Pulmonary Function Abnormalities Research Articles

Pulmonary function as a continuum of risk: critical care utilization and survival after allogeneic hematopoietic stem cell transplantation - a multicenter cohort study.

Abnormal pre-transplant pulmonary function tests (PFTs) are associated with reduced survival after allogeneic HCT. Existing scoring systems consider risk dichotomously, attributing risk only to those with abnormal lung function. In a multicenter cohort of 1717 allo-HCT recipients, we examined the association between pre-transplant PFT measures and need for ICU admission (120d), frequency of mechanical ventilation (120d) and overall survival (5 y). Predictive models were developed and validated using Cox proportional hazards, incorporating age, FEV1 (forced expiratory volume in 1-second) and diffusing capacity (DLCO). In univariate analysis, hazard ratios for each outcome (95% CI) were: mechanical ventilation (FEV1: 0.60 [0.52-0.69], DLCO: 0.69 [0.61-0.77], p < 0.001), ICU admission (FEV1: 0.74 [0.67-0.82], DLCO: 0.79 [0.72-0.86], p < 0.001) and overall survival (FEV1: HR 0.87 [0.81-0.94], DLCO: 0.83 [0.77-0.89], p < 0.001). A multivariable Cox model was developed and compared to the HCT-CI Pulmonary score in a validation cohort. This model was better at predicting need for ICU admission and mechanical ventilation, while both models predicted overall survival (p < 0.001). In conclusion, the risk conferred by pre-transplant pulmonary function should be considered in a continuous rather than dichotomous manner. A more granular prognostication system can better inform risk of critical care utilization in the early post-HCT period.

A cross-sectional study on the correlation of pulmonary function with duration of Rheumatoid arthritis

Rheumatoid arthritis (R.A.) is characterized by chronic inflammatory symmetrical peripheral polyarthritis associated with systemic involvement. Pulmonary involvement is one of them. Therefore, early assessment of pulmonary function may help identify the type of respiratory involvement and hence can contribute to properly managing pulmonary complications.The study aims to evaluate the type and extent of pulmonary function abnormality in relation to the duration of Rheumatoid Arthritis. Settings and Design: tertiary care treating hospital- a cross-sectional observational study A cross-sectional study was conducted at Department of General Medicine in forty R.A. cases in whom spirometry was performed and values of spirometry results were statistically compared with the duration of R.A. using S.P.S.S. software. Cases were grouped into three categories based on the duration of the disease. Category A –less than 5 yrs, category B– 5 to 9 yrs and category C – more than 9 yrs duration. In category A, abnormal pulmonary function test (PFT) was observed in 43.5% of cases in the form of obstructive 17.4%, restrictive 17.4% and mixed pattern in 8.7% of cases. In category B, the restrictive pattern was found in 63.6% and a mixed pattern in 9.1% of cases. However, abnormal pulmonary function test (PFT) was observed in all cases in category C, with obstructive pattern in 16.7%, restrictive 66.7% and mixed 16.7% cases. From the study, it was found that early pulmonary involvement occurs in R.A. With an increase in the duration of R.A., pulmonary involvement becomes a more severe and restrictive pattern. Most cases with a duration of more than 9yrs had significant pulmonary involvement either in the form of restrictive or obstructive pattern. Hence PFT should be done at the earliest as a routine and repeated periodically in R.A. cases. 

Long-term Radiological and Pulmonary Function Abnormalities at 3-year post COVID-19 Hospitalization: A Longitudinal Cohort Study.

This study aimed to evaluate the longitudinal progression of residual lung abnormalities (ground-glass opacities, reticulations, and fibrotic-like changes) and pulmonary function, three years following coronavirus disease 2019(COVID-19). This prospective, longitudinal cohort study enrolled COVID-19 survivors who exhibited residual lung abnormalities upon discharge from two hospitals. Follow-up assessments were conducted at 6 months, 12 months, 2 years, and 3 years post-discharge, and included pulmonary function tests, 6-minute walk distance (6MWD), chest CT scans, and symptom questionnaires. Non-COVID-19 controls were retrospectively recruited for comparative analysis. 728 COVID-19 survivors and 792 controls were included. From 6 months to 3 years, there was a gradual improvement in reduced diffusing capacity of the lungs for carbon monoxide (DLCO＜80% predicted, 49% versus 38%, p=0.001), 6MWD (496 m versus 510 m, p=0.002) and residual lung abnormalities(46% versus 36%, p<0.001), regardless of the disease severity. Patients with residual lung abnormalities at 3 years more commonly had respiratory symptoms (32% versus 16%, p<0.001), lower 6MWD (494 m versus 510 m, p=0.003), and abnormal DLCO (57% versus 27%, p<0.001) compared to those with complete resolution. Compared to the controls, the proportion of DLCO impairment (38% versus 17%, p<0.001) and respiratory symptoms (23% versus 2.2%, p<0.001) were significantly higher in the matched COVID-19 survivors at the 3-year follow-up. Most patients exhibited improvement in radiological abnormalities and pulmonary function over time following COVID-19. However, more than one-third continued to have persistent lung abnormalities at the 3-year mark, which were associated with respiratory symptoms and reduced diffusion capacity.

A toxicology study of Csf2ra complementation and pulmonary macrophage transplantation therapy of hereditary PAP in mice

Pulmonary macrophage transplantation (PMT) is a gene and cell transplantation approach in development as therapy for hereditary pulmonary alveolar proteinosis (hPAP), a surfactant accumulation disorder caused by mutations in CSF2RA/B (and murine homologs). We conducted a toxicology study of PMT of Csf2ra gene-corrected macrophages (mGM-Rα+Mϕs) or saline-control intervention in Csf2raKO or wild-type (WT) mice including single ascending dose andrepeat ascending dose studies evaluating safety, tolerability, pharmacokinetics, and pharmacodynamics. Lentiviral-mediated Csf2ra cDNA transfer restored GM-CSF signaling in mGM-Rα+Mϕs. Following PMT, mGM-Rα+Mϕs engrafted, remained within the lungs, and did not undergo uncontrolled proliferation or result in bronchospasm, pulmonary function abnormalities, pulmonary or systemic inflammation, anti-transgene product antibodies, or pulmonary fibrosis. Aggressive male fighting caused a similarly low rate of serious adverse events in saline- and PMT-treated mice. Transient, minor pulmonary neutrophilia and exacerbation of pre-existing hPAP-related lymphocytosis were observed 14days after PMT of the safety margin dose but not the target dose (5,000,000 or 500,000 mGM-Rα+Mϕs, respectively) and only in Csf2raKO mice but not in WT mice. PMT reduced lung disease severity in Csf2raKO mice. Results indicate PMT of mGM-Rα+Mϕs was safe, well tolerated, and therapeutically efficacious in Csf2raKO mice, andestablished a no adverse effect level and 10-fold safety margin.

Incidence and risk factors for long COVID in children with COVID-19 pneumonia.

There are only a few reports of long COVID including pulmonary function in children after COVID-19 pneumonia. We determined the incidence of long COVID and abnormal pulmonary function in those children and identify risk factors. This cohort study enrolled children admitted with COVID-19 pneumonia during 2021-2022. We gathered clinical characteristics during admission and at follow-up 3 months after. We determined the incidence of long COVID at 39.7% (95% confidence interval [CI]: 30.7%-49.1%). All severe pneumonia cases consistently reported persistent symptoms. Exercise intolerance, cough, and fatigue were the three most common persistent symptoms in 26 (22.4%), 21 (18.1%), and 18 (15.5%) of the patients, respectively. At the follow-up, 21 cases (18.1%) demonstrated persistent abnormal chest radiographs. Three cases (6.9%) demonstrated restrictive ventilatory defects. Among those, one case (2.3%) demonstrated concomitant diffusion defect. Three cases (6.0%) demonstrated exercise-induced hypoxemia after the 6-minute walk test. Comparing spirometry variables between children with long COVID and without revealed significant difference of FEF25-75 (z score) between two groups. Age [adjusted OR (95% CI): 1.13 (1.05-1.22), p value 0.002], allergic diseases [adjusted OR (95% CI): 4.05 (1.36-12.06), p value 0.012], and living in polluted areas [adjusted OR (95% CI): 2.73 (1.18-6.33), p value 0.019] were significantly associated with long COVID. A significant percentage of children developed long COVID after COVID-19 pneumonia. We should give additional attention to those who have exercise intolerance, chronic cough, or fatigue, especially older children, severe cases, children with allergic diseases, and those living in polluted areas.

Pulmonary Function Abnormalities and their Association in Children with Beta-Thalassemia Major Age (10-18 years)

Pulmonary Function Abnormalities and their Association in Children with Beta-Thalassemia Major Age (10-18 years)

Pulmonary function in children post -SARS-CoV-2 infection: a systematic review and meta-analysis

ObjectiveThere are some concerns regarding long-term complications of COVID-19 in children. A systematic review and meta-analysis was performed evaluating the respiratory symptoms and pulmonary function, post-SARS-CoV-2 infection.MethodsA systematic search was performed in databases up to 30 March 2023. Studies evaluating respiratory symptoms and pulmonary function after COVID-19 infection in children were selected. The major outcomes were the frequency of respiratory symptoms and the mean of spirometry parameters. A pooled mean with 95% confidence intervals (CIs) was calculated.ResultsA total of 8 articles with 386 patients were included in meta-analysis. Dyspnea, cough, exercise intolerance, and fatigue were the most common symptoms. The meta-mean of forced expiratory volume (FEV1) and forced vital capacity (FVC) was 101.72%, 95% CI= (98.72, 104.73) and 101.31%, 95% CI= (95.44, 107.18) respectively. The meta-mean of FEV1/FVC and Forced expiratory flow at 25 and 75% was 96.16%, 95% CI= (90.47, 101.85) and 105.05%, 95% CI= (101.74, 108.36) respectively. The meta-mean of diffusing capacity for carbon monoxide was 105.30%, 95%CI= (88.12, 122.49). There was no significant difference in spirometry parameters before and after bronchodilator inhalation.ConclusionsDespite some clinical respiratory symptoms, meta-results showed no abnormality in pulmonary function in follow-up of children with SARS-CoV-2 infection. Disease severity and asthma background had not confounded this outcome.

Clinical Profile of Post-COVID-19 Patients with Persistent Chest Pain: A Cross-sectional Study

Introduction: Chest pain in post-COVID-19 patients can be due to serious alarming post-COVID-19 sequelae, such as Coronary Artery Disease (CAD), pulmonary embolism, myocarditis, etc. Approximately one-fifth of patients attending clinical Outpatient Department (OPD) following COVID-19 recovery present with persistent chest pain. There is limited knowledge about the clinical profile of patients experiencing persistent post-COVID19 chest pain. Aim: To describe the clinical profile of such patients to fill the knowledge gap and acquire new insights into patients with post-COVID-19 persistent chest pain. Materials and Methods: A cross-sectional study was conducted, enrolling a total of 259 patients with persistent chest pain (i.e., chest pain lasting more than 24 weeks after COVID-19 diagnosis). After detailed history-taking and clinical and laboratory examinations, observed data were collected, compiled, evaluated, and analysed to achieve the study objectives. Results: Out of 259 patients, 133 (51.4%) had cardiac {85 (32.8%)} or pulmonary {48 (18.5%)} abnormalities. Among patients with cardiac abnormalities, CAD, arrhythmia, myocarditis/cardiomyopathy, pericarditis, and PAH were detected in 38 (14.6%), 18 (6.9%), 7 (2.7%), 11 (4.2%), and 11 (4.2%), respectively. Meanwhile, among patients with pulmonary abnormalities, 36 (13.9%) patients had organic residual lesions in the lung parenchyma, 7 (2.7%) had pleuritis, and 28 (10.8%) had pulmonary function abnormalities. The remaining 126 (48.6%) patients experienced chest pain due to non cardiopulmonary aetiologies like gastrointestinal {45 (17.3%)}, musculoskeletal {38 (14.6%)}, psychomotor {35 (13.5%)}, autonomic {8 (3.3%)}, etc. Conclusion: This study found that chest pain in post-COVID-19 patients arises due to multisystemic aetiologies such as cardiac, pulmonary, visceral, autonomic, psychomotor, musculoskeletal, etc. A wide spectrum of serious cardiac abnormalities (such as CAD, arrhythmia, myocarditis, pericarditis, PAH, etc.) contributes to about 1/3rd of cases of persistent chest pain in post-COVID-19 patients.

A prospective phase II single-arm study and predictive factor analysis of irinotecan as third-line treatment in patients with metastatic gastric cancer.

Currently, there is no recommended standard third-line chemotherapy for metastatic gastric cancer. In this study, we aimed to evaluate irinotecan's efficacy and safety in treating metastatic gastric cancer after the failure of first- and second-line chemotherapy. Prospective single-arm, two-center, phase II trial. Patients were aged 18-70 years, with histologically confirmed gastric adenocarcinoma and an Eastern Cooperative Oncology Group performance status of 0-1, progressed during or within 3 months following the last administration of second-line chemotherapy and had no other severe hematologic, cardiac, pulmonary, hepatic, or renal functional abnormalities or immunodeficiency diseases. Eligible patients received 28-day cycles of irinotecan (180 mg/m2 intravenously, days 1 and 15) and were assessed according to the RECIST 1.1 criteria every two cycles. Patients who discontinued treatment for any reason were followed up every 2 months until death. The primary endpoint was overall survival (OS), and the secondary endpoints were progression-free survival (PFS), objective response rate (ORR), disease control rate (DCR), and toxicity. A total of 98 eligible patients were enrolled in this study. In the intention-to-treat population, the median OS was 7.17 months, the median PFS was 3.47 months, and the ORR and DCR were 4.08% and 47.96%, respectively. In the per-protocol population, the median OS was 7.77 months, the median PFS was 3.47 months, and the ORR and DCR were 4.82% and 50.60%, respectively. The incidence of grade 3 or 4 hematological and non-hematological toxicities was 19.4%, and none of the patients died owing to adverse events. Cox regression analysis revealed neutropenia and baseline thrombocyte levels were independently correlated with PFS and OS. Irinotecan monotherapy is an efficient, well-tolerated, and economical third-line treatment for patients with metastatic gastric cancer as a third-line treatment. ClinicalTrials.gov identifier: NCT02662959.

Correlation between chest computed tomography findings and pulmonary function test results in the post-recovery phase of COVID-19.

The radiological and functional lung sequelae in COVID-19 survivors remain unclear. We compared the chest computed tomography findings of COVID-19 patients with normal and abnormal pulmonary function test results in the post-recovery phase. The data of consecutive patients who underwent pulmonary function tests and chest computed tomography within 14 days after recovery from COVID-19 at two medical centers between May and October 2020 were collected retrospectively. Two thoracic radiologists who were blinded to the clinical information and pulmonary function test results classified the patients according to the computed tomography features, evidence of fibrotic-like changes, and semi-quantitative quantification of the extent of pulmonary abnormalities. The clinical characteristics and computed tomography findings of patients with normal pulmonary function test results were compared with those of patients with abnormal results. A total of 101 COVID-19 survivors, comprising 48 ambulatory and 53 hospitalized patients, were included at a median of 95 days from initial symptom onset. Computed tomography revealed fibrotic-like changes in 10.9% of patients. A reduction in the diffusion capacity of carbon monoxide was the most common lung function abnormality (19.8%). Abnormal diffusion capacity of carbon monoxide was associated with the presence and extension of lung opacities on chest computed tomography scans and fibrotic pulmonary abnormalities. The sensitivity, specificity, and accuracy of reduced diffusion capacity of carbon monoxide for detecting fibrotic-like pulmonary changes on chest computed tomography scans were 72.7%, 87.8%, and 86.1%, respectively. Our study suggests that the presence of an abnormal diffusion capacity of carbon monoxide in the post-recovery phase of COVID-19 is associated with a greater risk of long-term parenchymal lung disease, as evidenced by the presence of fibrotic-like changes on chest computed tomography scans, such as traction bronchiectasis and architectural distortion.

Association Between Pretreatment Chest Imaging and Immune Checkpoint Inhibitor Pneumonitis Among Patients With Lung Cancer.

Immune checkpoint inhibitors (ICIs) are a first-line and perioperative treatment for lung cancer. Pneumonitis is a potentially life-threatening complication of ICI treatment in 2% to 5% of patients; however, risk factors for developing ICI pneumonitis (ICI-p) remain undefined. We conducted a retrospective cohort study of consecutive patients with lung cancer who received at least one dose of ICI from 2015 through 2020 at The Ohio State University. Pneumonitis cases were documented by the treating oncologist and retrospectively evaluated for agreement between an oncologist and a pulmonologist. Patient demographic and clinical characteristics were recorded and summarized between those with and without pneumonitis for the overall cohort. Univariate and multivariable survival analyses using the Fine-Gray competing risk model were used to examine the associations. A total of 471 patients with lung cancer were included, of which 402 had non-small cell lung cancer and 69 had small cell lung cancer; 39 (8%) patients in the overall cohort developed ICI-p. Preexisting interstitial abnormalities and prior chest radiation were both significantly associated with ICI-p on univariate analysis (hazard ratio [HR], 8.91; 95% CI, 4.69-16.92; P<.001; and HR, 2.81; 95% CI, 1.50-5.28; P=.001). On multivariable analyses, interstitial abnormalities remained a strong independent risk factor for ICI-p when controlling for chest radiation and type of immunotherapy (HR, 9.77; 95% CI, 5.17-18.46; P<.001). Among patients with ICI-p (n=39), those with severe (grade 3-5) pneumonitis had worse overall survival compared with those with mild (grade 1 or 2) pneumonitis (P=.001). Abnormal pulmonary function test results at both 12 and 18 months prior to ICI initiation were not significantly associated with ICI-p. Preexisting interstitial abnormalities on chest CT and prior chest radiation are independent risk factors that are strongly associated with ICI-p in patients with lung cancer. These findings highlight a potential need for closer observation for ICI-p among patients with these risk factors.

Longitudinal assessment of lung function in Swiss childhood cancer survivors-A multicenter cohort study.

Childhood cancer survivors are at risk for pulmonary morbidity due to exposure to lung-toxic treatments, including specific chemotherapeutics, radiotherapy, and surgery. Longitudinal data on lung function and its change over time are scarce. We investigated lung function trajectories in survivors over time and the association with lung-toxic treatments. This retrospective, multicenter cohort study included Swiss survivors diagnosed between 1990 and 2013 and exposed to lung-toxic chemotherapeutics or thoracic radiotherapy. Pulmonary function tests (PFTs), including forced expiration volume in the first second (FEV1), forced vital capacity (FVC), FEV1/FVC, total lung capacity, and diffusion capacity of the lung for carbon monoxide, were obtained from hospital charts. We calculated z-scores and percentage predicted, described lung function over time, and determined risk factors for change in FEV1 and FVC using multivariable linear regression. We included 790 PFTs from 183 survivors, with a median age of 12 years at diagnosis and 5.5 years of follow-up. Most common diagnosis was lymphoma (55%). Half (49%) of survivors had at least one abnormal pulmonary function parameter, mainly restrictive (22%). Trajectories of FEV1 and FVC started at z-scores of -1.5 at diagnosis and remained low throughout follow-up. Survivors treated with thoracic surgery started particularly low, with an FEV1 of -1.08 z-scores (-2.02 to -0.15) and an FVC of -1.42 z-scores (-2.27 to -0.57) compared to those without surgery. Reduced pulmonary function was frequent but mainly of mild to moderate severity. Nevertheless, more research and long-term surveillance of this vulnerable population is needed.

Risk factors and biomarkers for post-tuberculosis lung damage in a Chinese cohort of male smokers and non-smokers: protocol for a prospective observational study

IntroductionPost-tuberculosis lung damage (PTLD) refers to the residual pulmonary impairment following the completion of antituberculosis (TB) therapy, characterised by persistent respiratory symptoms and abnormal pulmonary function. The risk factors and...

Molecular insights using spatial transcriptomics of the distal lung in congenital diaphragmatic hernia.

Abnormal pulmonary vascular development and function in congenital diaphragmatic hernia (CDH) is a significant factor leading to pulmonary hypertension. The lung is a very heterogenous organ and has marked cellular diversity that is differentially responsive to injury and therapeutic agents. Spatial transcriptomics provides the unmatched capability of discerning the differences in the transcriptional signature of these distinct cell subpopulations in the lung with regional specificity. We hypothesized that the distal lung parenchyma (selected as a region of interest) would show a distinct transcriptomic profile in the CDH lung compared with control (normal lung). We subjected lung sections obtained from male and female CDH and control neonates to spatial transcriptomics using the Nanostring GeoMx platform. Spatial transcriptomic analysis of the human CDH and control lung revealed key differences in the gene expression signature. Increased expression of alveolar epithelial-related genes (SFTPA1 and SFTPC) and angiogenesis-related genes (EPAS1 and FHL1) was seen in control lungs compared with CDH lungs. Response to vitamin A was enriched in the control lungs as opposed to abnormality of the coagulation cascade and TNF-alpha signaling via NF-kappa B in the CDH lung parenchyma. In male patients with CDH, higher expression of COL1A1 (ECM remodeling) and CD163 was seen. Increased type 2 alveolar epithelial cells (AT-2) and arterial and lung capillary endothelial cells were seen in control lung samples compared with CDH lung samples. To the best of our knowledge, this is the first use of spatial transcriptomics in patients with CDH that identifies the contribution of different lung cellular subpopulations in CDH pathophysiology and highlights sex-specific differences.NEW & NOTEWORTHY This is the first use of spatial transcriptomics in patients with congenital diaphragmatic hernia (CDH) that identifies the contribution of different lung cellular subpopulations in CDH pathophysiology and highlights sex-specific differences.

Pulmonary sarcoidosis: A comprehensive review: Past to present

Sarcoidosis is a sterile non-necrotizing granulomatous disease without known causes that can involve multiple organs with a predilection for the lung and thoracic lymph nodes. Worldwide it is estimated to affect 2–160/100,000 people and has a mortality rate over 5 years of approximately 7%. For sarcoidosis patients, the cause of death is due to sarcoid in 60% of the cases, of which up to 80% are from advanced cardiopulmonary failure (pulmonary hypertension and respiratory microbial infections) in all races except in Japan were greater than 70% of the sarcoidosis deaths are due to cardiac sarcoidosis.Scadding stages for pulmonary sarcoidosis associates with clinical outcomes. Stages I and II have radiographic remission in approximately 30%–80% of cases. Stage III only has a 10%–40% chance of resolution, while stage IV has no change of resolution. Up to 40% of pulmonary sarcoidosis patients progress to stage IV disease with lung parenchyma fibroplasia, bronchiectasis with hilar retraction and fibrocystic disease. These patients are at highest risk for the development of precapillary pulmonary hypertension, which may occur in up to 70% of these patients. Sarcoid patients with pre-capillary pulmonary hypertension can respond to targeted pulmonary arterial hypertension medications. Stage IV fibrocytic sarcoidosis with significant pulmonary physiologic impairment, >20% fibrosis on HRCT or pre-capillary pulmonary hypertension have the highest risk of mortality, which can be >40% at 5-years.First line treatment for patients who are symptomatic (cough and dyspnea) with parenchymal infiltrates and abnormal pulmonary function testing (PFT) is oral glucocorticoids, such as prednisone with a typical starting dose of 20–40 mg daily for 2 weeks to 2 months. Prednisone can be tapered over 6–18 months if symptoms, spirometry, PFTs, and radiographs improve. Prolonged prednisone may be required to stabilize disease. Patients requiring prolonged prednisone ≥10 mg/day or those with adverse effects due to glucocorticoids may be prescribed second and third line treatements. Second and third line treatments include immunosuppressive agents (e.g., methotrexate and azathioprine) and anti-tumor necrosis factor (TNF) medication; respectively. Effective treatments for advanced fibrocystic pulmonary disease are being explored. Despite different treatments, relapse rates range from 13% to 75% depending on the stage of sarcoid, number of organs involved, socioeconomic status, and geography. ConclusionThe mortality rate for sarcoidosis over a 5 year follow up is approximately 7%. Unfortunately, 10%–40% of patients with sarcoidosis develop progressive pulmonary disease, and >60% of deaths resulting from sarcoidosis are due to advance cardiopulmonary disease. Oral glucocorticoids are the first line treatment, while methotrexate and azathioprine are considered second and anti-TNF agents are third line treatments that are used solely or as glucocorticoid sparing agents for symptomatic extrapulmonary or pulmonary sarcoidosis with infiltrates on chest radiographs and abnormal PFT. Relapse rates have ranged from 13% to 75% depending on the population studied.

Association of lung function by spirometry with serum ferritin in transfusion-dependent thalassemia patients in a tertiary care centre

Background: β-thalassemia major is an inherited, transfusion-dependent chronic anemia which is caused by decreased production of β-globin chains required for formation of hemoglobin. Regular blood transfusion is most important factors that help in improving the survival of patients with TM; however, it leads to iron deposition in many organs such as lung. Most studied concerned about effect of iron on lung functions, revealed abnormalities, but limited data were observed. The aim of our study was to determine pulmonary function abnormalities in children with thalassemia major and assess the relation between these abnormalities and iron overload. Methods: This study had included 51 children in each group, aged between 6 and 18 years (28 males and 23 females) with TM. All included children were subjected to full clinical examination and laboratory investigations including complete blood counts and serum ferritin. Pulmonary function tests (PFTs) were assessed in all included children using spirometry. Results: 31.4% patients had restrictive lung functions. Spirometer parameters i.e., FEF25-75% (p value≤0.0001) and PEFR (%pred) (p value≤0.0001) had significant difference in both groups. FEF25-75% and PEFR had no effect of serum ferritin. Conclusions: The lung may be considered a site for organ damage, and alteration of pulmonary function may be expected in transfusion-dependent patients in spite of no pulmonary symptoms or normal chest X-ray.

E36 Juvenile scleroderma in rheumatology unit Tripoli children hospital

Abstract Introduction Juvenile scleroderma is a rare chronic systemic connective tissue disease. It is classified into two main forms: localized scleroderma and systemic sclerosis. Since it is rare in children, many aspects of the disease remain undiscussed. Aim To study the clinical and demographic characteristics of patients diagnosed with various types of scleroderma who presented at the pediatric rheumatology unit in Tripoli Children hospital from the year 2000–2022 Materials and methods This is a retrospective case series conducted by reviewing the files of patients with scleroderma diagnosed and followed up in pediatric rheumatology unit from year 2000–2022 and collecting the data regarding patients’ demographics, time of diagnosis, clinical features, and laboratory results. Results Seven patients who met preliminary classification criteria for JSS were included. 2 males and 5 females. 1 patient with systemic sclerosis, 1 patient with linear morphea, 2 patients with generalized morphea, 1 patient with Parry Romberg syndrome, 1 patient with CREST syndrome, and 1 patient with deep morphea. The mean age at diagnosis was 8.2 ± 4.5 years. The first symptom attributable to JSS was hyperpigmented skin patches in 5 cases, no one presented with Raynaud's phenomenon. Respiratory involvement occurred in one patient, and it manifested as dyspnea as well as abnormal imaging and/or pulmonary function tests showed mild restrictive lung disease. Dysphagia was the commonest gastrointestinal symptom (4 patients, 57%). The most frequent musculoskeletal symptom was arthritis found in in 4 (57.1%) patients. Uveitis in 1 (14.3%), ANA was positive in 4 (57%) children: RF positive in 2 (28.6%) patients, ENA negative in the 7 (100%) patients. Skin biopsy was done in 2 patients and was consistent with scleroderma. Regarding treatment, six patients received MTX, one patient received prednisolone and one patient received MMF. Conclusion This report is the first describing Libyan patients with scleroderma. As reported in the literature, localized forms are more common than systemic sclerosis. Also a rare form of scleroderma (Parry Romberg syndrome) was present. Data about the disease mainly based on studies on adults and we need more studies on pediatric patients. Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.

Assessment of respiratory health status of workers in flour mills of Assiut

BackgroundWorking environment should not present a risk of injury or disease but many thousands of workers worldwide remain exposed to hazardous substances particularly in developing countries. Flour dust is one of those hazardous substances to which the flour mills workers are exposed to, inhalation of flour dust may cause diverse lung diseases with different severity of symptoms ranging from simple irritation to allergic reaction and chronic respiratory disorders, including asthma. Therefore, the present study was done to detect the prevalence of respiratory problems among flour mills workers.MethodsThe study was carried out in the flour mills of Assiut, it was a cross-sectional study among 203 workers. The study instruments were a semi-structured questionnaire about respiratory symptoms, chest examination and pulmonary function tests (PFT).ResultsThe age of the study workers ranged from 19 to 60 years old, 94.1% were males, 50.7% were from urban regions. Pulmonary function abnormalities were found in 36.4% of them and 20.7% were diagnosed with bronchial asthma. Significant relationships were detected between abnormal pulmonary function tests and workers ≥ 40 years old, low educational level and those who worked in the flour mills for ≥ 10 years.ConclusionsFlour dust cause respiratory symptoms and impair the pulmonary function of the flour mills workers and they may develop bronchial asthma which affect their work efficiency.RecommendationsMedical care must be provided regularly to the flour mills workers and safety measures are mandatory.Trial registrationClinicalTrials.gov. NCT03678519. Registered August 10, 2022.

The relationship between preserved ratio impaired spirometry and mortality in the myocardial infarction survivors: a population-based cohort study

IntroductionPreserved ratio impaired spirometry (PRISm) is a subtype of pulmonary function abnormality which is characterized by a proportional reduction in non-obstructive expiratory lung volume. Currently, no studies have shown a relationship between PRISm and mortality in myocardial infarction (MI) survivors.MethodsWe used cohort data from U.S. adults who attended the National Health and Nutrition Examination Survey (NHANES) from 2007 to 2012. According to the ratio of forced expiratory volume in the first second (FEV1) to forced vital capacity (FVC), we divided lung function into normal spirometry (FEV1/ FVC) ≥ 70%, FEV1 ≥ 80%), PRISm (FEV1/FVC ≥ 70%, FEV1 < 80%) and obstructive spirometry (FEV1/FVC < 70%). Cox regression was used to estimate the correlation between lung functions and mortality among MI patients. Kaplan-Meier survival curves compared the prognosis of MI with three different lung functions. We further verify the stability of the results by sensitivity analysis.Results411 subjects were included in our research. The mean follow-up time for the study was 105 months. Compared with normal spirometry, PRISm was significantly correlated with a greater relative risk for all-cause mortality (adjust HR 3.41, 95% confidence interval [95%CI]: 1.76–6.60, P < 0.001) and cardiovascular mortality (adjust HR 13.9, 95%CI: 2.60–74.6, P = 0.002). PRISm remains more correlated with all-cause mortality (adjust HR 2.73, 95%CI: 1.28–5.83, P = 0.009) relative to obstructive spirometry. The results are basically stable after sensitivity analysis. Kaplan-Meier survival curves showed that patients with PRISm tended to have the lowest survival during the follow-up period.ConclusionPRISm is an independent risk factor for all-cause and cardiovascular mortality in MI survivors. The presence of PRISm was associated with a significantly higher risk of all-cause mortality compared with obstructive spirometry.

Understanding Early COPD.

Whereas COPD is currently defined as the presence of spirometric obstruction, the pathologic changes in individuals at risk including chronic mucus hypersecretion and emphysema have been recognized for centuries. At the same time, we have struggled to define criteria that would help us identify patients at an early stage, prior to the development of pulmonary function abnormality. The concept of GOLD 0 was introduced in the hopes that symptoms would help to identify those at greatest risk for progression. While symptoms are a risk factor, in particular chronic bronchitis, the term was abandoned as the majority of individuals at risk who progress to COPD do not have symptoms. Since then, the related terms pre-COPD and early COPD have been introduced. They are similar in that the term pre-COPD identifies individuals based on symptoms, physiologic, or radiographic abnormality that do not meet criteria for COPD but are clearly at risk. The term early COPD extends that concept further, focusing on individuals who have early physiologic or radiographic abnormality but at the same time are young, thereby excluding those with late mild disease who may be less likely to progress. Whereas individuals with early COPD are now being recruited for observational studies, we are still challenged with determining the best way to identify patients at risk who should undergo additional testing as well as developing specific therapies for patients with early-stage disease.