bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pulmonary hypertension in patients carrying FLNA loss-of-function variants
Authors: Stourm Laura , Grynblat Julien , Savale Laurent , Lacoste-Palasset Thomas , Jaïs Xavier , Coulet Florence , Levy Marilyne , Meyrignac Olivier , Ghigna Maria-Rosa , Cottin Vincent , Sitbon Olivier , Bonnet Damien , Goupil Francois , Humbert Marc , Gagnadoux Frederic , Montani David 
Published: November 2024

BackgroundPulmonary hypertension (PH) is an unusual complication of X-linked disease caused by loss-of-function (LOF) variants in thefilamin A(FLNA) gene. Patients withFLNALOF may also present dysmorphic facial features, aortic dilation, thrombopenia, and periventricular nodular heterotopia (PVNH).MethodsWe reported clinical, functional, radiologic, and hemodynamic characteristics of patients withFLNALOF variants and PH from the French PH Network.ResultsNine patients were identified with a female to male ratio of 8:1. PH was diagnosed at a median age of 36 [0–69] years. Associated conditions included epilepsy (n=5), PVNH (n=7), valvular heart disease (n=8), congenital heart diseases (n=4), thrombocytopenia (n=4), and hyperlaxity (n=4). Right heart catheterisation confirmed moderate-to-severe precapillary PH with a median mPAP of 33 [22–49] mmHg and PVR of 4.7 [2.4–8.0] WU. The DLCO was markedly decreased (48 [22–64] %pred) and five patients had obstructive ventilatory disorder. High-resolution CT showed heterogeneous parenchyma (n=8), emphysema (n=3), presence of a peripheral hyperclear band (n=3) and aortic ectasia (n=4). Pathologic assessment available in one patient revealed significant remodelling of small pulmonary arteries, interstitial edema, and irregular alveoli shapes. During follow-up, three patients died, including two from right heart failure. No patient died from aortic rupture.ConclusionsPrecapillary PH, likely due to multiple mechanisms, may complicate the course of patients with LOFFLNAvariants and may be the presenting symptom leading to diagnosis. The combination of PH with parenchymal involvement and extrapulmonary symptoms (epilepsy, congenital heart diseases, valvular and aortic involvement, thrombocytopenia) should prompt genetic screening forFLNA.
Vascular involvement in idiopathic pulmonary fibrosis
Authors: Mondoni Michele , Rinaldo Rocco , Ryerson Christopher J. , Albrici Cristina , Baccelli Andrea , Tirelli Claudio , Marchetti Francesca , Cefalo Jacopo , Nalesso Giulia , Ferranti Giulia , Alfano Fausta , Sotgiu Giovanni , Guazzi Marco , Centanni Stefano 
Published: November 2024

BackgroundIdiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing and progressive interstitial lung disease of unknown aetiology with a pathogenesis still partly unknown. Several microvascular and macrovascular abnormalities have been demonstrated in the pathogenesis of IPF and related pulmonary hypertension (PH), a complication of the disease.MethodsWe carried out a non-systematic, narrative literature review aimed at describing the role of the vasculature in the natural history of IPF.ResultsThe main molecular pathogenetic mechanisms involving vasculature (i.e.endothelial-to-mesenchymal transition, vascular remodelling, endothelial permeability, occult alveolar haemorrhage, vasoconstriction and hypoxia) and the genetic basis of vascular remodelling are described. The prevalence and clinical relevance of associated PH are highlighted with focus on the vasculature as a prognostic marker. The vascular effects of current antifibrotic therapies, the role of pulmonary vasodilators in the treatment of disease, and new pharmacological options with vascular-targeted activity are described.ConclusionsThe vasculature plays a key role in the natural history of IPF from the early phases of disease until development of PH in a subgroup of patients, a complication related to a worse prognosis. Pulmonary vascular volume has emerged as a novel computed tomography finding and a predictor of mortality, independent of PH. New pharmacological options with concomitant vascular-directed activity might be promising in the treatment of IPF.
Long COVID-19 and longer sick leave: longitudinal study of economically active patients
Authors: Gallo González Virginia , López-Padilla Daniel , Díez Javier de Miguel , Suárez Escudero Sergio , Ojeda Castillejo Elena , Ji Zichen , Puente Maestu Luis 
Published: November 2024

IntroductionSick leave was also part of the numerous consequences of the COVID-19 pandemic. Given the relevance of occupational status for any individual, the aim of the study was to evaluate the impact of persistent symptoms after active infection and determine associated factors with longer sick leaves (LSL).MethodsObservational study of economically active patients attending a post-COVID outpatient clinic for persistence of symptoms or radiological alterations after active infection. LSL temporal cut off point was defined by the third tertile of total leave days. Median leave time was compared with the optimal sick leave time for any other viral pneumonia, estimated by the local Ministry of Employment. To determine factors associated with LSL, multivariate models were performed.ResultsA total of 248 patients were included. Median sick leave time of all population was 53 days (interquartile range [IQR] 37–126.5), global sum of 30 169 days; median optimal sick leave time was 21.9 days (IQR 19.7–25.9) (p<0.05). Third tertile cut off point for LSL was 83 days, and multivariate analysis showed that dyspnea associated significantly with it (OR 3.26, 95% CI 1.59–6.70, p=0.0001), while physical exercise of at least 10 min a day significantly associated with shorter sick leaves (OR 0.45, 95% CI 0.20–0.98, p=0.04).DiscussionCOVID-19 sick leave was considerably longer than that stipulated for non-SARS-CoV-2 viral pneumonia. LongCOVID syndrome, especially dyspnea, seems to be a very present factor in the inability to work of these patients.
Acute effect of acetazolamide on exercise hemodynamics in patients with pulmonary arterial and chronic thromboembolic pulmonary hypertension: a randomised controlled trial
Authors: Carta Arcangelo F. , Saxer Stéphanie , Mayer Laura , Schneider Simon R. , Furian Michael , Schwarz Esther I. , Swenson Erik R. , Bloch Konrad E. , Lichtblau Mona , Ulrich Silvia 
Published: November 2024

Background/AimsThe aim of this study was to investigate the acute effect of acetazolamide on pulmonary hemodynamics during exercise in patients with pulmonary vascular disease (PVD).MethodsPatients with PVD diagnosed as pulmonary arterial or chronic thromboembolic pulmonary hypertension (PAH/CTEPH) underwent right heart catheterisation with hemodynamic measurements at rest and during stepwise incremental cycling exercise 60 min after intravenous administration of acetazolamide (500 mg) or placebo-saline according to a double-blind, randomised-controlled cross-over design. The main outcomes were the difference between pulmonary vascular resistance (PVR) and its components mean pulmonary artery pressure (mPAP), cardiac output (CO) and pulmonary arterial wedge pressure (PAWP) during exercise assessed by mixed linear regression analysis.Results24 patients (7 PAH, 17 CTEPH, 17 male) were included (mean±sdage 59±14 years). Treatment with acetazolamide compared to placebo-saline showed the following average marginal effects during exercise: unchanged end exercise power (mean difference and 95%CI, −0.9 W, −5.7 to 4.1 W, p=0.740), reduced mPAP (−1.7 mmHg, −2.9 to −0.5 mmHg, p=0.007), tendency to reduced CO (−0.3 L·min−1, −0.7 to 0.1 L·min−1, p=0.097), unchanged PVR (−0.1 WU, −0.3 to 0.2 WU, p=0.694), unchanged PAWP (−0.0 mmHg, −0.2 to 0.3 mmHg, p=0.783) and unchanged mPAP/CO-slope (0.5 mL·min−1, −0.7 to 1.6, p=0.431).ConclusionIntravenous acetazolamide was well tolerated and resulted in a significant but small decrease of mPAP while CO, PVR and the pressure-flow slope during exercise were unchanged.
Effect of Benralizumab treatment on the airway microbiome in COPD
Authors: Haldar Koirobi , Mistry Vijay , Richardson Mathew , Hamblet Corinne , Jison Maria , Barer Michael R. , McCrae Christopher , Brightling Christopher E. 
Published: November 2024

BackgroundOne-third of patients with Chronic obstructive pulmonary disease (COPD) have an eosinophilic inflammatory phenotype. Benralizumab is an afucosylated humanised monoclonal antibody that targets the IL-5 receptor α subunit, leading to rapid and near-complete eosinophil depletionviaantibody-dependent cellular cytotoxicity. We hypothesized that benralizumab targeted immune modulation could have an impact on the airway microbiome in COPD.ObjectiveTo investigate the effect of Benralizumab treatment on inflammation and the sputum microbiome in COPD.MethodsSputum samples from 94 COPD patients enrolled to the GALATHEA trial (NCT02138916) and randomized to receive placebo (33), benralizumab at 100 mg (29) or 30 mg (32) over 52 weeks, were analysed at baseline, week 24 and at end-of-treatment (week 56). Sputum microbiota taxonomic profiles and diversity indices, generated from paired-end Illumina sequencing targeting the 16sRNA gene, were used for comparative analyses. Linear mixed model analyses were applied on blood and sputum cell count and eosinophil mediators for within and between treatment group analysis.ResultsParticipants treated with 100 and 30 mg benralizumab respectively, showed a significant reduction from baseline in blood and sputum eosinophil counts (Blood p=1.2E-10 & p=8.8E-10; Sputum p=0.03 & p=0.004) and eosinophil-derived serum mediators (ECP p<3E-09 & p<2E-08; EDN p<8E- 12 & p<2E-09). No significant changes in the composition or diversity of the sputum microbiome were observed.ConclusionsIn this study, the airway microbiome measured in sputum was unaffected by targeted reduction of eosinophilic inflammation with benralizumab treatment.
Mannitol-evoked cough response is reduced by inhaled salbutamol
Authors: Wahab Mustafaa , Cusack Ruth P. , Stevens Caitlin , Howie Karen J. , Schlatman Abbey , Killian Kieran J. , O'Byrne Paul M. , Satia Imran , Gauvreau Gail M. 
Published: November 2024

BackgroundMannitol inhalation induces bronchoconstriction and coughing in asthmatics. Salbutamol causes smooth muscle relaxation in acute asthma, but its effects on airway nerves are unclear. We conducted mannitol cough challenges in allergic asthmatics (AA) and controls (HC) to determine whether salbutamol would reduce mannitol-evoked coughs independent of changes in airway calibre.MethodsA double-blind, randomized, placebo-controlled, 2-way crossover study included 20 mild steroid-naïve AA and 20 HC. The maximum number of coughs (Emax) evoked by mannitol was compared between salbutamol and placebo.ResultsHC and AA were matched for age, lung function, and sex. Mannitol-evoked cough dose ratio (CDR) in HC was significantly lower than in AA (5.63 (1.84)versus7.32 (3.67) coughs, p=0.023). Both groups had similar Emax, C2 and C5 (dose causing 2 and 5 coughs, respectively). In AA mannitol decreased FEV1by 8.11±2.32% with placebo, which was attenuated to 3.19±1.16% with salbutamol (p=0.03). In HC, FEV1reduction was not significant. Emax in AA for placebo and salbutamol was 8.90 (8.09–9.80) coughs and 7.07 (6.28–8.00) coughs, respectively (p<0.001) and in HC 8.19 (7.08–9.61) coughs and 4.95 (4.38–5.66) coughs, respectively (p<0.001). The effect of salbutamol on Emax between groups was significantly different (p<0.001). Salbutamol improved CDR, C2 and C5 in AA (p<0.05) but without effect between groups.ConclusionSalbutamol reduced mannitol Emax in AA and HC. The reduced mannitol-evoked cough response by salbutamol in HC was independent of changes in airway calibre measured by FEV1, suggesting mechanisms of salbutamol extend beyond smooth muscle relaxation.
Contact Stiffness Identification Method for Joint Surface and the Effect of Contact Stiffness on the Dynamic Response of the Cable Bracket
Authors: Wei Xue , Ren Zunsong , Li Guangquan 
Published: November 2024

AbstractThe cable bracket is one of the crucial components of high-speed Electric Multiple Units (EMUs), which is fixed to the axle box by bolts to support the cable. As an important parameter of the joint surface, contact stiffness significantly affects the dynamic response and service life of the cable bracket. However, it is difficult to directly measure the contact stiffness of the joint surface. As a result, an identification method is proposed in this investigation to identify the contact stiffness. A finite element model incorporating both normal and tangential stiffness parameters is developed. According to the finite element model and modal testing results, an improved particle swarm optimization algorithm is employed to identify the contact stiffness of the joint surface. The effect of the contact stiffness on the vibrations of the cable bracket is investigated. The findings indicate that pre-tightening torque significantly influences the contact stiffness of the cable bracket. The contact stiffness has a great effect on the vibrations of the cable bracket. Optimal contact stiffness notably reduces vibrations of the cable bracket, thereby extending its service life.
Human Gb3/CD77 synthase: a glycosyltransferase at the crossroads of immunohematology, toxicology, and cancer research
Authors: Szymczak-Kulus Katarzyna , Czerwinski Marcin , Kaczmarek Radoslaw 
Published: November 2024

AbstractHuman Gb3/CD77 synthase (α1,4-galactosyltransferase, P1/Pk synthase, UDP-galactose: β-d-galactosyl-β1-R 4-α-d-galactosyltransferase, EC 2.4.1.228) forms Galα1 → 4Gal structures on glycosphingolipids and glycoproteins. These glycans are recognized by bacterial adhesins and toxins. Globotriaosylceramide (Gb3), the major product of Gb3/CD77 synthase, is a glycosphingolipid located predominantly in plasma membrane lipid rafts, where it serves as a main receptor for Shiga toxins released by enterohemorrhagic Escherichia coli and Shigella dysenteriae of serotype 1. On the other hand, accumulation of glycans formed by Gb3/CD77 synthase contributes to the symptoms of Anderson–Fabry disease caused by α-galactosidase A deficiency. Moreover, variation in Gb3/CD77 synthase expression and activity underlies the P1PK histo-blood group system. Glycosphingolipids synthesized by the enzyme are overproduced in colorectal, gastric, pancreatic, and ovarian cancer, and elevated Gb3 biosynthesis is associated with cancer cell chemo- and radioresistance. Furthermore, Gb3/CD77 synthase acts as a key glycosyltransferase modulating ovarian cancer cell plasticity. Here, we describe the role of human Gb3/CD77 synthase and its products in the P1PK histo-blood group system, Anderson–Fabry disease, and bacterial infections. Additionally, we provide an overview of emerging evidence that Gb3/CD77 synthase and its glycosphingolipid products are involved in cancer metastasis and chemoresistance.
Graphical Abstract
Challenges in measurement of adolescent mental health: how are gender patterns affected when level of symptoms is analysed simultaneously with impairment?
Authors: Hagquist Curt 
Published: November 2024

Abstract
Background
Adolescent mental health surveys in public health are sometimes questioned because of their main focus on self-reported symptoms, lacking data on impairment, e.g. the consequences on everyday life of the mental health problems. While public health studies typically reveal higher prevalence rates of internalising problems for girls than boys, there are indications that the gender pattern may change when self-reported data on symptoms are analysed simultaneously with impairment. The purpose is to determine how gender patterns of adolescent mental health solely based on symptoms are affected when level of symptoms is analysed simultaneously with impairment.

Methods
Questionnaire data on adolescent mental health were collected in schools by Statistics Sweden in the autumn of 2009 as part of a national total population study in grades 6 and 9 in Sweden. In this study only data from grade 9 students are used (n = 91 627; response rate = 80 per cent). Psychosomatic symptoms were measured with the Psychosomatic Problems scale including eight items. Impairment was measured with four items included in the SDQ impact supplement. The associations between these key constructs were analysed with logistic regression and contingency tables.

Results
When analysing variables on psychosomatic symptoms and impairment independently, the results are consistent with typical findings of gender patterns in adolescent internalising mental health. Girls report both more psychosomatic symptoms, and more negative consequences in everyday life, than boys. The gender patterns are, however, strongly affected when impairment is conditioned on level of psychosomatic symptoms. Except for the Home Life setting, in the settings of Friendships, Classroom Learning and Leisure Activities, the previously reported gender pattern favoring higher disturbances among girls becomes partly reversed implying that boys report more negative consequences than girls. Hence, while girls report a higher prevalence of psychosomatic symptoms, boys appear to suffer from such symptoms more than, or as much as, girls in three out of four everyday life settings.

Conclusions
The study confirms the insufficiency of solely including data on symptoms in the measurement of adolescent mental health. Regardless of the causes of the complex gender pattern shown in this study, the results highlight the importance of simultaneous inclusion of indicators of impairment as well as symptom counts and frequency in the measurement of adolescent mental health.
Head circumference and intelligence, schooling, employment, and income: a systematic review
Authors: Freire Marina B O , Slater Rebeccah , Santos Thiago M , da Silva Bruna G C , Baxter Luke , Menezes Ana M B 
Published: November 2024

Abstract
Background
No consensus exists about the role of head circumference in identifying children at risk of suboptimal development. The objective of this study was to evaluate the association between head circumference and intelligence, schooling, employment, and income. The review 1) summarizes the overall evidence and 2) restricts the evidence to a subset of articles that met minimum quality criteria.

Methods
PubMed, Web of Science, PsycINFO, LILACS, CINAHL, WHO Institutional Repository for Information Sharing and UNICEF Innocenti were searched to identify published studies. Cohort, case–control or cross-sectional studies which evaluated the associations of interest in the general population, premature babies, babies with low birth weight or small for gestational age were included; head circumference must have been measured before the age of 20 years. Two reviewers independently performed study selection, data extraction and quality assessments.

Results
Of 2521 records identified, 115 were included and 21 met the minimum quality criteria. Ninety studies investigated if early measures of head circumference predict later outcomes and 25 studies measured head circumference and the outcome at the same timepoint; 78 studies adjusted the head circumference for age and sex. We identified large heterogeneity and inconsistency in the effect measures and data reported across studies. Despite the relatively large number of included articles, more than 80% presented serious limitations such as lack of adjustment for confounding and severe selection bias. Considering the subset of articles which met the minimum quality criteria, 12 of 16 articles showed positive association between head circumference and intelligence in the general population. However, in premature babies, 2 of 3 articles showed no clear effect. Head circumference was positively associated with academic performance in all investigated samples (5 of 5 articles). No article which evaluated educational attainment and employment met the minimum quality criteria, but the association between head circumference and these outcomes seems to be positive.

Conclusions
Larger head circumferences are positively associated with higher levels of intelligence and academic performance in the general population, but there is evidence of non-linearity in those associations. Identifying a group of children in higher risk for worse outcomes by a simple and inexpensive tool could provide an opportunity to mitigate these negative effects. Further research is needed for a deeper understanding of the whole distribution of head circumference and its effect in premature babies. Authors should consider the non-linearity of the association in the data analysis.

Trial registration
Association between head circumference and intelligence, educational attainment, employment, and income: A systematic review, CRD42021289998.
Needs assessment for interprofessional education module on prevention and early detection of oral cancer among dental interns: a cross- sectional survey
Authors: Sujir Nanditha , Ahmed Junaid , Ramakrishna Anand , Mohammed Ciraj Ali , Unnikrishnan Bhaskaran , Gilbert John HV 
Published: November 2024

Abstract
Background
The challenges associated with ensuring widespread system changes to enable early diagnosis and prevention of oral cancer could benefit from interprofessional practice. A needs assessment study was conducted to inform the Interprofessional Education and Collaborative Practice (IPECP) course related to oral cancer. The primary objectives of this study were 1) to establish a tool assess the knowledge attitude and practice (KAP) related to prevention and early detection of oral cancer of health professional students, and 2) to assess the same KAP of pre-licensure dental students. Additional objectives were to consider the possibility that dental students would demonstrate good scores related to early detection and prevention of oral cancer thus indicating their readiness for interprofessional learning and collaborative practice.

Methods
Two questionnaires were utilized for this study which included 1) Readiness for interprofessional learning was assessed using the pre- validated tool of Readiness for Interprofessional Learning Scale (RIPLS) 2) A questionnaire to assess the KAP related to early diagnosis and prevention of oral cancer which was developed, validated, and evaluated. Statistical analysis includes, descriptive statistics, Mann–Whitney U test, Ordered logistic regression and Probit analysis. p value was set at < 0.05.

Results
A total of 130 dental students (74.6% female) were included in the study. Mean scores related to KAP were 15.96 ± 1.394, 4.70 + 1.146, 7.02 ± 1.019 respectively. The mean score of RIPLS was 73.15 ± 15.961. The probability of overall samples to have good RIPLS scores was around 0.68 to 0.76 (Male 0.68—0.82 & Female 0.68 -0.74). The percentage of students having good knowledge score was 93.8%, good attitude score was around 54.6% and good practice score was around 90%.

Conclusion
Knowledge and practice related to prevention and early detection of oral cancer were scored highly. Attitude scores were lower in a relatively higher proportion of participants and needed to be addressed in the curriculum. RIPLS score indicates a positive attitude towards interprofessional learning.