.56 and -0.57 with DLCO, respectively (p<0.002). CONCLUSIONS: MRI sequences may have similar accuracy to CT to determine SSc-ILD extent and severity, with analogous correlations with PFT.