Forced Vital Capacity Values Research Articles

Understanding changes in pulmonary function and functional status in breast cancer patients after systemic chemotherapy and radiotherapy: a prospective study

BackgroundRespiratory complications in breast cancer (BC) patients after chemotherapy (CT) and radiotherapy (RT) have been well acquainted and these complications should be investigated to prevent secondary problems and/or improve BC patients’ clinical outcomes. Therefore, this study aimed to assess the potential acute effect of systemic chemotherapy and radiotherapy on respiratory function and functional status of patients with breast cancer.MethodsA total of 25 BC patients who were candidates for systemic chemotherapy and radiotherapy were recruited after oncological examination and included in this study. Respiratory function and functional status were assessed with the Pulmonary Function Test (PFT) and the Six-Minute Walk Test (6MWT), respectively. Patients were assessed before CT (c0), after CT (c1), and after RT (r1).Results25 BC patients were assessed in c0 and c1 while only 15 out of 25 patients (60%) were assessed in r1. The actual values of Forced vital capacity (FVC) (t = 2.338, p =.028), Forced expiratory volume in 1s (FEV1 (t = 2.708, p =.012), and the forced expiratory flow of between 25% and 75% of vital capacity (FEF25-75%) (t = 2.200, p =.038) were found significantly different after systemic CT. Inspiratory (MIP) and expiratory (MEP) muscle strength also did not show a significant change from c0 to c1. A significant effect of the type of surgery was found (Wilks’ lambda, F [1, 19] = 6.561, p =.019, ηp2 = 0.25) between c0 and c1 in actual FVC value. The main effect of time was found significant in FVC (F [2, 28] = 4.840, p =.016, ηp2 = 0.25) from c0 to r1. Pairwise comparisons with Bonferroni correction showed that there was a significant difference between c0 and r1 (p =.037).DiscussionThe present study showed decreased FVC and FEV1 actual values and percent predicted rates from baseline to the completion of treatment. Since the interactional effect of the type of surgery was significant, we suggest that clinical and demographic factors such as age should be considered when interpreting the early changes in PFT. In addition, the significant linear trend of decreasing in some specific outcomes in respiratory function also highlighted the need for continuous monitoring of potential respiratory problems in patients with BC from baseline to the completion of chemotherapy and radiotherapy.

Role of serum B-cell-activating factor and interleukin-17 as biomarkers in the classification of interstitial pneumonia with autoimmune features.

Interstitial pneumonia with autoimmune features (IPAF) is a type of interstitial lung disease (ILD) with immune features that do not meet the diagnostic criteria for specific connective tissue diseases (CTDs). This retrospective case-control study investigated the role of serum B-cell-activating factor of the tumor necrosis factor family (BAFF) and interleukin (IL)-17 as biomarkers for IPAF. The differences in serum BAFF, IL-17, and IL-10 were compared among patients with idiopathic pulmonary fibrosis (IPF), IPAF, ILD associated with CTD (CTD-ILD), and healthy controls. The patients were treatment naïve. The correlations of BAFF with IL-10, IL-17, and pulmonary function were analyzed. The classifiable value of BAFF for IPAF was examined. The results showed that the serum levels of BAFF and IL-17 in the IPAF and CTD-ILD groups were higher than in the IPF group. High BAFF levels and high predicted diffusion capacity of the lungs for carbon monoxide (DLCO) were independent predictive factors for IPAF vs IPF. In the IPAF and CTD-ILD groups, serum BAFF levels were negatively correlated with predicted values of forced vital capacity (FVC%) and diffusing capacity of the lungs for carbon monoxide (DLCO%) and positively correlated with serum IL-17 and IL-10 levels. The cutoff value of combined BAFF and IL-17 was 0.704, and the sensitivity and specificity for classifying IPAF were 78.9 and 95.7%, respectively. In conclusion, combining serum BAFF and IL-17 as a biomarker may have classifiable value in differentiating IPAF from other forms of ILD.

The prevalence of obstructive sleep apnea in Japanese asthma patients.

Obstructive sleep apnea (OSA) occurs more commonly in asthma patients than in the general population because these conditions share some comorbidities. In Japan, the prevalence of OSA in the general population is reported to be approximately 20%; however, few reports have described the prevalence of OSA in asthma patients. Furthermore, the characteristics of Japanese patients with OSA and asthma are not clear. Adult asthma patients were recruited from the outpatient departments of our institution between August 31, 2017, and March 31, 2019. In all included patients, the presence and severity of OSA were evaluated by the Epworth Sleepiness Scale (ESS) and a home sleep test (HST) using portable polysomnography (PSG). The rate of coexisting OSA in asthma patients and the characteristics of those patients according to the severity of OSA were investigated. Fifty-three patients were included. OSA was detected in 36 (67.9%) patients (mild, n = 15; moderate, n = 14; and severe, n = 7). Patients with OSA had significantly higher body mass index, Brinkman index, apnea-hypopnea index (AHI), and 3% oxygen desaturation index (ODI) values in comparison to those without OSA, while the percentage of the predicted value of forced vital capacity (%FVC) and lowest SpO2 levels were significantly lower. As the severity of OSA increased, age, brain natriuretic peptide level, AHI, and 3%ODI increased, and in contrast, FVC, %FVC, forced expiratory volume in one second (FEV1), percentage of the predicted value of FEV1 (%FEV1), Epworth Sleepiness Scale (ESS), 3%ODI, and lowest SpO2 levels decreased. In particular, the fact that the ESS value was inversely correlated with the severity of OSA in our patients was different from the general characteristics of OSA. Moreover, the AHI value was negatively correlated with FVC, %FVC, FEV1, and %FEV1. BMI was the only independent factor for the presence of OSA, and for asthma severity (FEV1, % of predicted), there was a weak correlation with smoking history. This is the first report to investigate the prevalence of OSA in Japanese asthma patients, using an HST. This study suggests that an HST should be performed in addition to the sleep interview for asthma patients with refractory disease, a low pulmonary function, advanced age, and high BMI because the more severe the OSA, the lower the ESS value may be.

Bosentan and ambrisentan in the treatment of idiopathic pulmonary fibrosis: a meta-analysis.

The aim is to showcase the effectiveness and safety of bosentan or ambrisentan in individuals diagnosed with idiopathic pulmonary fibrosis (IPF) and offer fresh evidence for the management of this condition. For this research, we conducted a meta-analysis of randomized controlled trials by searching various databases, including the Cochrane Library, Excerpta Medica Database, PubMed, and Web of Science. The retrieval was conducted until November 2021. We analyzed the variances in 6-minute walk distance (6MWD), death, diffusion capacity for carbon monoxide (DLCO), forced vital capacity (FVC), hospitalization, IPF worsening, mean pulmonary arterial pressure, serious adverse events (SAEs), Short Form-36 improved, and St. George's Respiratory Questionnaire between the treatment and control groups. A sum of six studies involving 1,928 participants were found to meet the inclusion criteria. The quality of evidence was high. The control group had significantly higher values for 6MWD, DLCO, and FVC compared to the ambrisentan treatment group. The rates of hospitalization and IPF worsening were considerably greater in comparison with the control group. The bosentan group exhibited significantly reduced rates of hospitalization and IPF worsening in comparison with the control group. Both drugs did not cause any raising in death or SAEs when in comparison with the control group. The findings of this research validate the effectiveness and safety of bosentan for treating IPF patients. This medication can enhance the quality of life for individuals with IPF without causing any significant increase in SAEs. However, it does not have a notable influence on the long-term prognosis. The findings of this research do not endorse the utilization of ambrisentan in individuals diagnosed with IPF.

The Performance of Pulmonary Function Tests in Predicting Systemic Sclerosis—Interstitial Lung Disease in the European Scleroderma Trial and Research Database

Background and Objectives: In SSc, ILD is a major cause of morbidity and mortality. We aimed to investigate the performance of DLCO (diffusing capacity of lung carbon monoxide) and FVC (forced vital capacity) delta change (Δ) and baseline values in predicting the development of SSc-ILD. Methods: Longitudinal data of DLCO, FVC, and ILD on the HRCT of SSc patients from the EUSTAR database were evaluated at baseline (t0) and after 12 (±4) (t1) and 24 (±4) (t2) months. Results: 474/17805 patients were eligible for the study (403 females); 46 (9.7%) developed ILD at t2. Positivity for anti-topoisomerase antibodies (117 patients) showed an association with ILD development at t2 (p = 0.0031). Neither the mean t0 to t1 change (Δ) of DLCO nor the mean t0 to t1 FVCΔ predicted the appearance of ILD at t2. Investigating the possible role of baseline DLCO and FVC values in predicting ILD appearance after 24 (±4) months, we observed a moderate predictive capability of t0 DLCO < 80%, stronger than that of FVC < 80%. Conclusions: We suggest that an impaired baseline DLCO may be predictive of the appearance of ILD after 2 years of follow-up. This result advances the hypothesis that a reduction in gas exchange may be considered an early sign of lung involvement. However, further rigorous studies are warranted to understand the predictive role of DLCO evaluation in the course of SSc.

Patients With Post-COVID-19 Respiratory Condition: Chest Computed Tomography Findings and Pulmonary Function Tests and Comparison With Asymptomatic Participants.

The aims of this study were to assess the chest computed tomography (CT) findings in post-COVID-19 respiratory condition (rPCC) patients and compare the findings with asymptomatic participants (APs). It also aimed to evaluate the relationship between CT findings and pulmonary function tests (PFTs) in rPCC patients. Finally, it aimed to compare the quantitative chest CT findings and PFT results of patients with rPCC and APs. We retrospectively enrolled consecutive patients with rPCC who underwent unenhanced chest CT and PFTs between June 2020 and September 2022. In addition, a control group (APs) was prospectively formed and underwent nonenhanced chest CT and PFTs. The presence and extent of abnormalities in unenhanced chest CT images were evaluated qualitatively and semiquantitatively in a blinded manner. We used fully automatic software for automatic lung and airway segmentation and quantitative analyses. Sixty-three patients with rPCC and 23 APs were investigated. Reticulation/interstitial thickening and extent of parenchymal abnormalities on CT were significantly greater in the rPCC group than in the control group ( P = 0.001 and P = 0.004, respectively). Computed tomography extent score was significantly related to length of hospital stay, age, and intensive care unit stay (all P s ≤ 0.006). The rPCC group also had a lower 85th percentile attenuation lung volume ( P = 0.037). The extent of parenchymal abnormalities was significantly correlated with carbon monoxide diffusing capacity ( r = -0.406, P = 0.001), forced vital capacity (FVC) ( r = -0.342, P = 0.002), and forced expiratory volume in 1 second/FVC ( r = 0.427, P < 0.001) values. Pulmonary function tests revealed significantly lower carbon monoxide diffusing capacity ( P < 0.001), FVC ( P = 0.036), and total lung capacity ( P < 0.001) values in the rPCC group. The rPCC is characterized by impaired PFTs, a greater extent of lung abnormalities on CT, and decreased 85th percentile attenuation lung volume. Advanced age, intensive care unit admission history, and extended hospital stay are risk factors for chest CT abnormalities.

The Association Between Dietary Magnesium Intake and Pulmonary Function: Recent Fndings from NHANES 2007-2012.

This article aims to study the correlation between dietary magnesium intake and pulmonary function, utilizing data from the National Health and Nutrition Examination Survey (NHANES) database. This cross-sectional study examined representative samples of adults from the USA (n = 818; NHANES 2007-2012) to explore the correlation between magnesium intake and pulmonary function. We obtained the average magnesium intake over 2days, as well as measured pulmonary function parameters, including forced expiratory volume in the first second (FEV1), forced vital capacity (FVC), FEV1/FVC, peakexpiratoryflow rate (PEF), and forcedexpiratoryflow between25and75%ofFVC (FEF25-75%). Weighted multivariable linear regression was used to investigate the relationship between magnesium intake and pulmonary function. Additionally, subgroup analyses, interaction tests, and sensitivity analyses were conducted. Weighted multiple linear regression models revealed a significant positive correlation between magnesium and pulmonary function, even after adjusting for all included confounding variables. When we categorized magnesium intake into tertiles, we found that participants in the highest tertile of magnesium intake had significantly higher values for FVC (β: 898.54, 95%CI: 211.82-1585.25), FEV1 (β: 858.16, 95%CI: 212.41-1503.91), FEV1/FVC (β: 0.024, 95%CI: 0.004-0.044), PEF (β: 1324.52, 95%CI: 481.71-2167.33), and FEF25-75% (β: 831.39, 95%CI: 84.93-1577.84). Upon stratifying the data by age and sex, it was observed that this positive correlation was particularly pronounced among men aged 40-79. At the same time, the stability of the results was further confirmed by sensitivity analyses. This study suggested that dietary magnesium intake may improve pulmonary function.

A Preliminary Study on the Impact of Household Air Pollution on Adult Respiratory Health in Urban and Rural Settings of Jaipur, India.

This study aimed to investigate the association between household air pollution and chronic respiratory illness (CRI) in Jaipur, India. A total of 147 participants over 18 years of age from 45 households were randomly included in the study, and follow-up visits were conducted once every fortnight over a three-month period. Spirometry tests were conducted using a portable hand-held digital spirometer. A self-designed, pretested, semi-structured questionnaire was utilized to collect socio-demographic information from the participants. Statistical tests, such as t-tests were used to analyze the association between household air pollution and CRI. The prevalence of COPD and bronchial asthma was 2% and 4%, respectively. Various risk factors for CRI were identified within households, and only 4 episodes of acute exacerbation of chronic respiratory illness were observed, resulting in no incidence density calculation. The mean values of forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1) were significantly lower in females (P < 0.001) Also, the mean values of FVC and FEV1 were significantly lower in rural areas (P < 0.005) than urban areas. Our findings highlight the importance of government and stakeholder action to implement clean air policies and protect respiratory health. Further studies are needed to expand on these findings and delve deeper into understanding the complex interactions between various risk factors and respiratory health in this population.

Effects of Thoracoscopic Right Upper Lobe Apical Segmentectomy on Exercise Capacity and Quality of Life in Early-Stage NSCLC Patients.

Thoracoscopic lobectomy (TL) is an effective surgical approach for resecting tumor lesions in patients with early non-small cell lung cancer (NSCLC). However, TL may result in damage to normal lung tissue, potentially impacting prognosis. Thoracoscopic right upper lobe apical segmentectomy (TS) has been proposed as an alternative to improve surgical outcomes, but its impact on exercise capacity and quality of life remains unclear. This study aimed to investigate the effect of TS on exercise capacity and quality of life in patients with early-stage NSCLC. A retrospective analysis was conducted on the clinical data of 120 patients with early-stage NSCLC who underwent surgical treatment in Shangyu People's Hospital of Shaoxing between August 2020 and August 2023. The patients were divided into two groups based on the surgical approach: the TL group (n = 66) and the TS group (n = 54). The primary objective was to compare surgery-related indicators and the overall incidence of complications between the TS group and the TL group. Additionally, changes in forced vital capacity (FVC), forced expiratory volume in one second (FEV1), maximum ventilatory volume (MVV), 6-minute walking distance (6MWD), and European Organization for Research and Treatment of Cancer quality of life (EORTC QLQ-C30) scores were evaluated before and after operation. The TS group showed significantly reduced intraoperative blood loss, chest drainage, and hospital stay compared to the TL group (p < 0.05). However, there was no significant difference in the operation time and the number of lymph node dissections between the two groups after operation (p > 0.05). FVC, FEV1, MVV, and 6MWD values of the two groups were significantly lower than those before operation (p < 0.05). However, FVC, FEV1, MVV, and 6MWD in the TS group were significantly higher than those in the TL group (p < 0.05). The scores of roles, emotion, cognition, social function, and total health status in the two groups after operation were significantly higher than those before operation, and the scores of physical functions, shortness of breath, diarrhea, fatigue, pain, cough and insomnia were significantly lower than those before operation (p < 0.05). Compared to the TL group, the TS group showed higher scores of physical, social function dimensions, and total health status, as well as lower scores of fatigues, shortness of breath, insomnia, and pain (p < 0.05). TS treatment has less surgical trauma and a lower risk of complications for patients with early-stage NSCLC, which is beneficial for promoting postoperative recovery, reducing lung function damage and improving the quality of life of patients.

Diaphragmatic ultrasonography as a predictor of respiratory muscle fatigue in myasthenia gravis.

Easy fatigability, the clinical hallmark of generalized myasthenia gravis (GMG), cannot be detected in a dynamic way. The aim of this study was to assess respiratory function dynamically through diaphragmatic ultrasonography (DUS) in GMG patients. GMG patients and controls were recruited in a 1:1 ratio. DUS was performed during one quiet breath and 15 consecutive deep breaths. The diaphragm thicknesses were measured at different positions. Diaphragm thickening fraction (TFdi) and the maximal change in diaphragm thickness (Tmax) during 15 consecutive deep breaths were calculated and transformed to normality, named N-TFdi and N-Tmax, respectively. The percentages of changes in TFdi and Tmax compared with baseline were named ΔTFdi and ΔTmax, respectively. The diagnostic parameter for respiratory muscle fatigue was chosen from ΔTFdi and ΔTmax at different deep breath times according to their ability to distinguish GMG patients from controls and the interrater reliability of TFdi and Tmax. Thirty-four GMG patients and 30 healthy controls were enrolled. N-TFdi and N-Tmax significantly changed as the number of deep breaths increased (p < .001) in GMG patients, but not in controls. ΔTmax of the 15th deep breath (ΔTmax15) was selected as the diagnostic parameter for respiratory muscle fatigue. There were no significant differences in percentage of predicted values of forced vital capacity and arterial partial pressure of carbon dioxide between patients with normal and abnormal ΔTmax15. DUS could identify diaphragm fatiguability in GMG patients, which may be more reliable and sensitive in assessment of diaphragm fatigue than conventional methods.

Extracting forced vital capacity from the electronic health record through natural language processing in rheumatoid arthritis-associated interstitial lung disease.

To develop a natural language processing (NLP) tool to extract forced vital capacity (FVC) values from electronic health record (EHR) notes in patients with rheumatoid arthritis-interstitial lung disease (RA-ILD). We selected RA-ILD patients (n = 7485) in the Veterans Health Administration (VA) between 2000 and 2020 using validated ICD-9/10 codes. We identified numeric values in proximity to FVC string patterns from clinical notes in the EHR. Subsequently, we performed processing steps to account for variability in note structure, related pulmonary function test (PFT) output, and values copied across notes, then assigned dates from linked administrative procedure records. NLP-derived FVC values were compared to values recorded directly from PFT equipment available on a subset of patients. We identified 5911 FVC values (n = 1844 patients) from PFT equipment and 15 383 values (n = 4982 patients) by NLP. Among 2610 date-matched FVC values from NLP and PFT equipment, 95.8% of values were within 5% predicted. The mean (SD) difference was 0.09% (5.9), and values strongly correlated (r = 0.94, p < 0.001), with a precision of 0.87 (95% CI 0.86, 0.88). NLP captured more patients with longitudinal FVC values (n = 3069 vs. n = 1164). Mean (SD) change in FVC %-predicted per year was similar between sources (-1.5 [30.0] NLP vs. -0.9 [16.6] PFT equipment; standardized response mean = 0.05 for both). NLP of EHR notes increases the capture of accurate, longitudinal FVC values by three-fold over PFT equipment. Use of this NLP tool can facilitate pharmacoepidemiologic research in RA-ILD and other lung diseases by capturing this critical measure of disease severity.

Sex-Linked Differences in Pulmonary Functions of COVID-19 Patients After a Six-Minute Walk Test.

Coronavirus disease 2019 (COVID-19) predominantly impacts the respiratory system. Historically, numerous lung diseases have shown sex-related differences throughout their progression. This study aimed to identify sex-linked disparities in pulmonary function tests (PFTs) among individuals who have recovered from COVID-19 when subjected to a six-minute walk test (6MWT). In this observational cross-sectional study, we analyzed 61 participants, consisting of 39 (64%)males and 22 (36%) females, all of whom previously contracted COVID-19 three months or more prior. We measured vitals such as blood pressure, pulse, oxygen saturation, and PFT values before and after the 6MWT. The post-6MWT evaluation revealed notable mean differences between males and females in parameters systolic blood pressure (SBP) (p = 0.003), diastolic blood pressure (DBP) (p = 0.026), forced expiratory volume in the first second (FEV1) (p = 0.038), forced vital capacity (FVC) (p = 0.041), and maximum voluntary ventilation (MVV) index (p = 0.011). PFT outcomes indicated sex-based variations among post-COVID-19 subjects. Specifically, post-stress values for FEV1, FVC, MVV index, SBP, and DBP were more elevated in males than in females. However, females presented with higher oxygen saturation levels post-COVID-19 compared to males. Using multiple linear regression modeling, sex was not found to be a strong predictor of PFT results. However, individual regression analyses for FEV1, FVC, and MVV index consistently showcased higher values in males. In conclusion, significant PFT differences exist between males and females after recovery from COVID-19 when exposed to stress induction via the 6MWT.

Mycophenolate mofetil versus cyclophosphamide plus in patients with connective tissue disease-associated interstitial lung disease: Efficacy and safety analysis.

The decision for definitive therapy for the treatment of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) is difficult. Patients with CTD-ILD received 0.5 g twice a day of mycophenolate mofetil for 2 years (MMF cohort, n = 105) or cyclophosphamide 50 mg once every other day, and the cumulative dose of cyclophosphamide should not exceed 10 g (CYC cohort, n = 140). After complete of treatment (EL), % forced vital capacity (FVC) and % diffusing capacity of the lungs for carbon monoxide were increased in both the MMF and CYC cohorts as compared to before treatment (p < 0.001 for all). There were higher changes in % FVC values and a greater number of patients with significant change in % FVC (>10% change) in the CYC cohort than in the MMF cohort (p < 0.0001 for both) at EL. Patients in the CYC cohort had higher rates of leukopenia, thrombocytopenia, serious adverse effects related to treatment(s), and death than those in the MMF cohort (p < 0.05 for all). Cyclophosphamide plus prednisolone superiorly improved % FVC compared to mycophenolate mofetil plus prednisolone. Mycophenolate mofetil and cyclophosphamide improved pulmonary function. Mycophenolate mofetil is less toxic and increased patient survival.

Artificial Intelligence-based Quantification of Pleural Plaque Volume and Association With Lung Function in Asbestos-exposed Patients.

Pleural plaques (PPs) are morphologic manifestations of long-term asbestos exposure. The relationship between PP and lung function is not well understood, whereas the time-consuming nature of PP delineation to obtain volume impedes research. To automate the laborious task of delineation, we aimed to develop automatic artificial intelligence (AI)-driven segmentation of PP. Moreover, we aimed to explore the relationship between pleural plaque volume (PPV) and pulmonary function tests. Radiologists manually delineated PPs retrospectively in computed tomography (CT) images of patients with occupational exposure to asbestos (May 2014 to November 2019). We trained an AI model with a no-new-UNet architecture. The Dice Similarity Coefficient quantified the overlap between AI and radiologists. The Spearman correlation coefficient ( r ) was used for the correlation between PPV and pulmonary function test metrics. When recorded, these were vital capacity (VC), forced vital capacity (FVC), and diffusing capacity for carbon monoxide (DLCO). We trained the AI system on 422 CT scans in 5 folds, each time with a different fold (n = 84 to 85) as a test set. On these independent test sets combined, the correlation between the predicted volumes and the ground truth was r = 0.90, and the median overlap was 0.71 Dice Similarity Coefficient. We found weak to moderate correlations with PPV for VC (n = 80, r = -0.40) and FVC (n = 82, r = -0.38), but no correlation for DLCO (n = 84, r = -0.09). When the cohort was split on the median PPV, we observed statistically significantly lower VC ( P = 0.001) and FVC ( P = 0.04) values for the higher PPV patients, but not for DLCO ( P = 0.19). We successfully developed an AI algorithm to automatically segment PP in CT images to enable fast volume extraction. Moreover, we have observed that PPV is associated with loss in VC and FVC.

Real world experience on the effectiveness and safety of pirfenidone in patients with idiopathic pulmonary fibrosis in Taiwan.

Randomized controlled trials have demonstrated a reduction in the decline of lung function and a reduced risk of acute exacerbation in patients with idiopathic pulmonary fibrosis treated with the antifibrotic prifenidone. The present study aimed to investigate the real-world effectiveness and safety profile of pirfenidone treatment for patients with IPF in Taiwan. Between January 1, 2019 and December 31, 2020, we enrolled 50 patients who were newly diagnosed with IPF and had at least 12 months follow-up period after pirfenidone administration. The primary outcome of pharmacologic effect showed that the mean differences in the absolute values of forced vital capacity from baseline were 0.2 liter (n = 36), 0.13 liter (n = 32), 0.04 liter (n = 26), and - 0.004 liter (n = 26) after 3, 6, 9, and 12 months of administration, respectively. A slight improvement in quality of life, including scores of chronic obstructive pulmonary disease assessment test and St. George's respiratory questionnaire scores. The most common adverse effects were gastrointestinal upset and dermatological problems. No new safety concerns were observed in the present study. Our real-world study describe for the first time in Taiwan, the use of pirfenidone over a 12 months period. This drug preserves the lung function and improves quality of life with tolerable side effects.

Respiratory function in a large cohort of treatment-naïve adult spinal muscular atrophy patients: a cross-sectional study

Due to poor data in literature, we aimed to investigate the respiratory function in a large cohort of naïve Italian adult (≥18 years) SMA patients in a multi-centric cross-sectional study. The following respiratory parameters were considered: forced vital capacity (FVC), forced expiratory volume in one second (FEV1) and need for non-invasive ventilation (NIV). We included 145 treatment-naïve adult patients (SMA2=18, SMA3=125; SMA4=2), 58 females (40 %), with median age at evaluation of 37 years (range 18–72). Fifty-six (37 %) and 41 (31 %) patients had abnormal (<80 %) values of FVC and FEV1, respectively. Fourteen (14 %) patients needed NIV, started at median age of 21 (range 4–68). Motor function, measured by Hammersmith Functional Motor Scale Expanded and Revised Upper Limb Module as well as SMA2, loss of walking ability, surgery for scoliosis, use of NIV, and cough assisting device (CAD) were all significantly associated to lower FVC and FEV1 values, while no association with age at baseline, disease duration, gender or 6 min walking test was observed, except for a correlation between FVC and age in SMA3 walkers (p < 0.05). In conclusion, respiratory function in adult SMA patients is relatively frequently impaired, substantially stable, and significantly correlated with motor function and disease severity.

Joint modeling of forced vital capacity measures with time to onset of polycythemia among chronic obstructive pulmonary outpatients follows-up: A case of University of Gondar Referral Hospital.

Chronic obstructive pulmonary disease (COPD) causes airflow obstruction and respiratory problems. Thus, the main objective of this study was to determine the risk factors for the progression of COPDusing longitudinally measured forced vital capacity with time to onset of polycythemia outpatients follow-up. A retrospective study design was used to gather the related data on longitudinal change of forced vital capacity and time to onset of polycythemia from the medical charts. The joint model consists of a longitudinal submodel for the change of forced vital capacity and a survival submodel for the time to onset of polycythemia of chronic obstructive pulmonary patients. From the total of 266 patient's estimated value of forced vital capacity of chronic obstructive pulmonary patients was 74.45 years with a standard deviation of 8.59. The estimated value of the association parameter was -0.006, which indicates that the lower value for a forced vital capacity measure was associated with the higher risk of polycythemia and vice versa "Based on the joint model analysis found that the predictor smoking, comorbidities, marital status, weight, and HIV" jointly affected the two responses, which are change of forced vital capacity and time to onset of polycythemia among chronic obstructive pulmonary patients. The overall performance of separate and joint models, joint modeling of longitudinal measures with the time-to-event outcome was the best model due to smaller standard errors and statistical significance of both the association parameters.

Impact of chronic fibrosing interstitial lung disease on healthcare use: association between fvc decline and inpatient hospitalization

BackgroundMany types of interstitial lung diseases (ILDs) may transition to progressive chronic-fibrosing ILDs with rapid lung function decline and a negative survival prognosis. In real-world clinical settings, forced vital capacity (FVC) measures demonstrating progressive decline may be linked to negative outcomes, including increased risks of costly healthcare resource utilization (HRU). Thus, we assessed the relationship between rate of decline in lung function and an increase in HRU, specifically inpatient hospitalization, among patients with chronic fibrosing ILD.MethodsThis study utilized electronic health records from 01-Oct-2015 to 31-Oct-2019. Eligible patients (≥ 18 years old) had ≥ 2 fibrosing ILD diagnosis codes, clinical activity for ≥ 15 months, and ≥ 2 FVC tests occurring 6 months apart. Patients with missing demographic data, IPF, or use of nintedanib or pirfenidone were excluded. Two groups were defined by relative change in percent of predicted FVC (FVC% pred) from baseline to 6 months: significant decline (≥ 10%) vs. marginal decline/stable FVC (decrease < 10% or increase). The primary outcome was defined as the occurrence of an inpatient hospitalization 6 months after the first FVC value. Descriptive and multivariable analysis was conducted to examine the impact of FVC decline on occurrence of inpatient hospitalization.ResultsThe sample included 566 patients: 13% (n = 75) with significant decline and 87% (n = 491) with marginal decline/stable FVC; their mean age (SD) was 65 (13.7) years and 56% were female. Autoimmune diagnoses were observed among 40% of patients with significant decline, and 27% with marginal decline/stable FVC. The significant decline group had better lung function at baseline than the marginal/stable group. For patients with FVC% <80% at baseline, reduction of FVC% ≥10% was associated with significantly increased odds of an inpatient hospitalization (odds ratio [OR] 2.85; confidence interval [CI] 1.17, 6.94 [p = 0.021]).ConclusionDecline in FVC% ≥10% was associated with increased odds of inpatient hospitalization among patients with reduced lung function at baseline. These findings support the importance of preserving lung function among patients with fibrosing ILD.

Outcomes in progressive systemic sclerosis treated with autologous hematopoietic stem cell transplantation compared with combination therapy.

Autologous hematopoietic stem cell transplantation (AHSCT) has been shown to improve long-term survival for early diffuse progressive SSc compared with CYC. CYC, however, does not provide a long-term benefit in SSc. The combination of MMF and rituximab is a potent alternative regimen. We aimed to retrospectively compare the outcomes of SSc patients who underwent AHSCT to patients who met the eligibility criteria for AHSCT but received upfront combination therapy with MMF and rituximab. Repeated assessments of modified Rodnan Skin Score (mRSS), forced vital capacity (FVC), and diffusing capacity (DLCO) values were conducted. Clinical improvement was defined as an mRSS decrease >25% or an FVC increase >10%. Event-free survival (EFS) was defined in the absence of persistent major organ failure or death. Twenty-one SSc patients in the combination therapy group were compared with 16 in the AHSCT group. Age, sex and disease duration were similar between the two groups. Clinical improvement at 12 months was seen in 18 (86%) patients in the combination group compared with 13 (81%) in the AHSCT group (P = 0.7). The hazard ratio for EFS at 24 months favoured the combination group (HR = 0.09, P = 0.04). During follow-up, both groups exhibited a significant and comparable reduction in mRSS and an increase in FVC values at each time interval up to 24 months. MMF and rituximab compared with AHSCT in SSc patients eligible for AHSCT resulted in similar skin and lung clinical improvement with a better safety profile at 24 months.

RESPIRATORY FUNCTION IN CHILDREN WITH CEREBRAL PALSY

Purpose: to characterize the pulmonary function of children with cerebral palsy (CP) and to compare it with children with typical development (TD). Methods: the study was with 20 children TD and 21 CP, both sexes aged between 6-12 years (9.41 ± 1.47), with cognitive ability to perform the spirometry test. Wilcoxon / Mann Whitney test hypothesis compare the values obtained in both groups and intragroup in relation to gender and in the functional classifications of CP. Results: TD group showed a significant difference and higher spirometry values in males, whereas for children with CP there was no difference in relation to sex. In addition, the TD group demonstrated better values of forced vital capacity (FVC), forced expiratory volume in the first second (FEV1), and peak expiratory flow (PEF) than the CP group. There were significant differences and better spirometric values for children with CP less compromised in the comparisons between the functional, topographic levels, and functional gait pattern. Conclusion: there is a tendency towards obstructive and restrictive breathing patterns in the children with CP, observable mainly by the decrease in FVC, FEV1, and PEF compared DT. CP children with worse functionality have greater respiratory impairments.