Respiration最新文献

German recommendations for the diagnosis of hypersensitivity pneumonitis (HP), also known as extrinsic allergic alveolitis (EAA), were last published in 2007 [1]. The current S2k Guideline for the Diagnosis and Treatment of Hypersensitivity Pneumonitis (HP) replaces these diagnostic recommendations. They were supplemented by the aspect of chronic, and in particular of the chronic fibrotic phenotype of HP, and also, as first HP guideline, include treatment recommendations. Based on current scientific evidence and on expert opinion 12 consensus recommendations were developed. They include important statements summarizing the diagnostic process, the treatment indication and therapeutic strategies for patients with HP. Particular emphasis was placed on the different clinical courses (acute and chronic) and their characteristics (inflammatory and/or fibrotic pattern), which present differential diagnostic challenges and ultimately result in different treatment approaches. In addition to general information (diagnosis, classification, clinical disease course, epidemiology, pathogenesis, risk factors, prognosis and special aspects associated with occupational disease), the guideline will present the various clinical disease entities of HP, some in detail, others in tabular form. A major focus is on the various diagnostic steps and the different treatment approaches. The background information serves to provide a deeper understanding and inform the implementation of the recommendations. In particular, other current international guidelines for the diagnosis of HP as well as German guidelines for the diagnosis of Interstitial lung diseases (ILDs) (Leitlinien zur Diagnostik interstitieller Lungenerkrankungen) in general and for the pharmacotherapy of fibrotic ILDs were integrated and considered in this guideline [2-6].

Introduction: Elexacaftor/tezacaftor/ivacaftor (ETI) has shown significant improvements in pulmonary and nutritional status in persons with cystic fibrosis (pwCF). Less is known about the extrapulmonary impact of ETI and effects on airway microbiology, lung clearance index (LCI) and fraction of exhaled nitric oxide (FeNO).

Methods: A multicentre prospective observational trial, including 79 pwCF ≥ 18 years eligible for ETI. Assessments were done at the initiation of, and 3 and 6 months into treatment with ETI. Outcomes included forced expiratory volume in 1 second (FEV1), LCI, FeNO, sputum or cough swab culture, body mass index (BMI), cystic fibrosis questionnaire-revised respiratory domain (CFQ-R RD), sinonasal outcome test-22 (SNOT-22), general anxiety disorder-7 (GAD-7), patient health questionnaire-9 (PHQ-9), fecal elastase-1 (FE-1), adherence to baseline therapies, exacerbation rate and adverse events.

Results: Our cohort included 79 pwCF (31±11(SD) years) with a baseline ppFEV1 of 68±23. Forty-two (53%) pwCF were previously treated with a CFTR modulator. In the entire study group, there were significant improvements from baseline in ppFEV1, LCI, FeNO, annualized exacerbation rate, BMI, CFQ-R RD and SNOT-22 (p<0.05 for all). Airway culture positivity for methicillin-susceptible Staphylococcus aureus and Pseudomonas aeruginosa also decreased during the study period. There was no significant change in FE-1, GAD-7 or PHQ-9. Adherence to dornase alfa and hypertonic saline decreased.

Conclusion: ETI treatment led to significant improvements in respiratory and nutritional status, alongside a decrease in adherence to chronic supportive therapies. We did not observe any significant changes in exocrine pancreas function or in questionnaire scores for depression and anxiety.

Background: Bronchiolitis is a heterogeneous group of disorders, each with its own clinical and radiographic presentation, treatment options and prognosis. Until now classifications were based on pathology and radiology.

Summary: Bronchioles are smaller than 2mm in diameter and do not contain cartilage and mucous glands in their walls. Bronchiolitis is the term used to designate a disorder of the bronchioles and can have multiple etiologies. A specific diagnosis requires an extensive anamnesis, physical examination, pulmonary function testing, radiographic studies and pathological investigation. We made a classification of bronchiolitis based on etiology, in order to give a diagnostic and therapeutic guidance. Further investigation is needed to optimize treatment for specific types of bronchiolitis.

Key messages: In this paper, a classification of bronchiolitis based on etiology is made. The paper provides in the diagnostic and therapeutic approach, although further investigation is needed to optimize treatment for specific types of bronchiolitis.

Introduction: Malignant Pleural Effusion (MPE) is common in advanced malignancy. Data are needed on health care utilization and costs.

Methods: This population-based retrospective cohort study included patients aged ≥18 years with an MPE diagnosed between January 1, 2004 and December 31, 2018. We described the incidence and prevalence of MPE from 2004 to 2018, and trends in hospital admissions, emergency department (ED) visits, and pleural-based procedures. In patients diagnosed with MPE from 2006 to 2016 we estimated 2-year health care costs (2018 US dollars) from the public payer perspective.

Results: The annual incidence and prevalence of MPE increased from 5,090 and 14,579 in 2004 to 6,977 and 23,204 in 2018, respectively. Annual mortality among prevalent patients decreased from 32% to 28%. In 2004, 57% of patients were hospitalized, decreasing to 50% in 2018. ED visits increased (59% to 61%). From 2004 to 2018, the number of patients who had insertions of thoracostomy tube (2,211 to 3,664) and tunnelled pleural catheter (383 to 1,164) increased. The number of patients receiving pleurodesis declined (565 to 284). No change occurred in the number of patients receiving video-assisted thoracoscopy (range 140 - 208/year). Between 2004 and 2016, 76,190 patients were diagnosed with MPE (mean age 71.2; 52% female). The median (interquartile range) health care cost during follow-up was $45,641 ($23,237-$86,700), with $19,753 ($9,590-$38,746) for inpatient hospitalization.

Conclusion: Hospitalization and temporizing MPE procedures are associated with high costs. This population-based study may guide clinicians and policy makers to improve MPE management and reduce health care costs.

Introduction: Advances in neonatal and pediatric intensive care have improved patient survival rates, emphasizing the need for respiratory support in cases of chronic respiratory failure, resulting in the establishment of the Turkish National Pediatric Patients Receiving Home Mechanical Ventilation (HMV) Support Registration System in 2023. This study aims to present the initial findings of the registry.

Methods: In this cross-sectional study, epidemiological and demographic data was obtained from the registry and compared between invasive ventilation (IV) and non-invasive ventilation (NIV) groups.

Results: The study included 952 patients registered from 23 centers with a median (IQR) age of 6.0 (3.0-12.0) years. IV support was observed in 51.1% of patients (n= 486). There was no difference in sex between IV and NIV groups, but a significant difference in age, nutrition, mobility status and education were observed between the subgroups. The largest patient group had neuromuscular disease, with spinal muscular atrophy being the most common primary diagnosis (15.7%). Most of the caregivers (78.7% in the NIV group and 93.8% in the IV group) received formal training before the initiation of HMV.

Conclusion: Turkey's first registry for pediatric HMV patients is introduced. This system helps understand and solve mobility and education issues for these patients in our country. Collaboration with relevant ministries is crucial. In addition, although most of the caregivers have received training before discharge, efforts are still needed to ensure that this rate is 100%. The long-term results will be presented in the future.

Introduction Bronchoscopic spray cryotherapy (SCT) is a novel treatment showing promise for chronic bronchitis (CB), characterized by excessive mucus secretion and productive cough. A large animal model for preclinical research of SCT is lacking, and its treatment's efficacy and mechanisms for CB are not well understood. Methods Eight Labradors were exposed to 200 ppm SO2 for 6 months to develop a CB model. Evaluations included pulmonary resistance, bronchoalveolar lavage fluid (BALF), CT images, and pathology. After model validation, six dogs received SCT and were observed for short-term (7 days) and long-term (30 days) outcomes. Metrics included pulmonary resistance, bronchoscopy findings, and BALF analysis for inflammatory factors, acetylcholine, and mucins. Bronchial tissue was assessed via HE staining, electron microscopy, and IHC staining. BEAS-2B cells were used to study MUC5AC expression in response to LPS and acetylcholine. Results SO2 exposure led to persistent cough, increased pulmonary resistance, goblet cell hyperplasia, and inflammation. Mucin, MUC5AC, and MUC5B levels in BALF increased over time, which validated the CB model. SCT treatment reduced mucus and pulmonary resistance, improved bronchial structure, and decreased goblet cells. SCT significantly reduced BALF mucin, MUC5AC, MUC5B, acetylcholine, IL-6, INF-γ, TNF-α, and IL-10, and bronchial MUC5AC and CHRM3. In the LPS treatment BEAS-2B cells, MUC5AC expression increased when acetylcholine pretreatment concentration increased. Conclusion The SO2 inhalation protocol effectively establishes a CB model in dogs. SCT effectively treats CB by reducing mucin levels and may lower MUC5AC expression by decreasing acetylcholine and CHRM3.

Introduction: Pulmonary exacerbations increase the requirement of aminoglycoside (AG) antibiotics in people with cystic fibrosis (pwCF). Several studies have shown that AGs have a cumulative effect on ototoxicity. We aimed to investigate the relationship between AG exposure and ototoxicity by using 3 different methods in patients with CF.

Materials/methods: The multicenter study included 121 pwCF aged between 5 and 18 years with a history of parenteral AG exposure. Standard pure-tone audiometry, extended high-frequency pure-tone audiometry (EHF-PTA), and distortion-product otoacoustic emissions (DPOAE) tests were performed. Mitochondrial mutation analysis for m1555G>A was performed in 61 patients.

Results: Median age was 12.85 years and 52.1% (n = 63) were male. 18.2% (n = 22) of the patients had received parenteral AGs more than 5 courses/lifetime. Ototoxicity was detected in at least one of the tests in 56.2% (n = 68) of the patients. Only 10.7% (n = 13) of the patients had reported a symptom indicating ototoxicity. 30.3% (n = 30) of the patients had ototoxicity in the low exposure group, while it was 45.5% (n = 10) in the high exposure group according to EHF-PTA (p > 0.05). Median number of parenteral amikacin courses was significantly higher in the ototoxic group (2 [1.25-5.75] vs. 2 [1-3]; p = 0.045). No m1555A>G mutation was detected in 61 patients who screened for mitochondrial mutation analysis.

Conclusion: As AG ototoxicity occurs primarily at high frequencies, EHF-PTA is important in early detecting ototoxicity. EHF-PTA and DPOAE detected ototoxicity in some patients with normal PTA results. All pwCF with a history of AG exposure should be evaluated for hearing loss since symptoms may only be noticed in the late period.

Introduction: Currently, there is a lack of bronchoscopic lung volume reduction options that do not depend on fissure integrity. Endobronchial coils have been extensively studied to address this need but exhibited variable patient response and have been discontinued. Lung tension device (LTD) coils represent the next-generation coil treatment. This study aimed to evaluate safety, feasibility, and efficacy of LTD-coil treatment.

Methods: Patients with advanced emphysema and hyperinflation were enrolled at two European sites. LTD-coils (Free Flow Medical, Fremont, CA, USA) were implanted in the two most destructed lobes, as determined by quantitative CT analysis, in two separate procedures. The primary endpoint was 3-month follow-up after the last treatment.

Results: Fourteen patients (50% male, median age 64 years, FEV1 23%predicted, RV 249%predicted) received LTD-coil treatment: 12 received bilateral and 2 unilateral treatment. Six serious respiratory adverse events occurred within the initial 3 months posttreatment, including one device-associated death. Treatment significantly reduced in- and expiratory volume of the treated lobes (-410 [-710, -340], p = 0.004 and -650 [-730, -190] mL, p < 0.001, respectively) and improved quality of life (SGRQ total score -4.6 [-21.0, -2.6], p < 0.001). However, at a group level, no significant improvements in pulmonary function or 6-min walk distance were observed. Responder rates ranged from 18% to 54% for the different endpoints.

Conclusion: This first-in-human study shows that the new LTD-coil procedure is feasible with a safety profile comparable to the previous coil treatment. While the treatment effectively reduced lobar volume and modestly improved quality of life up to 3 months' follow-up, at a group level it did not significantly enhance pulmonary function or exercise capacity.

Introduction: Survivors of severe COVID-19 face complex challenges and a high degree of pulmonary sequelae. Therefore, we aim to describe their ongoing health burden.

Methods: In this single-center prospective cohort study, COVID-19 ICU survivors were invited 3 and 6 months after ICU discharge. We examined pulmonary function with pulmonary function tests (PFT) and cardiopulmonary exercise testing (CPET), and we established health-related quality of life (HRQL) and health status (HS) with the EuroQol five-dimension five-level (EQ-5D-5L), the short-form health survey 12 (SF-12), and the modified British Medical Research Council dyspnea scale (mMRC) questionnaires.

Results: Out of the 53 individuals screened, 23 participated in this study. Throughout both assessment points, participants maintained PFT results within range, apart from a decline in the transfer factor of the lung for carbon monoxide (TLCO). CPET showed improved fitness but persistent ventilatory deficiencies, indicated by altered dead space ventilation (VD/VT) and elevated arterial-alveoli gradient for oxygen (AaDO2). HRQL and HS remained compromised, with both physical (PCS) and mental component summary (MCS) scores significantly lower than the standardized norm population scores. Also, there was a rise in the prevalence of issues related to mobility, pain/discomfort, and anxiety/depression, and an increase in reported dyspnea.

Conclusion: These results enhance our comprehension of the complex difficulties faced by COVID-19 ICU survivors. Six months post-discharge, CPET revealed the presence of ventilatory insufficiencies. Additionally, there was a decline in HRQL and HS, notably affected by mental health concerns and an increase in the level of dyspnea.

Introduction: Exacerbations of chronic obstructive pulmonary disease (COPD) have a significant impact on hospitalizations, morbidity, and mortality of patients. This study aimed to develop a model for predicting acute exacerbation in COPD patients (AECOPD) based on deep-learning (DL) features.

Methods: We performed a retrospective study on 219 patients with COPD who underwent inspiratory and expiratory HRCT scans. By recording the acute respiratory events of the previous year, these patients were further divided into non-AECOPD group and AECOPD group according to the presence of acute exacerbation events. Sixty-nine quantitative CT (QCT) parameters of emphysema and airway were calculated by NeuLungCARE software, and 2,000 DL features were extracted by VGG-16 method. The logistic regression method was employed to identify AECOPD patients, and 29 patients of external validation cohort were used to access the robustness of the results.

Results: The model 3-B achieved an area under the receiver operating characteristic curve (AUC) of 0.933 and 0.865 in the testing cohort and external validation cohort, respectively. Model 3-I obtained AUC of 0.895 in the testing cohort and AUC of 0.774 in the external validation cohort. Model 7-B combined clinical characteristics, QCT parameters, and DL features achieved the best performance with an AUC of 0.979 in the testing cohort and demonstrating robust predictability with an AUC of 0.932 in the external validation cohort. Likewise, model 7-I achieved an AUC of 0.938 and 0.872 in the testing cohort and external validation cohort, respectively.

Conclusions: DL features extracted from HRCT scans can effectively predict acute exacerbation phenotype in COPD patients.