Predicting exacerbations in α1-antitrypsin deficiency using clinical and pulmonary function tests: Portuguese EARCO registry.

INTRODUCTION: Exacerbations are common in individuals with alpha-1-antitrypsin deficiency (AATD) related lung disease. This study intended to identify independent predictive factors for exacerbations in AATD using the Portuguese European Alpha-1 Research Collaboration (EARCO) registry. METHODS: This study includes patients from the Portuguese EARCO registry, a prospective multicentre cohort (NCT04180319). From October 2020 to April 2023 this registry enrolled 137 patients, 14 of whom were excluded for analysis for either missing 12 months of follow-up or baseline pulmonary function. RESULTS: Among the 123 AATD patients, 27 (22.0%) had at least one exacerbation in the last 12 months of follow-up. Patients with Pi*ZZ phenotype were three times more likely than the rest of the population to experience any exacerbation (32.7% vs 14.1%, p=0.014; OR 3.0). BODE index was significantly higher in exacerbators than in non-exacerbators (3.9±2.4 vs 1.3±1.2; p<0.001), including on multivariate analysis (p=0.002). Similar results were found for BODEx (multivariate p<0.001). DLCO was the only functional parameter independently associated with exacerbations (p=0.024). CONCLUSIONS: DLCO, BODE and BODEx were independent predictors of exacerbations at 12 months in AATD patients. Understanding these risk factors can aid decision-making on AATD-related lung disease management and improve patient outcomes.

Sleep and Breathing Conference highlights 2023: a summary by ERS Assembly 4.

This paper presents some of the highlights of the Sleep and Breathing Conference 2023 https://bit.ly/46MxJml.

ERS International Congress 2022: highlights from the Thoracic Oncology Assembly.

Thoracic malignancies are associated with a substantial public health burden. Lung cancer is the leading cause of cancer-related mortality worldwide, with significant impact on patients' quality of life. Following 2 years of virtual European Respiratory Society (ERS) Congresses due to the COVID-19 pandemic, the 2022 hybrid ERS Congress in Barcelona, Spain allowed peers from all over the world to meet again and present their work. Thoracic oncology experts presented best practices and latest developments in lung cancer screening, lung cancer diagnosis and management. Early lung cancer diagnosis, subsequent pros and cons of aggressive management, identification and management of systemic treatments' side-effects, and the application of artificial intelligence and biomarkers across all aspects of the thoracic oncology pathway were among the areas that triggered specific interest and will be summarised here.

Craniofacial Microsomia: New Updates in Spinal Anomalies.

INTRODUCTION: Craniofacial microsomia (CFM) involves anomalies of the first and second pharyngeal arches, mainly of the mandible, maxilla, ears, and vertebral spine. This study aimed to identify the frequency and morphology of spinal anomalies of individuals with clinical diagnoses of CFM. In addition, the correlation between spinal anomalies and craniofacial involvement was performed. MATERIALS AND METHODS: This study was a retrospective review of individuals with a clinical diagnosis of craniofacial microsomia. The inclusion criteria were a clinical diagnosis of CFM with no overlap with any other syndromes of first and second pharyngeal arches and radiograph availability in the hospital's database. Prevalence and morphology of spinal anomalies were calculated and clinical details were recorded: types of spinal anomalies and correlations according to OMENS score. RESULTS: The sample consisted of 46 individuals with a clinical diagnosis of CFM, 24 (52,2%) female and 22 (47,8%) male (1M:1F). Twenty-one (45,7%) had unilateral craniofacial involvement and 25 (54,3%), bilateral. Twenty-eight (60,9%) individuals presented spinal anomalies. Those with unaltered spinal morphology showed a slight preference toward OMENS scores under 5: 7 patients did, only one of which had spinal alterations (14,3%); 68,8% (22) in the group with scores 5 to 9 (n=32) and 71,4% (5) in the 10 to 15 group (n=7) did as well. DISCUSSION AND CONCLUSIONS: Spinal anomalies in individuals with CFM are more common than usually reported in medical literature, mainly when associated with radial anomalies and correlate with statistical significance to facial features, mainly the OMENS score.

Craniocervical Instability in Oculoauriculovertebral Spectrum.

INTRODUCTION: The oculoauriculovertebral spectrum (OAVS) are anomalies of the first and second pharyngeal arches, causing craniofacial changes, principally facial asymmetry, as well as anomalies in the spine, that can be a cause of instability of the craniocervical junction - manipulation of an unstable spine can result in morbimortality. However, few studies have related OAVS to craniocervical instability. OBJECTIVES: Correlate patients with OAVS through radiography with craniocervical instability and prediction of its occurrence. MATERIAL AND METHODS: Through a radiographic study of the cervical spine, the assessment of vertebral malformations and the presence of craniocervical instability was performed and clinical assessment using the OMENS score as a phenotypic criteria for patients with OAVS, by a specialized multidisciplinary team. Student's t test, Kolmogorov-Smirnova, and χ 2 were performed. RESULTS: Twenty-six patients with OAVS were evaluated, 7 (26.9%) had craniocervical instability, the OMENS score was 3 times higher, but without statistical significance. All patients with instability also had spinal malformations, and of those without craniocervical instability (19 patients), 57.8% had spinal malformations. Vertebral malformations had a similar incidence in those with and without instability, the most common being scoliosis. There was no statistical significance in the presence or absence of spinal malformations with the presence or absence of craniocervical instability. Extracraniofacial findings were found in all patients with instability; 71.4% of them were radial. CONCLUSIONS: Patients with higher OMENS scores had more craniocervical instability but without statistical significance. It also did not show statistical relevance between the presence of malformations and craniocervical instability.

Prognostic yield of imaging before and after recanalization treatments in ischemic stroke.

OBJECTIVES: The scores to predict outcome in ischemic stroke were validated prior to the approval of modern revascularization treatments. We evaluated the accuracy of pre and post-treatment models in a recent recanalization therapy cohort and whether radiological and ultrasound findings could improve their accuracy. MATERIAL & METHODS: We included 375 anterior circulation ischemic stroke patients treated with intravenous thrombolysis or thrombectomy during 2017 and 2018. We collected demographic, clinical, and imaging data. We built pre and post-treatment logistic regression models to predict independence (modified Rankin Scale 0-2) at 3 months. The models included the Alberta Stroke Program Early CT Score (ASPECTS), infarct volume (ABC/2 method), and the Thrombolysis in Brain Ischemia (TIBI) ultrasonographic grade of recanalization. We compared areas under the receiver operating characteristic curve (AUC). RESULTS: Our preintervention model, combining neurological deficit severity, age, and admission glycemia, was not improved by the inclusion of ASPECTS (AUC 0.80 vs 0.79, P = .28). Early neurological recovery at 24-hour significantly increased prognostic performance (AUC = 0.85, P < .01), which did not change by adding final infarct volume or the persistence of arterial occlusion of the affected territory (AUC = 0.86 and 0.85, P > .05). CONCLUSIONS: Models that combine simple variables such as neurological deficit severity, age, and admission glycemia were the most useful for predicting functional outcome in ischemic stroke patients submitted to revascularization treatments. Pre and post-treatment imaging findings did not enhance prognostic accuracy when compared to the patient's clinical improvement.