Clinical Efficacy of Serum Antiglycopeptidolipid Core IgA Antibody Test for Screening Nontuberculous Mycobacterial Pulmonary Disease in Bronchiectasis: A European Multicenter Cohort Study.

Background: The serum antiglycopeptidolipid core IgA antibody test has been proposed as a diagnostic tool for Mycobacterium avium complex pulmonary diseases. Cross-reactivity with other nontuberculous mycobacteria (NTM), including Mycobacterium abscessus, indicates that it may have a role as a broader screening test for nontuberculous mycobacterial pulmonary disease (NTM-PD). NTM-PD is believed to be underdiagnosed in patients with bronchiectasis.

The ageing of people living with cystic fibrosis: what to expect now?

The prognosis of people with cystic fibrosis (pwCF) has improved dramatically with the introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulators (CFTRm). The ageing of the cystic fibrosis (CF) population is changing the disease landscape with the emergence of different needs and increasing comorbidities related to both age and long-term exposure to multiple treatments including CFTRm. Although the number of pwCF eligible for this treatment is expected to increase, major disparities in care and outcomes still exist in this population.

Williams-Campbell syndrome case series and discordant twins.

https://bit.ly/3Xqze8x.

The In-between: Time to Talk About Bronchiectasis in Adolescents and Their Transition to Adult Care.

Paediatric and adult bronchiectasis patients have been addressed in the literature as two different populations due to several differences, but there is insufficient evidence to understand how and when disease characteristics really change along patients' lifespan. This lack of knowledge is evident in all aspects of the transition: insufficient data is available about radiology, lung function, microbiology and treatment, and only limited information is currently available about changes in clinical presentation and psychosocial aspects. For instance, symptoms seem to improve during the third and fourth decades of life, a period sometimes referred to as the "honeymoon phase".

Patients at risk of nontuberculous mycobacterial pulmonary disease who need testing evaluated using a modified Delphi process by European experts.

Background: Identifying patients at risk of nontuberculous mycobacterial pulmonary disease (NTM-PD) is challenging. Delays in NTM-PD identification and management are associated with declining lung function and increased morbidity and mortality.

Study Design And Methods: European NTM-PD experts (n=12) participated in a three-round modified Delphi process to score symptoms and comorbidities potentially associated with NTM-PD as reasons to test for nontuberculous mycobacteria.

Airway IL-1β is related to disease severity and mucociliary function in bronchiectasis.

Rationale: The inflammasome is a key regulatory complex of the inflammatory response leading to interleukin-1β (IL-1β) release and activation. IL-1β amplifies inflammatory responses and induces mucus secretion and hyperconcentration in other diseases. The role of IL-1β in bronchiectasis has not been investigated.

Standards of care and educational gaps in adult cystic fibrosis units: a European Respiratory Society survey.

Background: Significant progress in the field of cystic fibrosis (CF) has substantially extended the life expectancy of patients with CF (pwCF). Consequently, the population of adult pwCF has outnumbered paediatric patients in most developed countries. Ageing is a new factor that can contribute to disease complexity and can require adaptation of CF units.

Maternal and fetal outcomes in multiparous women with Cystic Fibrosis.

Background: Quality of life and survival in Cystic Fibrosis (CF) have improved dramatically, making family planning a feasible option. Maternal and perinatal outcomes in women with CF (wwCF) are similar to those seen in the general population. However, the effect of undergoing multiple pregnancies is unknown.

Endotypes of Exacerbation in Bronchiectasis: An Observational Cohort Study.

Bronchiectasis is characterized by acute exacerbations, but the biological mechanisms underlying these events are poorly characterized. To investigate the inflammatory and microbial characteristics of exacerbations of bronchiectasis. A total of 120 patients with bronchiectasis were enrolled and presented with acute exacerbations within 12 months.

Challenges for a broad international implementation of the current severe community-acquired pneumonia guidelines.

Severe community-acquired pneumonia (sCAP) remains one of the leading causes of admission to the intensive care unit, thus consuming a large share of resources and is associated with high mortality rates worldwide. The evidence generated by clinical studies in the last decade was translated into recommendations according to the first published guidelines focusing on severe community-acquired pneumonia. Despite the advances proposed by the present guidelines, several challenges preclude the prompt implementation of these diagnostic and therapeutic measures.

Primary Ciliary Dyskinesia and Retinitis Pigmentosa: Novel Variant and Possible Modifier Gene.

We report a novel missense variant co-segregated with a familial X-linked retinitis pigmentosa (XLRP) case. The brothers were hemizygous for this variant, but only the proband presented with primary ciliary dyskinesia (PCD). Thus, we aimed to elucidate the role of the variant and other modifier genes in the phenotypic variability observed in the family and its impact on motile cilia.

Bronchiectasis and asthma: Data from the European Bronchiectasis Registry (EMBARC).

Background: Asthma is commonly reported in patients with a diagnosis of bronchiectasis.

Objective: The aim of this study was to evaluate whether patients with bronchiectasis and asthma (BE+A) had a different clinical phenotype and different outcomes compared with patients with bronchiectasis without concomitant asthma.

Methods: A prospective observational pan-European registry (European Multicentre Bronchiectasis Audit and Research Collaboration) enrolled patients across 28 countries.

The Association between Bronchiectasis and Chronic Obstructive Pulmonary Disease: Data from the European Bronchiectasis Registry (EMBARC).

COPD and bronchiectasis are commonly reported together. Studies report varying impacts of co-diagnosis on outcomes, which may be related to different definitions of disease used across studies. To investigate the prevalence of chronic obstructive pulmonary disease (COPD) associated with bronchiectasis and its relationship with clinical outcomes.

complex pulmonary disease patients with limited treatment options.

https://bit.ly/3QwLQ8T.

Ten Issues for Updating in Community-Acquired Pneumonia: An Expert Review.

Community-acquired pneumonia represents the third-highest cause of mortality in industrialized countries and the first due to infection. Although guidelines for the approach to this infection model are widely implemented in international health schemes, information continually emerges that generates controversy or requires updating its management. This paper reviews the most important issues in the approach to this process, such as an aetiologic update using new molecular platforms or imaging techniques, including the diagnostic stewardship in different clinical settings.

Serial lung ultrasound in monitoring viral pneumonia: the lesson learned from COVID-19.

Background: Lung ultrasound (LUS) has proven to be useful in the evaluation of lung involvement in COVID-19. However, its effectiveness for predicting the risk of severe disease is still up for debate. The aim of the study was to establish the prognostic accuracy of serial LUS examinations in the prediction of clinical deterioration in hospitalised patients with COVID-19.

Bronchiectasis in Europe: data on disease characteristics from the European Bronchiectasis registry (EMBARC).

Background: Bronchiectasis is a heterogeneous, neglected disease with few multicentre studies exploring the causes, severity, microbiology, and treatment of the disease across Europe. This aim of this study was to describe the clinical characteristics of bronchiectasis and compare between different European countries.

Methods: EMBARC is an international clinical research network for bronchiectasis.