Peripheral pulmonary artery stenosis in adults: a novel type of pulmonary vascular disease with a strong genetic background
Extract
Group 4 pulmonary hypertension (PH) includes several conditions associated with obstructions in the pulmonary arterial tree. Chronic thromboembolic PH (CTEPH), the most common type of group 4 PH in adults, has attracted the most attention to date. In this condition, the rise in pulmonary artery pressure results from a combination of the obstruction to flow caused by organised thrombus and pulmonary arterial hypertension (PAH)-like microvascular disease, which develops as a result of excessive blood flow and shear stress in unobstructed areas of the lung. For this reason, CTEPH is typically managed both through mech...
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Constantine, A., Dimopoulos, K., Gerges, C., Lang, I. M. Tags: Editorials Source Type: research
Having new eyes: MRI for visualisation of pulmonary vascular disease and prediction of bronchopulmonary dysplasia severity
Extract
Marcel Proust wrote that "the real voyage of discovery... consists not in seeking new landscapes but in having new eyes [1]." Indeed, our ability to noninvasively visualise the structure and function of human tissues has rapidly evolved. 130 years after Nikola Tesla described the first rotational magnetic field, modern magnetic resonance imaging (MRI) has transformed the ability to "see" the inner structures of human organs without having to surgically dissect them [2]. Innovative MRI protocols now allow for functional cardiac assessment and quantification of flow through the pulmonary circulation [3]. The study re...
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Sucre, J. M. S., Frank, D. B. Tags: Editorials Source Type: research
Gastro-oesophageal reflux and idiopathic pulmonary fibrosis: sorting the chicken and the egg by genetic link
Extract
Idiopathic pulmonary fibrosis (IPF) is a devastating condition that confers substantial morbidity and significantly reduces lifespan. Because of this, investigators have searched for modifiable risk factors to reduce the burden of IPF. While the aetiological holy grail for IPF remains elusive, there is mounting evidence that prevalent lung injuries on a background of genetic susceptibility culminate in this rare and "idiopathic" disease. Gastro-oesophageal reflux may be one of these prevalent lung injuries that culminate in lung fibrosis. In preclinical models, gastric acid aspiration into the lungs can trigger an ...
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Newton, C. A., Noth, I., Raghu, G. Tags: Editorials Source Type: research
AMR-Lung: a European Clinical Research Collaboration on antimicrobial resistance in chronic lung disease
Extract
Antimicrobial therapies, such as antibiotics and antifungals, occupy an important place in the treatment of patients with chronic respiratory diseases, such as bronchiectasis (including related to underlying cystic fibrosis (CF) and primary ciliary dyskinesia (PCD)) and COPD. These patients often become colonised with respiratory pathogens, which may cause chronic infection and play a role in acute exacerbations, episodes of increased airway inflammation with respiratory symptoms, that are usually infectious in origin. Depending on the underlying disease and patient conditions, antibiotics are commonly used to erad...
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Thee, S., Ekkelenkamp, M., Shah, A. Tags: Editorials Source Type: research
Epigenome-wide association studies: the exposures of yesterday form the methylations of tomorrow
Extract
Technological advances spur scientific discovery. This is certainly true for epigenomics, particularly DNA methylation (DNAm) analysis. Illumina's Infinium bead array platform has drastically evolved since the first array in 2010, now capturing >850 000 DNAm sites and has been used in over 8500 publicly available human datasets (gene expression omnibus; www.ncbi.nlm.nih.gov/geo/) spanning multiple organs, tissues and cell types. DNAm describes when a methyl group is chemically bound to a cytosine residue in DNA. The presence of this group can sterically interfere with gene expression through various complex mech...
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Weckmann, M., Reddy, K. D. Tags: Editorials Source Type: research
The results of genetic analysis and clinical outcomes after stent deployment in adult patients with isolated peripheral pulmonary artery stenosis
Extract
Peripheral pulmonary arterial stenosis (PPAS) is commonly observed in paediatric patients with associated congenital heart disease or systemic congenital syndromes, such as tetralogy of Fallot and Williams syndrome [1]. Meanwhile, isolated PPAS without other structural heart disease or systemic congenital syndromes is primarily reported in adulthood [2]. Recent genetic analyses have revealed that four cases of isolated PPAS in adulthood were homozygous for the ring finger protein 213 (RNF213) p.Arg4810Lys variant (also referred to as G to A alteration of rs112735431 and NM_001256071.3:c.14429G>A) [3]. RNF213 p.A...
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Kanezawa, M., Shimokawahara, H., Tsuji, M., Suruga, K., Miyagi, A., Marunaka, M., Mukai, T., Kawaguchi, T., Yang, T.-Y., Yamaguchi, I., Nagasaki, M., Matsuda, F., Matsubara, H. Tags: Pulmonary vascular disease Research Letters Source Type: research
Best step-up treatments for children with uncontrolled asthma: a systematic review and network meta-analysis of individual participant data
Conclusions
Uncontrolled children/adolescents on low-dose ICS should be recommended a change to medium-dose ICS+LABA to reduce the risk for exacerbation and improve lung function. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Cividini, S., Sinha, I., Donegan, S., Maden, M., Rose, K., Fulton, O., Culeddu, G., Hughes, D. A., Turner, S., Tudur Smith, C., on behalf of the EINSTEIN Collaborative Group Tags: Asthma and allergy Original Articles: Asthma Source Type: research
Adult-onset idiopathic peripheral pulmonary artery stenosis
Extract
Peripheral pulmonary artery stenosis (PPS) is defined as the obstruction of the pulmonary artery from the pulmonary artery trunk to the peripheral arteries. PPS is considered a paediatric-onset disease associated with systemic congenital diseases (congenital rubella syndrome, Williams syndrome, Alagille syndrome, Ehlers–Danlos syndrome and Noonan syndrome) [1–4]. Apart from PPS associated with congenital syndromes, adult-onset idiopathic PPS has been reported [5]; however, it is rare and has not been reported in more than 10 cases. Transcatheter pulmonary angioplasty for chronic thromboembolic pulmonary...
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Tamura, Y., Tamura, Y., Shigeta, A., Hosokawa, K., Taniguchi, Y., Inami, T., Adachi, S., Tsujino, I., Nakanishi, N., Sato, K., Sakamoto, J., Tanabe, N., Takama, N., Nakamura, K., Kubota, K., Komura, N., Kato, S., Yamashita, J., Takei, M., Joho, S., Ishii, Tags: Pulmonary vascular disease Original Articles: Pulmonary vascular disease Source Type: research
Novel insights into the whole-blood DNA methylome of asthma in ethnically diverse children and youth
Conclusions
We report novel whole-blood DNAm markers for asthma underlying key processes of the disease pathophysiology and confirm the transferability of previous asthma DNAm associations to ethnically diverse populations. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Herrera-Luis, E., Rosa-Baez, C., Huntsman, S., Eng, C., Beckman, K. B., LeNoir, M. A., Rodriguez-Santana, J. R., Villar, J., Laprise, C., Borrell, L. N., Ziv, E., Burchard, E. G., Pino-Yanes, M. Tags: Genetics, Asthma and allergy Original Articles: Asthma in children Source Type: research
Mediators of the association between gastro-oesophageal reflux disease and idiopathic pulmonary fibrosis
Extract
The causal relationship between idiopathic pulmonary fibrosis (IPF) and gastro-oesophageal reflux disease (GORD) has long been a subject of debate. However, it is only in the past 2 months that two studies utilising Mendelian randomisation have provided evidence of a significant causality between GORD and IPF, indicating that GORD indeed increases the risk of IPF. This finding has partially helped resolve the debate [1, 2]. Nevertheless, it should not be overlooked that previous meta-analyses have shown that treating GORD does not significantly improve the outcomes of IPF, contrary to expectations [3, 4]. This...
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Tao, H., Dong, Y., Chen, X., Peng, L. Tags: Interstitial and orphan lung disease Research Letters Source Type: research
MRI pulmonary artery flow detects lung vascular pathology in preterms with lung disease
Conclusions
Abnormalities in MRI PA flow and cardiac function exhibit significant, synergistic potential to detect BPD-associated PVD, advancing the possibilities of risk-adapted monitoring. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Authors: Häfner, F., Kindt, A., Strobl, K., Förster, K., Heydarian, M., Gonzalez, E., Schubert, B., Kraus, Y., Dalla Pozza, R., Flemmer, A. W., Ertl-Wagner, B., Dietrich, O., Stoecklein, S., Tello, K., Hilgendorff, A. Tags: Lung imaging, Pulmonary vascular disease Original Articles: Preterm birth and lung health Source Type: research
ERJ Podcast December 2023: Year in review
Extract
As part of the December issue, the European Respiratory Journal presents the latest in its series of podcasts. Chief Editor James Chalmers and Deputy Chief Editor Don Sin discuss their favourite articles from the past year and what they are looking forward to in 2024. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 21, 2023 Category: Respiratory Medicine Tags: Podcast Source Type: research
Clinical remission with biologic therapies in severe asthma: a matter of definition
Extract
Personalised medicine for treatment of severe asthma using relevant biologic therapies to target specific asthma phenotypes can be considered a major advance in disease management [1]. Agents that target eosinophilic inflammation, for example by interrupting the interleukin (IL)-5 pathway in patients with severe eosinophilic asthma, can halve the rate of severe asthma exacerbations [2]. It is possible that these agents influence the natural history of particular asthma phenotypes, potentially inducing disease remission (figure 1). (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 14, 2023 Category: Respiratory Medicine Authors: Beasley, R., Noble, J., Weatherall, M. Tags: Editorials Source Type: research
Reply: Simplifying pharmacotherapy for patients with COPD: a viewpoint
Extract
We have read the comments made by S. Suissa regarding the algorithm for COPD therapy that we recently proposed in our editorial viewpoint in the European Respiratory Journal [1]. As a statistician, he has concentrated on the possible confounders that support a lack of benefit on exacerbations and mortality when inhaled corticosteroids (ICS) are added to bronchodilators in COPD patients enrolled in clinical trials. He has done this over the years and indeed, he is the first author of all the six references supporting his argument in his correspondence. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 14, 2023 Category: Respiratory Medicine Authors: Celli, B., Vestbo, J. Tags: Correspondence Source Type: research
Simplifying pharmacotherapy for patients with COPD: a viewpoint
Extract
The opinion piece by Celli and Vestbo [1] proposing a simple algorithm for pharmacotherapy of patients with COPD, especially regarding inhaled corticosteroids (ICS), uses several randomised trials to support their recommendations. While simple, I submit that these are not supported by the evidence. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 14, 2023 Category: Respiratory Medicine Authors: Suissa, S. Tags: Correspondence Source Type: research
