Mucopolysaccharidosis type I (MPS I), is an autosomal recessive disorder caused by a deficiency in the enzyme α-L-iduronidase (IDUA), leading to the accumulation of glycosaminoglycans (GAGs) in tissues. Early diagnosis and treatment [i.e., bone marrow transplantation and/or enzyme replacement therapy (ERT) with laronidase] are essential to prevent irreversible damage. The long-term effectiveness of exclusive ERT has been primarily described in attenuated phenotypes, while only a few cases have been reported in severe phenotypes. This study is a retrospective analysis summarising the collective experience of disease progression in 48 patients with severe and attenuated MPS I who were treated exclusively with laronidase over a median of 10years at the Lyon Reference Centre for Hereditary Metabolic Diseases in France. Patients were categorised by genotype and further stratified by age at treatment initiation. The study assessed the evolution of urinary excretion of GAGs, pulmonary function, cardiac involvement and evolution, height, cognitive impairment, functional status, orthopaedic and ear-nose-throat (ENT) procedures, sleep apnoea, and carpal tunnel syndrome. Descriptive statistical analysis methods were used. ERT reduced the GAGus levels by 88% in severe MPS I and by 71% in attenuated MPS I, of which 47% and 65% patients, respectively achieved normal age-related GAG levels at the last follow-up. ERT provided stable or consistent improvement in forced vital capacity, slowed progression of adverse cardiac course and improved auditory transmission in majority of the severe and attenuated patients. At the last follow-up, 84% attenuated patients had normal cognitive development. In alive Hurler patients, cognitive development was very heterogenous; however, 73% patients had a developmental quotient (DQ) ≥ 70. Laronidase was effective in improving statural growth of attenuated patients treated before 9years of age. Early ERT and regular multidisciplinary management are effective in slowing disease progression in severe and attenuated patients with MPS I and helping to maintain autonomy in patients with attenuated MPS I, ensuring a better quality of life.

Population aging is accelerating worldwide, with 16% projected to be aged ≥ 65years by 2050. A practical index reflecting overall aging status is needed for population-based research, as existing indices often require specialised or cognitive assessments. We developed a Physiological Healthy Aging Index (PHAI) using accessible biomarkers and evaluated its association with mortality in Korean adults. A total of 6398 participants aged ≥ 40years from the Korean Genome and Epidemiology Study (KoGES) Ansan-Ansung cohort followed up for an average duration of 16.5years (2001-2022). The PHAI, based on systolic blood pressure, fasting blood glucose, serum creatinine, forced vital capacity, and C-reactive protein levels, was scored 0-10, with higher scores indicating healthier aging. Mortality risks across quartiles were estimated using Cox proportional hazard models. Long-term changes were classified as accelerated (decreased scores), stable (unchanged scores), or resilient (increased scores). During 105,597 person-years, 934 deaths occurred (778 age-related, 353 cancer-related, and 184 cardiovascular-related). Higher PHAI quartiles were linked with significantly lower mortality risk versus Q1. Fully adjusted hazard ratios (95% CIs) for all-cause mortality were 0.82 (0.69-0.98) for Q2, 0.50 (0.42-0.60) for Q3, and 0.51 (0.41-0.63) for Q4 (P for trend < 0.001). Similar associations were observed for age-related mortality (HR 0.51, 95% CI 0.40-0.64 for Q4 vs. Q1), cancer (HR 0.66, 95% CI 0.48-0.92), and cardiovascular mortality (HR 0.24, 95% CI 0.13-0.44). Resilient agers had much lower all-cause mortality than accelerated agers (HR 0.21, 95% CI 0.16-0.28), with stable agers also at reduced risk (HR 0.65, 95% CI 0.54-0.77). Higher scores also correlated with a lower cognitive impairment risk. The PHAI is a simple, robust predictor of mortality outcomes, supporting its use as a practical tool for assessing physiological aging in public health and clinical settings.

Changes in lung function and fractional exhaled nitric oxide across wildfire seasons in the wildland firefighter exposure and health effect (WFFEHE) study.

Impact of mepolizumab on airway remodeling and inflammation in severe eosinophilic asthma.

Introduction Growing evidence shows that voluntary breathing maneuvers modulate cortical oscillations, yet the precise frequency-specific signatures of functional connectivity (FC) remain unclear. Methods This study investigated the impact of different respiratory conditions on brain FC using EEG recordings. Three respiratory conditions were collected and analyzed: self-paced breathing (SB), breath-holding (BH), and computer-paced breathing (PB). The power spectral density (PSD), phase-locking value (PLV), and brain network characteristics were analyzed for these different conditions. Results The results all showed significant differences. The PSD analysis revealed increased low-frequency ( δ and θ ) activity during SB and higher high-frequency ( α and β ) activity during BH conditions. The PLV analysis demonstrated significant differences in FC between conditions, indicating specific modulation of brain networks by respiratory state. The brain network properties analysis uncovered frequency-specific changes in clustering coefficient (CC), global efficiency (GE), local efficiency (LE), and degree centrality (DC), reflecting alterations in brain network organization. The three-class classifier showed superior performance in the α band, suggesting its potential as a biomarker for distinguishing respiratory conditions. Correlation analysis with forced vital capacity (FVC) revealed significant associations between brain connectivity and FVC metrics. Discussion These findings highlight the complex interplay between respiratory conditions and brain FC. These findings suggest that controlled and uncontrolled breathing patterns can influence brain network organization, a mechanistic observation that may inform future respiratory-based interventions aimed at enhancing cognitive function, although behavioural or affective outcomes were not assessed here.

Active video games (AVG) offer an alternative or complementary approach to traditional rehabilitation, yet their effects in patients with interstitial lung disease (ILD) remain underexplored. This study aimed to evaluate the effectiveness of AVG in individuals with ILD. Forty-five patients with ILD were randomised into three equal groups: AVG, traditional aerobic exercise (TAE), and control (CG). Both the AVG and TAE groups participated in 30 min of moderate-intensity cycling twice weekly for 8 weeks. The AVG group additionally completed 30 min of exergaming after cycling. All groups were instructed to walk 30 min twice weekly. Outcomes were assessed at week 8. Primary outcomes included exercise capacity measured by the 6-min walk test (6MWT), incremental shuttle walk test (ISWT), and endurance shuttle walk test (ESWT). Secondary outcomes included spirometry, respiratory and quadriceps strength, fatigue, dyspnea, physical activity (PA) and psychological state. Satisfaction, attendance, and enjoyment were also recorded. After 8 weeks, the AVG group demonstrated greater improvements in 6MWT distance (p = 0.035), maximal inspiratory pressure (p = 0.002), St George's Respiratory Questionnaire (SGRQ) symptoms (p = 0.005) and activity (p = 0.008) scores, International Physical Activity Questionnaire (IPAQ) (p = 0.001), and the London Chest Activities of Daily Living Scale (LC-ADL) (p = 0.001) compared to TAE and CG. No significant differences were observed in ISWT, forced vital capacity (FVC), maximal expiratory pressure, quadriceps strength, dyspnea, or Hospital Anxiety and Depression Scale (HADS) scores (p > 0.05). AVG combined with traditional aerobic exercise appears to be a safe and effective intervention in ILD, improving submaximal capacity, symptom control, physical activity, satisfaction, and adherence. NCT06087692 at https://clinicaltrials.gov/ct2/show/NCT06087692.

Identifying patients at risk for acute exacerbations of COPD (AECOPDs) is crucial to improve outcomes. We aimed to evaluate the ability of three health status instruments to predict AECOPDs in subjects with and without previous AECOPDs. A prospective cohort study of COPD patients from primary and outpatient care in three Swedish regions. AECOPDs were retrieved from medical records. The modified Medical Research Council Dyspnoea scale (mMRC), the COPD Assessment Test (CAT), and the Clinical COPD Questionnaire (CCQ) were evaluated. Thresholds for AECOPD prediction were estimated using receiver operator characteristic (ROC) curves. Predictive values were assessed using crude and multivariable Cox regression models. We included 572 patients (59% women, age 69 ± 8 years, FEV1 57 ± 18% of predicted) in 2014-2016. All three instruments independently predicted future AECOPDs within three years (adjusted hazard ratio [aHR] 1.5-1.8) using thresholds mMRC ≥ 2, CAT ≥13, and CCQ ≥ 1.6. Patients without prior-year AECOPDs but high scores on all instruments had a similar AECOPD risk as those with prior AECOPDs but scores below threshold (aHR 2.4-2.5). Among patients with ≥1 AECOPD the year before inclusion and at least one of the three health status instruments above threshold, the aHR for future AECOPD during the study period ranged from 4.6 to 5.7. mMRC, CAT, and CCQ were independently associated with AECOPDs over the following three-year period. The health status instruments provided additional predictive value for future AECOPDS in patients both with and without previous AECOPDs. aHR: Adjusted Hazard Ratio; AECOPD: Acute Exacerbations of Chronic Obstructive Pulmonary Disease; AUC: Area Under Curve; BMI: Body Mass Index; CAT: COPD Assessment Test; CCQ: Clinical COPD Questionnaire; COPD: Chronic Obstructive Pulmonary Disease; DAG: Directed Acyclic Graphs; FEV1: Forced Expiratory Volume in 1 Second; FVC: Forced Vital Capacity; GOLD: Global Initiative for Obstructive Lung Disease; HR: Hazard Ratio; IHD: Ischaemic Heart Disease; ICS: Inhaled Corticosteroids; IQR: Interquartile Range; mMRC: Modified Medical Research Council Dyspnoea Scale; ROC: Receiver Operator Characteristic; SD: Standard Deviation; TIE: Tools for Identifying Exacerbations.

There is a need for more effective treatments for idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF). Nerandomilast (BI 1015550), an oral preferential inhibitor of phosphodiesterase 4B, is being evaluated in two randomized Phase III trials: FIBRONEER™-IPF (NCT05321069) and FIBRONEER™-ILD (NCT05321082). FIBRONEER™-ON is an open-label extension (OLE) of these studies that will evaluate the long-term safety and efficacy of nerandomilast. Here, we describe the study design of the OLE. This prospective 98-week OLE will follow the Phase III parent trials, which are currently underway with 1177 patients enrolled in FIBRONEER™-IPF and 1178 patients enrolled in FIBRONEER™-ILD. Approximately 1700 patients from 44 countries are expected to complete the parent trials and will be eligible for continuing into the OLE; this estimate assumes that there will be a discontinuation rate of ~25% over the duration of the parent trials and > 90% of eligible patients will agree to participate in the OLE. Irrespective of whether previously on active treatment or placebo, all patients in the OLE will be treated with nerandomilast at either 9mg or 18mg twice daily, depending on which dose demonstrates the most favorable benefit-risk profile in the parent trials. The primary endpoint will be the occurrence of any adverse event over the course of the OLE. This trial will also monitor long-term efficacy outcomes, including forced vital capacity change, and time to first exacerbation, disease progression, hospitalization and death. This trial will provide information on the long-term safety, tolerability and efficacy of nerandomilast in patients with IPF and PPF. FIBRONEER™-ON: ClinicalTrials.gov: NCT06238622, registered 2 February 2024. Protocol version and date: version 3.0, 29 Apr 2024. FIBRONEER™-IPF: ClinicalTrials.gov: NCT05321069, registered 11 March 2022.FIBRONEER™-ILD: ClinicalTrials.gov: NCT05321082, registered 11 March 2022.

Background: The popular anthropometric measure known as body mass index, or BMI, has been connected to several physiological changes, including modifications in respiratory mechanics. It is hypothesized that elevated BMI may influence lung volumes and airflow, potentially affecting the forced expiratory volume in one second (FEV₁) to forced vital capacity (FVC) ratio, an essential spirometry index for evaluating pulmonary function and detecting obstructive or restrictive ventilatory defects. Objective: The purpose of this study was to evaluate the relationship between pulmonary function and BMI, specifically the FEV₁/FVC ratio, and to explore how increasing BMI may impact respiratory physiology among healthy young adults. Methodology: A cross-sectional study was conducted in the Department of Physiology at a private medical college in Lahore. A total of 122 healthy second-year medical students (62 males and 60 females) aged 18–22 years, with BMIs ranging from 18.5 to 40 kg/m², were selected through convenient sampling. Spirometric assessments were performed to obtain FEV₁/FVC ratios. Ethical approval was obtained from the Institutional Review Board prior to data collection. Results: Statistical analysis revealed no significant correlation between BMI and FEV₁/FVC ratio (p = 0.078). The observed variations in pulmonary function across different BMI categories were not statistically significant. Conclusion: BMI does not significantly affect spirometric parameters. Future studies with larger, more diverse groups are recommended to confirm these results and explore potential gender or age interactions.

Case Illustration: Laparoscopy can reduce vital capacity, increase dead space, hypercapnia and acidemia due to CO2 absorption. This has the potential to cause massive subcutaneous emphysema which is rarely encountered in laparoscopic surgery. A woman, 47 years old, is a potential kidney donor for her child. The patient was classified as ASA 1, underwent general anesthesia and bilateral quadratus lumborum block. After 2.5 hours of surgery, she had hypertension and tachycardia, and ETCO2 rised up to 49 mmHg. Swelling and crepitus appear in the neck, chest and all four extremities.Case Management: Blood gas analysis was carried out. Changes in ventilator settings was done to increase ventilation rate and tidal volume, the soda lime was replaced, and we communicated with the operator so the surgery could be postponed temporarily. After ETCO2 returns to normal, the surgery continues with lower CO2 gas insufflation. Patient’s condition was stable during the rest of procedure.Successful of Management: The patient in this case had no comorbid conditions or risk factors for subcutaneous emphysema. However, massive subcutaneous emphysema still occurred. The conservative anesthetic management carried out is in accordance with the literature and has been proven to improve patient’s outcome.

Although overt thyroid dysfunction has been associated with changes in pulmonary function, the effects of thyroid hormone levels and thyroid autoimmunity on lung function in euthyroid individuals remain unclear. We investigated the associations between subtle changes in thyroid hormones and thyroid peroxidase antibodies (TPOAb) and pulmonary function in a nationally representative cohort of Korean adults. We analyzed data from 2,626 euthyroid participants aged ≥ 40 years from the Korea National Health and Nutrition Examination Survey (2013-2015). Pulmonary function was assessed using spirometry-derived forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), and FEV1/FVC ratio. Multivariable linear regression analyses were conducted after adjusting for age, sex, body mass index (BMI), smoking status, alcohol intake, and physical activity. In the unadjusted models, higher free thyroxine levels were positively associated with FVC and FEV1, while higher TSH levels were inversely associated. In contrast, elevated TPOAb levels were independently associated with reduced FEV1 (β = -0.330, P = 0.021). These associations were more pronounced among adults aged ≥60 years and individuals with a BMI ≤ 23 kg/m². Thyroid autoimmunity, as reflected by elevated TPOAb levels, was associated with a decline in pulmonary function among euthyroid individuals, independent of thyroid hormone levels. Our results support the clinical utility of TPOAb status as an early marker for detecting subclinical pulmonary vulnerability, particularly in older adults and those with a lower BMI.

PurposeAlthough overt thyroid dysfunction has been associated with changes in pulmonary function, the effects of thyroid hormone levels and thyroid autoimmunity on lung function in euthyroid individuals remain unclear. We investigated the associations between subtle changes in thyroid hormones and thyroid peroxidase antibodies (TPOAb) and pulmonary function in a nationally representative cohort of Korean adults.MethodsWe analyzed data from 2,626 euthyroid participants aged ≥ 40 years from the Korea National Health and Nutrition Examination Survey (2013–2015). Pulmonary function was assessed using spirometry-derived forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), and FEV1/FVC ratio. Multivariable linear regression analyses were conducted after adjusting for age, sex, body mass index (BMI), smoking status, alcohol intake, and physical activity.ResultsIn the unadjusted models, higher free thyroxine levels were positively associated with FVC and FEV1, while higher TSH levels were inversely associated. In contrast, elevated TPOAb levels were independently associated with reduced FEV1 (β = −0.330, P = 0.021). These associations were more pronounced among adults aged ≥60 years and individuals with a BMI ≤ 23 kg/m².ConclusionThyroid autoimmunity, as reflected by elevated TPOAb levels, was associated with a decline in pulmonary function among euthyroid individuals, independent of thyroid hormone levels. Our results support the clinical utility of TPOAb status as an early marker for detecting subclinical pulmonary vulnerability, particularly in older adults and those with a lower BMI.

Introduction: Prolonged usage of masks has been inevitable for healthcare workers. This study examines the impact of prolonged mask use on healthcare workers, focusing on its effects on lung function, endurance, and cognitive abilities. Method: 106 healthcare workers aged 25-45 years were included in the study conducted at selected South Bangalore, India hospitals. Spirometry, a 6-minute walk test and cognition failure questionnaire were used to assess their lung function, endurance and cognitive abilities respectively. Result: More number of female participants (64.15%) volunteered for the study. A low percentage of participants (49.06) used surgical masks and the majority did not engage in regular exercise. Around 50% of the participants had contracted COVID-19 at least once during the past 3 years. Furthermore, we found that regardless of the type of mask used, there were no significant differences in Forced Expiratory Volume in 1 second (FEV1), Forced Vital Capacity (FVC) and Peak Expiratory Flow (PEF) parameters, while FEV1/FVC values reached a statistical significance level. Further, the healthcare workers had no significant changes in the 6-minute walk distance and there was a slight reduction in cognitive function among them. Conclusion: The healthcare workers were invited for an observational cross-sectional study, aimed to assess the effect of prolonged use of different types of masks owing to their occupational requirements. We found that there was a clinically significant reduction in the pulmonary function parameters of the participants in comparison with the age, gender and Body Mass Index (BMI) matched normative values though it did not reach statistical significance. Also, we found that there was no difference in the age and sex-matched 6-minute walk distance scores of our study participants. The use of face masks demonstrated to have an implication on the cognitive functions of Health Care Workers (HCWs), though not severe. Major Findings: 1. No statistically significant differences in the Pulmonary Function Tests (PFT) values were documented except for the FEV1/FVC ratio between surgical and N95 mask groups. 2. The 6-Minute Walk Distance (MWD) among both, N95 and surgical mask users as well as the male and female individuals were found to be well within the prescribed ranges for that age and sex. 3. The use of face masks is known to have an implication on the cognitive function of HCWs, though not severe.

Pain after breast plastic surgery affects quality of life. Physical therapy offers effective interventions for this condition, such as transcutaneous electrical nerve stimulation (TENS). Although this resource has been used for more than 20years, no studies have been published that support its use following this type of surgery. Therefore, the aim of the proposed study is to evaluate the effect of TENS on pain intensity in patients undergoing mastopexy with implants, given the existing evidence on the success of TENS for other conditions. A two-arm, randomized, sham-controlled trial will be conducted with blinded assessors. The study will be carried out at the Exercise Physiology and Metabolism Lab of Finis Terrae University. Eligible participants will be women undergoing mastopexy with implants invited by a board-certified plastic surgeon. The participants will be randomly assigned to one of two study groups: Group 1 (surgery + TENS) and Group 2 (surgery + sham TENS). TENS will be administered only 1 h after surgery and will remain for 1 h. Four assessments will be performed: before treatment (T0), immediately after treatment (T1), 1 h (T2), and 4 h after TENS (T3). The primary outcome will be pain intensity at rest, pain intensity during movement (standardized movements of both arms: anterior flexion, abduction, and external rotation), and during respiratory function tests. Secondary outcome measures will be maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), and vital capacity (VC). In this study, the effects of TENS on patients with pain following mastopexy with implants will be compared to the effects of a sham TENS intervention. This RCT will offer novel evidence on the potential benefits of TENS in terms of pain intensity at rest as well as during movements and respiratory function tests. ClinicalTrials.gov NCT06202794. Registered on January 11, 2024.

Thoracotomy often causes respiratory muscle injury, reduced chest wall expansion, decreased lung volume, and limited shoulder range of motion (ROM). Respiratory muscle-stretching exercises (RMSE) have been proposed to enhance chest expansion and facilitate recovery. This aimed to compare the effects of RMSE combined with conventional physical therapy versus conventional therapy alone in patients undergoing elective thoracotomy. A single-center randomized controlled trial was conducted at Songklanagarind Hospital, Thailand, between August 2013 and December 2019. Twenty-eight patients scheduled for elective thoracotomy were recruited, and 23 (mean age 47.1 ± 15.2 years; 18 males, 5 females) completed the trials. Participants were randomized to an intervention group (RMSE plus conventional therapy, n = 12) or a control group (conventional therapy only, n = 11). RMSE consisted of four stretching exercises performed twice daily for eight days. Primary outcomes were middle and lower chest expansion (MCE, LCE), slow vital capacity (SVC), and shoulder flexion and abduction ROM, assessed preoperatively, on postoperative day 2, and day 8. Both groups demonstrated significant within-group improvement between days 2 and 8. In the intervention group, MCE increased by 1.46cm (95%CI: -2.5 to -0.87), LCE by 1.75cm (95%CI: -2.42 to -1.08), SVC by 438 mL (95%CI: -761 to -115), shoulder flexion by 48.3°, and abduction by 38.8° (95%CI: -57.2 to -20.4). Similar improvements were observed in the control group: MCE 1.18cm (95%CI: -1.76 to -0.61), LCE 1.27cm (95%CI: -1.91 to -0.65), SVC 347 mL (95%CI: -690 to -4), shoulder flexion 35.4° (95%CI: -50.6 to -20.2), and abduction 35.7° (95%CI: -54.0 to -17.4). Between-group comparisons showed no significant differences for any outcome (all Group x Time, p-value > 0.05). Respiratory muscle-stretching exercise combined with conventional therapy facilitated significant recovery in chest wall mobility, lung volume, and shoulder motion after thoracotomy. However, these benefits were not significantly greater than those achieved with conventional therapy alone. Clinically, Respiratory muscle-stretching exercise is feasible, safe, and may be considered as an adjunct to standard care. TCTR20140224004.

Diabetes, a significant global health concern, affects one in 10 adults, with many unaware. Despite its prevalence, evidence on its impact on pulmonary function is limited. Given the occupational exposure to silica dust, mine workers may face heightened pulmonary risks with diabetes. Our objective was to estimate differences in pulmonary functions between diabetic and non-diabetic mine workers and determine predictors of pulmonary dysfunction. We conducted a cross-sectional study of 215 mine workers from Gujarat's lignite mines in December 2021. Data on demographics, occupation, comorbidities, anthropometry, blood pressure, and random blood glucose (RBG) were collected. Diabetes was defined as self-reported history or RBG ≥200 mg/dL with symptoms. Spirometry measured forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC). Group differences were analyzed using independent t-tests, and linear regression identified predictors of pulmonary function. Diabetic workers (18%) exhibited lower FEV1 (2.80 ± 0.65 vs. 3.11 ± 0.77 L, p = .022) and FVC (3.21 ± 0.69 vs. 3.55 ± 0.84 L, p = .017). On linear regression, male gender and longer work experience significantly predicted FEV1 and FVC, while adjustments for confounding variables diminished diabetes's impact. Lower FEV1 and FVC among diabetic mine workers suggest a potential role of diabetes in pulmonary impairment. The diminished effect after adjustment underscores the need for mining cohorts to confirm these findings. Mine workers should undergo annual diabetes screening and respiratory evaluations beyond routine periodic medical examinations. Workplace programs targeting non-communicable diseases should be implemented to support long-term worker health.

Sleep apnea in interstitial lung disease: A systematic review and meta-analysis of prevalence, severity, and risk factors.

Effectiveness of Mindfulness-Based Interventions on Breathlessness in Lung Cancer: A Meta-Analysis.

Association of serum amide herbicide levels and genetic susceptibility with lung function: A prospective cohort study in general Chinese adults.

Prenatal exposure to a mixture of per- and polyfluoroalkyl substances (PFAS) and lung function and immune-related outcomes among males in childhood and young adulthood.