Pulmonary Function Test Results Research Articles

Radiological appearance and lung function six months after invasive ventilation in ICU for COVID-19 pneumonia: An observational follow-up study.

Respiratory functional sequelae in COVID-19 patients admitted to the intensive care unit for invasive ventilation are sparsely reported. The aim of this study was to investigate the radiological lung appearance, lung function and their association at 6 months after hospital discharge. It was hypothesized that the degree of pathological morphology on CT scans would correlate with lung function at the time of follow-up. In this single-centre prospective observational study, 86 from 154 patients admitted to ICU due to COVID-19 between March 2020 and May 2021 were followed up at 6 months post discharge with computed tomography (CT) of the chest and pulmonary function tests (PFTs). The PFT results were expressed as z-scores calculated as the difference between the measured and predicted values divided by the standard deviation obtained from a reference population. Correlations were evaluated by Spearman's rho including the 95% confidence interval. Pathological changes on CT were found in 78/85 participants with fibrous parenchymal bands being the most prevalent finding (91%) followed by traction bronchiectasis (64%) and ground glass opacities (41%). Sixty-five participants performed PFTs, and a restrictive pattern was the most prevalent abnormality (34%). Diffusing capacity of the lung for carbon monoxide (DLCO) was reduced in 66% of participants. The CT severity score weakly correlated with forced vital capacity (FVC) z-score (0.295, p = 0.006), DLCO z-score (-0.231, p = 0.032) and alveolar volume (VA) z-score (0.253, p = 0.019). Most patients showed persistent radiological abnormalities on CT and reduced lung volumes, impaired diffusion capacity and patterns of restrictive lung function at 6 months post discharge from the ICU. The correlations between abnormalities on CT and lung function tests were weak. Further, studies with a long-term follow-up of lung function in this group of patients are needed.

The Subglottic Stenosis 6 Questionnaire: A Novel Quality-of-Life Survey Validated to Predict Need for Intervention in Patients With Idiopathic Subglottic Stenosis

Idiopathic subglottic stenosis (iSGS) is a recurrent, chronic disease defined by fibroinflammatory narrowing of the subglottic airway. A key challenge in treatment is monitoring disease progression, which may be debilitating and unpredictable in its timing. Can the Subglottic Stenosis 6 (SGS-6) questionnaire be validated as a novel quality-of-life (QOL) instrument to monitor breathing, disease progression, and disease severity proactively in patients with iSGS? Panel data from 51 patients with iSGS were collected from January 2012 through June 2022, representing 1,684 patient encounters including routine office visits and treatment encounters. Subjective QOL scores (including the novel SGS-6 and established RAND-36 and EuroQol Five Dimensions [EQ-5D] Visual Analog Scale) and objective pulmonary function test (PFT) results were collected at each visit. Subjective SGS-6 QOL scores were repeated within 1week of initial reporting. Panel regression analyses were performed to assess the relationship between SGS-6 scores, PFT results, and a patient's need for intervention. Minimal clinically important differences (MCIDs) for SGS-6 and peak expiratory flow percentage (PEF%) were assessed using receiver operating characteristic (ROC) curve analysis and a patient's need for intervention as the external anchor. Each one-point increase in SGS-6 score (of a maximum of 27) was associated with a 3.26%decrease in PEF%, a 1.93-point decrease in RAND-36 Physical Health composite score, a 1.27-point decrease in RAND-36 Mental Health composite score, and a 0.88-point decrease in EQ-5D Visual Analog Scale score. The intracorrelation coefficient for the SGS-6 composite score is 0.838 (95%CI, 0.770-0.888). Compared with patient baselines, SGS-6 scores were 4.66 points greater at the time of intervention with an MCID of 2.25 from a patient's baseline. The area under the ROC curve for SGS-6 and a patient's intervention point was 0.81. iSGS disease severity can be modeled using the SGS-6 questionnaire, offering physicians and patients a potentially new method of tracking disease progression and need for intervention remotely.

A Real-World Multicenter Retrospective Observational Study on Polish Experience with Nintedanib Therapy in Patients with Idiopathic Pulmonary Fibrosis: The PolExNIB Study.

Nintedanib is a disease-modifying agent licensed for the treatment of IPF. Data on Polish experience with nintedanib in IPF are lacking. The present study aimed to describe the safety and efficacy profiles of nintedanib in a large real-world cohort of Polish patients with IPF. This was a multicenter, retrospective, observational study of IPF patients treated with nintedanib between March 2018 and October 2021. Data collection included baseline clinical characteristics, results of pulmonary function tests (PFTs), and a six-minute walk test (6MWT). Longitudinal data on PFTs, 6MWT, adverse drug reactions (ADRs), and treatment persistence were also retrieved. A total of 501 patients (70% male) with a median age of 70.9 years (IQR 65-75.7) were included in this study. Patients were followed on treatment for a median of 15 months (7-25.5). The majority of patients (66.7%) were treated with the full recommended dose of nintedanib and 33.3% of patients were treated with a reduced dose of a drug. Intermittent dose reductions or drug interruptions were needed in 20% of patients. Over up to 3 years of follow-up, pulmonary function remained largely stable with the minority experiencing disease progression. The most frequent ADRs included diarrhea (45.3%), decreased appetite (29.9%), abdominal discomfort (29.5%), weight loss (32.1%), nausea (20.8%), fatigue (19.2%), increased liver aminotransferases (15.4%), and vomiting (8.2%). A total of 203 patients (40.5%) discontinued nintedanib treatment due to diverse reasons including ADRs (10.2%), death (11.6%), disease progression (4.6%), patient's request (6.6%), and neoplastic disease (2.2%). This real-world study of a large cohort of Polish patients with IPF demonstrates that nintedanib therapy is safe, and is associated with acceptable tolerance and disease stabilization. These data support the findings of previously conducted clinical trials and observational studies on the safety and efficacy profiles of nintedanib in IPF.

Re-hospitalisation predicts poor prognosis after acute exacerbation of interstitial lung disease

BackgroundSeveral markers have been identified to increase the risk for acute exacerbation of interstitial lung disease (AE-ILD) or mortality related to AE-ILD. However, less is known about the risk predictors of ILD patients who have survived AE. The aim of the study was to characterise AE-ILD survivors and investigate prognostic factors in this subpopulation.MethodsAll AE-ILD patients (n = 95) who had been discharged alive from two hospitals located in Northern Finland were selected from a population of 128 AE-ILD patients. Clinical data related to the hospital treatment and six-month follow-up visit were collected retrospectively from medical records.ResultsFifty-three patients with idiopathic pulmonary fibrosis (IPF) and 42 patients with other ILD were identified. Two thirds of the patients had been treated without invasive or non-invasive ventilation support. The clinical features of six-month survivors (n = 65) and non-survivors (n = 30) did not differ in terms of medical treatment or oxygen requirements. Of the patients, 82.5% used corticosteroids at the six-month follow-up visit. Fifty-two patients experienced at least one non-elective respiratory re-hospitalisation before the six-month follow-up visit. In a univariate model, IPF diagnosis, high age and a non-elective respiratory re-hospitalisation increased the risk of death, although re-hospitalisation was the only independent risk factor in a multivariate model. In six-month survivors, there was no statistically significant decrease in pulmonary function test results (PFT) examined at the follow-up visit compared with earlier PFT examined near the time of AE-ILD.ConclusionsThe AE-ILD survivors were a heterogeneous group of patients both clinically and in terms of their outcome. A non-elective respiratory re-hospitalisation was identified as a marker of poor prognosis among AE-ILD survivors.

Is there a relationship between Haller Index and cardiopulmonary function in children with pectus excavatum?

This study aims to systematically examine the cardiopulmonary functions in children with pectus excavatum and to compare the obtained findings with the Haller Index. Between September 2017 and June 2018, medical records of a total of 31 patients (27 males, 4 females; mean age: 14.8±2.0 years; range, 9 to 18 years) with pectus excavatum were retrospectively analyzed. The patients were divided into Group 1 (<2.5), Group 2 (2.5 to 3.19), and Group 3 (>3.2) according to the Haller Index. All groups were systematically evaluated based on pulmonary function tests and echocardiography. Forced vital capacity, forced expiratory volume in 1 second, and the forced expiratory volume in 1 second/ forced vital capacity ratio were calculated. Left ventricular enddiastolic diameter, ejection fraction, mitral valve prolapses, and right ventricular cavity in the apical four-chamber position were evaluated with echocardiography. Of the patients, 19.4% were in Group 1, 38.7% in Group 2, and 41.9% in Group 3. The mean Haller Index value was 3.09±0.64. According to pulmonary function test results, 16.1% of the patients had restrictive disease and 6.5% had obstructive disease. There was a negative correlation between the index and forced expiratory volume in 1 second and forced vital capacity, and there was a statistically significant decrease in these values, as the Haller Index increased (p<0.017). There was a significant difference in the ejection fraction among the groups (p<0.001) and, as the Haller Index increased, ejection fraction statistically significantly decreased. Our study results show a negative correlation between the severity of pectus excavatum and pulmonary dysfunction and, as the severity increases, left ventricular function may be affected by the deformity. As a result, there seems to be a significant relationship between the severity of the deformity and cardiopulmonary functions.

KL-6 levels in the connective tissue disease population: typical values and potential confounders-a retrospective, real-world study.

Krebs von den Lungen 6 (KL-6) is a potential biomarker for determining the severity of interstitial lung disease (ILD) in patients with connective tissue disease (CTD). Whether KL-6 levels can be affected by potential confounders such as underlying CTD patterns, patient-associated demographics, and comorbidities needs further investigation. From the database created by Xiangya Hospital, 524 patients with CTD, with or without ILD, were recruited for this retrospective analysis. Recorded data included demographic information, comorbidities, inflammatory biomarkers, autoimmune antibodies, and the KL-6 level at admission. Results of CT and pulmonary function tests were collected one week before or after KL-6 measurements. The percent of predicted diffusing capacity of the lung for carbon monoxide (DLCO%) and computed tomography (CT) scans were used to determine the severity of ILD. Univariate linear regression analysis showed that BMI, lung cancer, TB, lung infections, underlying CTD type, white blood cell (WBC) counts, neutrophil (Neu) counts, and hemoglobin (Hb) were related to KL-6 levels. Multiple linear regression confirmed that Hb and lung infections could affect KL-6 levels independently; the β were 9.64 and 315.93, and the P values were 0.015 and 0.039, respectively. CTD-ILD patients had higher levels of KL-6 (864.9 vs 463.9, P < 0.001) than those without ILD. KL-6 levels were closely correlated to the severity of ILD assessed both by CT and DLCO%. Additionally, we found that KL-6 level was an independent predictive factor for the presence of ILD and further constructed a decision tree model to rapidly determine the risk of developing ILD among CTD patients. KL-6 is a potential biomarker for gauging the incidence and severity of ILD in CTD patients. To use this typical value of KL-6, however, doctors should take Hb and the presence of lung infections into account.

Clinical Outcomes After Tracheobronchoplasty With Ringed Polytetrafluoroethylene Vascular Graft

BackgroundThis report describes the surgical technique and outcomes of tracheobronchoplasty (TBP) with ringed polytetrafluoroethylene (PTFE) vascular graft. MethodsWe identified all patients who underwent PTFE-TBP for severe expiratory central airway collapse from January 1, 2018 to August 2021 at Mayo Clinic, Florida. Preoperative and postoperative St George’s Respiratory Questionnaire (SGRQ), Cough-Specific Quality of Life Questionnaire (CSQLQ), pulmonary function testing, 6-minute walk test, and blinded dynamic bronchoscopy videos at 3-month follow-up were used to assess outcomes. ResultsFourteen patients (median age, 62.5 years; 64.3% female) underwent PTFE-TBP. The median operative time was 355 minutes, median hospital length of stay was 5 days, and median intensive care unit stay was 1 day. One patient had a Clavien-Dindo grade ≥3 complication. Comparison of preoperative and postoperative questionnaire scores demonstrated improvement in median SGRQ score by 14.79 (P = .013) and CSQLQ score by 22 (P = .005). Preoperative and postoperative pulmonary function and 6-minute walk test results showed no significant difference. Postoperative bronchoscopy demonstrated improvement in median collapsibility of mid trachea by 39.6% (P < .001), distal trachea by 50% (P < .001), left main bronchus by 38.2% (P < .001), right main bronchus by 37.9% (P < .001), and bronchus intermedius by 30.7% (P < .001). ConclusionsPTFE-TBP provides significant improvement in patients' symptoms and expiratory central airway collapse as judged by preoperative and postoperative quality of life questionnaires and bronchoscopy.

Clinical course and risk factors for development and progression of interstitial lung disease in primary Sjögren’s syndrome

This single-center, retrospective study aimed to investigate the course and prognostic factors of patients with primary Sjögren syndrome-associated interstitial lung disease (pSS-ILD). We included 120 pSS patients who underwent at least two high-resolution computed tomography (HRCT) scans between 2013 and 2021. Clinical symptoms, laboratory data, HRCT findings, and pulmonary function test results were collected. Two thoracic radiologists reviewed the HRCT findings. In patients with pSS without ILD at baseline (n = 81), no development of ILD was found on follow-up (median, 2.8 years). In patients with pSS-ILD (n = 39), total disease extent, extent of coarse reticulation, and traction bronchiectasis increased on HRCT, whereas the extent of ground glass opacity (GGO) decreased at follow-up (median, 3.2 years) (each p < 0.001). In progressive group of pSS-ILD (48.7%), the extent of coarse reticulation and coarseness score of fibrosis were increased at follow-up (p < 0.05). Usual interstitial pneumonia pattern on CT (OR, 15.237) and follow-up duration (OR, 1.403) were independent risk factors for disease progression in patients with pSS-ILD. In both progressive and non-progressive pSS-ILD, GGO decreased, whereas the extent of fibrosis increased even after treatment with glucocorticoid and/or immunosuppressants. In conclusion, progression occurred in approximately half of the pSS-ILD patients with slow gradual deterioration. Our study identified a definite group of progressive pSS-ILD who did not respond to current anti-inflammatory treatment.

Efficacy of mecasin for treatment of amyotrophic lateral sclerosis: A phase IIa multicenter randomized double-blinded placebo-controlled trial

Ethnopharmacological relevanceAmyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disorder characterized by progressive paralysis of voluntary muscles. Mecasin, the extract of modified jakyakgamchobuja-tang—a herbal preparation comprising of Radix Paeoniae Alba, Radix Glycyrrhizae, Radix Aconiti Lateralis Preparata, Radix Salviae Miltiorrhizae, Rhizoma Gastrodiae, Radix Polygalae, Curcuma Root, Fructus Chaenomelis, and Rhizoma Atractylodis Japonicae—shows neuroprotective and anti-neuroinflammatory effects and alleviates the symptoms in patients with ALS. Aim of the studyThis trial aimed to evaluate the efficacy and safety of mecasin in these patients. Material and methodsPatients were randomized to receive mecasin 1.6 g daily, mecasin 2.4 g daily, or placebo for 12 weeks. The primary endpoint was the Korean version of ALS Functional Rating Scale-Revised (K-ALSFRS-R) score. The secondary endpoints were muscular atrophy measurements, pulmonary function test results, creatine kinase levels, body weight, safety, and scores of the Medical Research Council (MRC) scale for muscle strength; Visual Analog Scale for pain (VAS pain); Hamilton Rating Scale for Depression; and Fatigue Severity Scale. ResultsAmong the 30 patients randomized, 24 completed the follow-up. Significant between-group differences were detected in the primary endpoint using the omnibus F-test. The changes in the K-ALSFRS-R score between 12 weeks and baseline were −0·25, −1·32, and −2·78 in the mecasin 1.6 g, mecasin 2.4 g, and placebo groups, respectively. The difference in the K-ALSFRS-R score between the mecasin 1.6 g and placebo groups was 2·53 points (95% confidence interval [CI]: 0·61–4·45), and that between the 2.4 g and placebo groups was 1·46 points (95% CI: 0·48–3·40). However, no significant differences were detected in the secondary endpoints (MRC: dyspnea, p = 0·139; VAS pain, p = 0·916; forced vital capacity, p = 0·373). The incidence of adverse events was similar and low in all groups. ConclusionsMecasin may retard symptomatic progression without major adverse effects. A phase IIb study to evaluate its long-term effects in ALS is ongoing.

Can CT-Based Arterial and Venous Morphological Markers of Chronic Obstructive Pulmonary Disease Explain Pulmonary Vascular Remodeling?

We analyzed changes in quantitative pulmonary artery and vein parameters to investigate pulmonary vascular remodeling characteristics in chronic obstructive pulmonary disease (COPD) patients. This retrospective study recruited healthy volunteers and COPD patients. Participants undergoing standard-of-care pulmonary function testing (PFT) and computed tomography (CT) evaluations were classified into five groups: normal and Global Initiative for Chronic Obstructive Lung Disease (GOLD) grades 1-4. Artery and vein analyses (volumes, numbers, densities, and fractions) were performed using artificial intelligence. Among 139 subjects (136 men; mean age, 64years±8 [SD]) with GOLD grade 1 (n=13), grade 2 (n=49), grade 3 (n=42), grade 4 (n=17) and control subjects (n=18) enrolled, differences in arterial volumes (BV5-10, BV10+, pulmonary arterial volume) and venous densities (BV5 density, BV10+ density, pulmonary venous density, pulmonary venous branch density) among control and GOLD grades 1-4 were statistically significant (P<.05). Higher pulmonary arterial volumes and lower number were observed with more advanced COPD. The number and volumes of pulmonary veins were lower in GOLD grades 2 and 3 than in GOLD grade 1 but higher in GOLD grade 4 than in GOLD grade 3. The numbers and volumes of pulmonary arteries and veins showed varying positive correlations (γ=0.18-0.96, P<.05). Pulmonary vascular densities were mildly to moderately correlated with PFT results (γ=0.236-0.495, P<.05) and were moderately negatively correlated with the emphysema percentage (γ=-0.591 to -0.315, P<.05). Patients with COPD exhibited pulmonary vascular remodeling, which occurred in the arteries at the early grade of COPD and in the veins at the late grade. CT-based quantitative analysis of pulmonary vasculature may become an imaging marker for early diagnosis and assessment of COPD severity.

Hemidiaphragmatic paralysis after costoclavicular approach versus other brachial plexus blocks in upper limb surgery: a meta-analysis.

The costoclavicular brachial plexus block (CCB) is a recently established technique that uses the infraclavicular approach and is performed just below the clavicle. This meta-analysis aimed to determine whether CCB can reduce the incidence of hemidiaphragmatic paralysis (HDP), which is a major adverse event related to brachial plexus block (BPB), while yielding comparable block performance as other BPB techniques. We searched electronic databases to identify relevant studies that compared the incidence of HDP between CCB and other BPB techniques. The primary outcome was the incidence of HDP following CCB and other BPB techniques. The secondary outcomes were pulmonary function test results, other adverse events, and block performance parameters such as onset and performance time. We included six randomized controlled trials that included 414 patients. Compared with the other BPB group, the CCB group had a significantly lower incidence of HDP (relative ratio: 0.21, 95% CI [0.12, 0.36], P < 0.001) and higher peak expiratory flow rate (mean difference: 0.68 L/s, 95% CI [0.13, 1.23], P = 0.015). There were no significant between-group differences with respect to other adverse events and block performance parameters. Compared with other BPB techniques, CCB involves a lower incidence of HDP with comparable onset and performance time.

Long-Term Radiographic and Pulmonary Function Outcomes After Dual Growing-Rod Treatment for Severe Early-Onset Scoliosis.

We evaluated long-term outcomes (radiographic parameters and pulmonary function) at a minimum follow-up of 5 years after use of dual growing rods (DGRs) to treat severe early-onset scoliosis (sEOS). Among a total of 112 patients who were diagnosed with early-onset scoliosis (EOS) and were treated with DGRs between 2006 and 2015, 52 patients had sEOS, with a major Cobb angle of >80°. Of these patients, 39 with a minimum follow-up of 5 years had complete radiographic and pulmonary function test results and were included. The Cobb angle of the major curve, T1-S1 height, T1-T12 height, and maximum kyphosis angle in the sagittal plane were measured on radiographs. Pulmonary function test results were collected in all patients before the initial operation (preoperatively), 12 months after the initial operation (postoperatively), and at the last follow-up. The changes in pulmonary function and complications during treatment were analyzed. The mean age of patients before the initial operation was 7.7 ± 1.2 years, and the mean follow-up period was 75.0 ± 14.1 months. The mean number of lengthenings was 4.5 ± 1.3, and the mean interval between lengthenings was 11.2 ± 2.1 months. The Cobb angle improved from 104.5° ± 18.2° preoperatively to 38.1° ± 10.1° after the initial surgical procedure (postoperatively) and 21.9° ± 8.6° at the final follow-up. The T1-S1 height increased from 25.1 ± 4.0 cm preoperatively to 32.4 ± 3.5 cm postoperatively and to 39.5 ± 4.0 cm at the final follow-up. However, no significant difference was detected between the increased pulmonary function parameters at 1 year and those before the operation (p > 0.05), except for residual volume, whereas pulmonary function parameters had significantly increased at the final follow-up (p < 0.05). During the treatment period, 17 complications occurred in 12 patients. DGRs are effective in treating sEOS in the long term. They allow longitudinal growth of the spine, and the correction of the spinal deformity can provide conditions that make improving pulmonary function possible in patients with sEOS. Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence.

Exertional Desaturation Is More Severe in Idiopathic Pulmonary Fibrosis Than in Other Interstitial Lung Diseases.

Interstitial lung disease (ILD) is classified into several disease groups. Among them, idiopathic pulmonary fibrosis (IPF) has higher incidence and poor prognosis; therefore, it is important to characterize specific IPF symptoms. Exercise desaturation is a strong factor related to mortality in patients with ILD. Thus, the purpose of this study was to compare the degree of oxygen desaturation between IPF and other ILD (non-IPF ILD) patients during exercise, using the 6-minute walk test (6MWT). This retrospective study included 126 stable patients with ILD who underwent 6MWT in our outpatient department. The 6MWT was used to assess desaturation during exercise, 6-minute walk distance (6MWD), and dyspnea at the end of exercise. In addition, patient characteristics and pulmonary function test results were recorded. Study subjects were divided into 51 IPF patients and 75 non-IPF ILD patients. The IPF group had significantly lower nadir oxygen saturation determined by pulse oximetry (SpO2) during 6MWT than the non-IPF ILD group (IPF, 86.5 ± 4.6%; non-IPF ILD, 88.7 ± 5.3%; p = 0.02). The significant association between the nadir SpO2 and IPF or non-IPF ILD grouping remained even after adjusting for gender, age, body mass index, lung function, 6MWD, and dyspnea (β = -1.62; p <0.05). Even after adjusting for confounding factors, IPF patients had lower nadir SpO2 during 6MWT. Early assessment of exercise desaturation using the 6MWT may be more important in patients with IPF compared with patients with other ILDs.

Can transbronchial lung cryobiopsy benefit adaptive treatment strategies in connective tissue disease-associated interstitial lung disease?

BackgroundSome patients with connective tissue disease (CTD)-associated interstitial lung disease (ILD) progress to pulmonary fibrosis over their disease course despite initial improvement, potentially indicating a poor prognosis. Transbronchial lung cryobiopsy (TBLC) is a new bioptic approach used in diffuse parenchymal lung diseases. This study of CTD-ILD assessed the utility of TBLC in determining therapeutic decision-making strategies.MethodsWe analyzed medical records of 31 consecutive CTD-ILD patients who underwent TBLC focusing on radio-pathological correlation and disease course. A TBLC-based usual interstitial pneumonia (UIP) score was used that assessed three morphologic descriptors: i) patchy fibrosis, ii) fibroblastic foci, and iii) honeycombing.ResultsAmong the patients with CTD-ILD, 3 had rheumatoid arthritis, 2 systemic sclerosis, 5 polymyositis/dermatomyositis, 8 anti-synthetase syndrome, 6 Sjögren’s syndrome, and 5 had microscopic polyangiitis. Pulmonary function test results showed a mean %FVC of 82.4% and %DLCO of 67.7%. Among the 10 CTD patients and TBLC-proven pathological UIP, 3 patients had prominent inflammatory cells in addition to a framework of UIP, and pulmonary function of most patients improved with anti-inflammatory agents. Six (40%) of 15 patients with TBLC-based UIP score ≥ 1 had a progressive disease course during follow-up, of whom 4 patients received anti-fibrotic agents.ConclusionsTBLC in patients with CTD-ILD can help determine an appropriate medication strategy, particularly when UIP-like lesions are present. TBLC may be useful when judging which agents to prioritize, anti-inflammatory or anti-fibrotic, is difficult. Moreover, additional information from TBLC may be beneficial when considering early intervention with anti-fibrotic agents in clinical practice.

SMARCA4-deficient undifferentiated thoracic tumor: A case report

SMARCA4-deficient undifferentiated tumors (SMARCA4-DUTs) present with diverse clinical manifestations and progress to metastasis and even cause death within a few months. This novel subset of undifferentiated tumors occurs in the middle-aged population and is strongly associated with a smoking history. Distinguishing it from other malignancies is challenging. A 62-year-old man presented with chest pain for 7 d. The patient had no respiratory symptoms and normal pulmonary function test results. The patient had been a smoker for 8 years and quit smoking 2 years ago. Chest computed tomography revealed a huge mass involving the left upper and lower lung lobes with pericardial invasion and multiple metastases. Tumor samples were obtained using open frozen biopsy, after several unsuccessful attempts. The tumor was composed of sheets of undifferentiated disclosive cells with vesicular nuclei and prominent nucleoli. The differential diagnosis included high-grade lymphoma, germ cell tumor, NUT carcinoma, undifferentiated carcinoma, and sarcoma. The tumor cells were large, arranged in sheets, and did not exhibit glandular or squamous differentiation. Frequent foci of necrosis were noted. There was no evidence of epithelial differentiation on immunohistochemical staining. The SMARCA4 stain showed complete loss of expression of SMARCA4, which is diagnostic. In the present case, thoracic SMARCA4-DUT was diagnosed based on clinical features, absence of epithelial differentiation, and negative SMARCA4 expression.

Breathing, Obstruction, Restriction, and Gas Exchange: A Pulmonary Function Testing Interpretation Framework for Novice Learners.

Pulmonary function testing (PFT) is a common method of assessing patients with respiratory symptoms, yet exposure to PFT is variable throughout medical training. Therefore, incorporating a dedicated approach to teaching PFT into the formal medical education curriculum can ensure that trainees become familiar with both the relevant physiologic principles involved in interpreting PFT results and the indications for performing PFT in clinical practice. In this "How I Teach" article, we present breathing, obstruction, restriction, and gas exchange (BORG), a novel, small-group workshop designed to teach novice learners a sequential framework for PFT interpretation. The BORG workshop comprises two segments: a whiteboard minilecture that illustrates the BORG framework and a case-based worksheet whereby learners apply this approach to sets of PFTs with increasing difficulty. Our workshop is grounded in two cognitive psychology frameworks: the cognitive theory of multimedia learning and the dual-process theory. We provide three figures and four supplementary videos to illustrate our workshop's design and delivery, as well as both learner and instructor versions of our BORG worksheet. Last, we address three PFT concepts that have challenged us as instructors and provide evidence-based teaching scripts. The BORG workshop can be used by medical educators working with medical students and residents as a means of helping learners progress along the continuum from a basic understanding of spirometry to independent analysis and interpretation of PFTs to application of PFT results to medical decision making.

Saccadic oscillations as a biomarker of clinical symptoms in amyotrophic lateral sclerosis.

Among eye movements in amyotrophic lateral sclerosis (ALS), we identified the characteristics of square-wave jerks (SWJs) seen during times without visual fixation (VF) and analyzed their relationships with clinical parameters. Clinical symptoms were evaluated and eye movements were tested using electronystagmography in 15 patients with ALS (10 men, 5 women; mean age, 66.9 ± 10.5years). SWJs with and without VF were recorded, and their characteristics were identified. Relationships between each SWJ parameter and clinical symptoms were evaluated. Results were compared with eye movement data from 18 healthy individuals. The frequency of SWJs without VF was significantly higher in the ALS group than in the healthy group (P < 0.001). When the condition was changed from VF to no-VF in the ALS group, the frequency of SWJs was significantly higher in healthy subjects (P = 0.004). A positive correlation was seen between frequency of SWJs and percentage predicted forced vital capacity (%FVC) (R = 0.546, P = 0.035). The frequency of SWJs was higher with VF in healthy people, and was suppressed without VF. In contrast, the frequency of SWJs was not suppressed without VF in ALS patients. This suggests that SWJs without VF have some clinical significance in ALS patients. Moreover, a relationship was noted between the parameters of SWJs without VF in ALS patients and results of pulmonary function tests, suggesting that SWJs during times without VF may offer a clinical parameter of ALS.

Pulmonary and cardiac involvement in patients with rheumatoid arthritis and ankylosing spondylitis

Objectives: Our aim is to study the disease activity status, laboratory data and to determine the frequency of cardiac and pulmonary findings in patients with Rheumatoid arthritis (RA) and ankylosing spondylitis (AS). Methods: Forty-five AS patients and 78 RA patients took part in the study. The C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), albumin, creatinine, ALT, leukocyte, neutrophil ratio, hemoglobin, platelet, rheumatoid factor (RF), and anti-cyclic citrullinated peptide (anti-CCP) levels were measured. In addition, the patients' thoracic computed tomography, high-resolution computed tomography (HRCT), echocardiography (ECHO), and pulmonary function test (PFT) results were evaluated. Disease activity was assessed with the Disease Activity Score-28 (DAS-28) in RA patients, whereas the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) was used in patients with AS. Results: The relationship between the anti-CCP value and gender was significant in RA patients. Significant differences were present between gender and hemoglobin value in RA and AS patients, whereas no significant differences were determined between CRP, ESR, platelet, and DAS-28 values. There was a significant difference between RF and ESR values in RA patients, whereas no significant difference was present between CRP, leukocyte, DAS-28, hemoglobin, and platelet values. Pulmonary involvement was determined in 35.7% of RA patients and 4.2% of AS patients. The difference between these was statistically significant. Conclusions: The most common pulmonary involvement is millimetric nonspecific nodule and the most common lesion among ECHO findings was left ventricular diastolic dysfunction in RA and AS patients. In RA, no significant difference was determined between PFT and DAS-28 results, as a result, the disease activity will not directly indicate pulmonary involvement.

Computed Tomography Radiomics-based Prediction Model for Gender–Age–Physiology Staging of Connective Tissue Disease-associated Interstitial Lung Disease

To analyze the feasibility of predicting gender-age-physiology (GAP) staging in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) by radiomics based on computed tomography (CT) of the chest. Chest CT images of 184 patients with CTD-ILD were retrospectively analyzed. GAP staging was performed on the basis of gender, age, and pulmonary function test results. GAP I, II, and III have 137, 36, and 11 cases, respectively. The cases in GAP Ⅱ and Ⅲ were then combined into one group, and the two groups of patients were randomly divided into the training and testing groups with a 7:3 ratio. The radiomics features were extracted using AK software. Multivariate logistic regression analysis was then conducted to establish a radiomics model. A nomogram model was established on the basis of Rad-score and clinical factors (age and gender). For the radiomics model, four significant radiomics features were selected to construct the model and showed excellent ability to differentiate GAP I from GAP Ⅱ and Ⅲ in both the training group (the area under the curve [AUC]=0.803, 95% confidence interval [CI]: 0.724-0.874) and testing group (AUC=0.801, 95% CI:0.663-0.912). The nomogram model that combined clinical factors and radiomics features improved higher accuracy of both training (88.4% vs. 82.1%) and testing (83.3% vs. 79.2%). The disease severity assessment of patients with CTD-ILD can be evaluated by applying the radiomics method based on CT images. The nomogram model demonstrates better performance for predicting the GAP staging.

The association between oxidative balance score and lung function: A nationwide cross-sectional survey

PurposeGiven the impact of oxidative imbalance on the development of airway pathologies, this study was undertaken to investigate the association between oxidative balance (OB) scores and lung function in the adult Korean population. MethodsData of 17,368 adults with available OB scores and pulmonary function test results were extracted from the 2013–2019 Korean National Health and Nutrition Examination Surveys. Multivariable logistic regression models were used to calculate the odds ratios (ORs) and 95% confidence intervals (CIs) for reduced forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) per 1-point decrease in OB score. Dose dependent association of reduced lung function with OB scores was also investigated. ResultsMales, low-income subjects, individuals with comorbidities, and those with reduced pulmonary function had lower OB scores (representing oxidative balance). Overall, the association between oxidative imbalance and reduced lung function was remarkable in FVC than FEV1 (OR [95% CI], 1.06 [1.04–1.07] vs. 1.03 [1.02–1.04]; both p < 0.001). Linear relationships between the level of reduced lung function and OB scores were significantly noted (p for trend<0.001 in both FEV1 and FVC). ConclusionOur findings suggest that oxidative imbalance is associated with reduced pulmonary function.