bluuebunny/crossref_metadata_2025_split huggingface dataset:
Nebulized tranexamic acid for recurring hemoptysis in critically ill patients: case series
Authors: Alabdrabalnabi Fatimah , Alshahrani Mohammed , Ismail Nadia 
Published: August 2020

Abstract
Background
Hemoptysis is a clinical condition encountered in the emergency department (ED) and must be managed and investigated urgently to maintain the patient’s hemostasis. The management of hemoptysis depends on treating the underlying cause. Tranexamic acid (TXA) is an anti-fibrinolytic drug used to systemically control bleeding. There are a few studies available that investigate the use of nebulized tranexamic acid for hemoptysis with contradictory results. Our paper demonstrates three cases where patients presented with significant hemoptysis and had significant improvement in symptoms following the administration of nebulized tranexamic acid. The overall need for blood transfusion was reduced.

Results
Three patients presented to the emergency room for evaluation of hemoptysis. All three patients had different underlying pathologies resulting in their hemoptysis and were monitored in the ICU. Initial conventional medical therapies including the correction of coagulopathy and discontinuing offending agents were utilized for treatment. After persistent symptoms, nebulized TXA at a dose of 500 mg three times a day was administered. The patients were all discharged from the hospital with improvement in their symptoms.

Conclusion
Tranexamic acid may be considered in the treatment of hemoptysis regardless of the underlying cause. This may be utilized pending further workup and investigation into the underlying source of the bleeding.
Genomic and transcriptomic insights into Raffaelea lauricola pathogenesis
Authors: Zhang Yucheng , Zhang Junli , Vanderpool Dan , Smith Jason A. , Rollins Jeffrey A. 
Published: August 2020

Abstract
Background
Laurel wilt caused by Raffaelea lauricola is a lethal vascular disease of North American members of the Lauraceae plant family. This fungus and its primary ambrosia beetle vector Xyleborus glabratus originated from Asia; however, there is no report of laurel wilt causing widespread mortality on native Lauraceae trees in Asia. To gain insight into why R. lauricola is a tree-killing plant pathogen in North America, we generated and compared high quality draft genome assemblies of R. lauricola and its closely related non-pathogenic species R. aguacate.

Results
Relative to R. aguacate, the R. lauricola genome uniquely encodes several small-secreted proteins that are associated with virulence in other pathogens and is enriched in secondary metabolite biosynthetic clusters, particularly polyketide synthase (PKS), non-ribosomal peptide synthetase (NRPS) and PKS-NRPS anchored gene clusters. The two species also exhibit significant differences in secreted proteins including CAZymes that are associated with polysaccharide binding including the chitin binding CBM50 (LysM) domain. Transcriptomic comparisons of inoculated redbay trees and in vitro-grown fungal cultures further revealed a number of secreted protein genes, secondary metabolite clusters and alternative sulfur uptake and assimilation pathways that are coordinately up-regulated during infection.

Conclusions
Through these comparative analyses we have identified potential adaptations of R. lauricola that may enable it to colonize and cause disease on susceptible hosts. How these adaptations have interacted with co-evolved hosts in Asia, where little to no disease occurs, and non-co-evolved hosts in North America, where lethal wilt occurs, requires additional functional analysis of genes and pathways.
International severe asthma registry (ISAR): protocol for a global registry
Authors: FitzGerald J. Mark , Tran Trung N. , Alacqua Marianna , Altraja Alan , Backer Vibeke , Bjermer Leif , Bjornsdottir Unnur , Bourdin Arnaud , Brusselle Guy , Bulathsinhala Lakmini , Busby John , Canonica Giorgio W. , Carter Victoria , Chaudhry Isha , Cho You Sook , Christoff George , Cosio Borja G. , Costello Richard W. , Eleangovan Neva , Gibson Peter G. , Heaney Liam G. , Heffler Enrico , Hew Mark , Hosseini Naeimeh , Iwanaga Takashi , Jackson David J. , Jones Rupert , Koh Mariko S. , Le Thao , Lehtimäki Lauri , Ludviksdottir Dora , Maitland-van der Zee Anke H. , Menzies-Gow Andrew , Murray Ruth B. , Papadopoulos Nikolaos G. , Perez-de-Llano Luis , Peters Matthew , Pfeffer Paul E. , Popov Todor A. , Porsbjerg Celeste M. , Price Chris A. , Rhee Chin K. , Sadatsafavi Mohsen , Tohda Yuji , Wang Eileen , Wechsler Michael E. , Zangrilli James , Price David B. 
Published: August 2020

Abstract
Background
Severe asthma exerts a disproportionately heavy burden on patients and health care. Due to the heterogeneity of the severe asthma population, many patients need to be evaluated to understand the clinical features and outcomes of severe asthma in order to facilitate personalised and targeted care. The International Severe Asthma Registry (ISAR) is a multi-country registry project initiated to aid in this endeavour.

Methods
ISAR is a multi-disciplinary initiative benefitting from the combined experience of the ISAR Steering Committee (ISC; comprising 47 clinicians and researchers across 29 countries, who have a special interest and/or experience in severe asthma management or establishment and maintenance of severe asthma registries) in collaboration with scientists and experts in database management and communication. Patients (≥18 years old) receiving treatment according to the 2018 definitions of the Global Initiative for Asthma (GINA) Step 5 or uncontrolled on GINA Step 4 treatment will be included. Data will be collected on a core set of 95 variables identified using the Delphi method. Participating registries will agree to provide access to and share standardised anonymous patient-level data with ISAR. ISAR is a registered data source on the European Network of Centres for Pharmacoepidemiology and Pharmacovigilance. ISAR’s collaborators include Optimum Patient Care, the Respiratory Effectiveness Group (REG) and AstraZeneca. ISAR is overseen by the ISC, REG, the Anonymised Data Ethics & Protocol Transparency Committee and the ISAR operational committee, ensuring the conduct of ethical, clinically relevant research that brings value to all key stakeholders.

Conclusions
ISAR aims to offer a rich source of real-life data for scientific research to understand and improve disease burden, treatment patterns and patient outcomes in severe asthma. Furthermore, the registry will provide an international platform for research collaboration in respiratory medicine, with the overarching aim of improving primary and secondary care of adults with severe asthma globally.
Older persons are frailer after an emergency care visit to the out-of-hours general practitioner cooperative in the Netherlands: a cross-sectional descriptive TOPICS-MDS study
Authors: Bloemhoff Anneke , Schoon Yvonne , Smulders Kien , Akkermans Reinier , Vloet Lilian C. M. , van den Berg Karin , Berben Sivera A. A. ,   On behalf of the TOPICS-MDS Consortium
Published: August 2020

Abstract
Background
In the Netherlands, community-dwelling older people with primary care emergency problems contact the General Practitioner Cooperative (GPC) after hours. However, frailty remains an often unobserved hazard with adverse health outcomes. The aim of this study was to provide insight into differences between older persons with or without GPC emergency care visits (reference group) regarding frailty and healthcare use.

Methods
A cross-sectional descriptive study design was based on data from the public data repository of The Older Persons and Informal Caregivers Survey Minimum Dataset (TOPICS-MDS). Frailty in older persons (65+ years, n = 32,149) was measured by comorbidity, functional and psychosocial aspects, quality of life and a frailty index. Furthermore, home care use and hospital admissions of older persons were identified. We performed multilevel logistic and linear regression analyses. A random intercept model was utilised to test differences between groups, and adjustment factors (confounders) were used in the multilevel analysis.

Results
Compared to the reference group, older persons with GPC contact were frailer in the domain of comorbidity (mean difference 0.52; 95% CI 0.47–0.57, p < 0.0001) and functional limitations (mean difference 0.53; 95% CI 0.46–0.60, p < 0.0001), and they reported less emotional wellbeing (mean difference − 4.10; 95% CI -4.59- -3.60, p < 0.0001) and experienced a lower quality of life (mean difference − 0.057; 95% CI -0.064- -0.050, p < 0.0001). Moreover, older persons more often reported limited social functioning (OR = 1.50; 95% CI 1.39–1.62, p < 0.0001) and limited perceived health (OR = 1.50, 95% CI 1.39–1.62, p < 0.0001). Finally, older persons with GPC contact more often used home care (OR = 1.37; 95% CI 1.28–1.47, p < 0.0001) or were more often admitted to the hospital (OR = 2.88; 95% CI 2.71–3.06, p < 0.0001).

Conclusions
Older persons with out-of-hours GPC contact for an emergency care visit were significantly frailer in all domains and more likely to use home care or to be admitted to the hospital compared to the reference group. Potentially frail older persons seemed to require adequate identification of frailty and support (e.g., advanced care planning) both before and after a contact with the out-of-hours GPC.
A novel delins (c.773_819+47delinsAA) mutation of the PCCA gene associated with neonatal-onset propionic acidemia: a case report
Authors: Wang Hai-rong , Liu Yan-qiu , He Xue-lian , Sun Jun , Zeng Fan-wei , Yan Cheng-bin , Li Hao , Gao Shu-yang , Yang Yun 
Published: August 2020

Abstract
Background
Propionic acidemia (PA)(OMIM#606054) is an inborn error of branched-chain amino acid metabolism, caused by defects in the propionyl-CoA carboxylase (PCC) enzyme which encoded by the PCCA and PCCB genes.

Case presentation
Here we report a Chinese neonate diagnosed with suspected PA based on the clinical symptoms, gas chromatography-mass spectrometry (GC/MS), and brain imaging tests. Targeted next-generation sequencing (NGS) was performed on the proband. We detected only one heterozygous recurrent nonsense variant (c.937C > T, p.Arg313Ter) in the PCCA gene. When we manually checked the binary alignment map (BAM) diagram of PCCA gene, we found a heterozygous deletion chr13:100915039-100915132delinsAA (c.773_819 + 47delinsAA) (GRCh37.p13) inside the exon 10 in the PCCA gene. The results were validated by Sanger sequencing and qPCR method in the family: the variant (c.937C > T, p.Arg313Ter) was in the maternal allele, and the delins was in the paternal allele. When the mother was pregnant again, prenatal diagnosis was carried out through amniocentesis at 18 weeks gestation, the fetus carried neither of the two mutations. After birth, newborn screening was undertaken, the result was negative.

Conclusions
We identified a recurrent c.937C > T and a novel c.773_819 + 47delinsAA mutations in the PCCA gene, which may be the genetic cause of the phenotype of this patient. Our findings expanded the spectrum of causative genotype-phenotype of the PCCA gene. For the cases, the NGS results revealed only a heterozygous mutation in autosomal recessive disease when the gene is associated with phenotypes, it is necessary to manually check the BAM diagram to improve the detection rate. Targeted NGS is an effective technique to detect the various genetic lesions responsible for the PA in one step. Genetic testing is essential for genetic counselling and prenatal diagnosis in the family to avoid birth defects.
Association among kidney function, frailty, and oral function in patients with chronic kidney disease: a cross-sectional study
Authors: Kosaka Shiho , Ohara Yuki , Naito Shotaro , Iimori Soichiro , Kado Hiroshi , Hatta Tsuguru , Yanishi Masaaki , Uchida Shinichi , Tanaka Makoto 
Published: August 2020

Abstract
Background
Chronic kidney disease (CKD) involves many factors that can cause frailty and oral hypofunction. We aimed to investigate the prevalence of frailty and oral hypofunction and to examine the associations among kidney function, frailty, and oral function in adults with CKD in Japan.

Methods
This cross-sectional study was conducted at two institutions. The participants included 109 patients with CKD stages 3–5 who visited outpatient clinics or were admitted for inpatient treatment. Frailty was evaluated using the Japanese version of the Cardiovascular Health Study frailty criteria. Oral function was evaluated by assessing oral motor skills [oral diadochokinesis (ODK) rate], masticatory ability, and the repetitive saliva swallowing test. The estimated glomerular filtration rate (eGFR) was used to indicate kidney function. We examined the associations among kidney function, frailty, and oral function using binomial logistic regression analysis.

Results
In total, 31 participants (28.4%) were classified as being frail. Univariate analysis showed that age, body mass index, eGFR, and haemoglobin level were significantly associated with frailty. ODK and swallowing function were significantly associated with frailty. Multivariate analysis revealed that frailty was significantly associated with eGFR [odds ratio (OR) 0.96, 95% confidence interval (CI) 0.92–1.00, p = 0.048] and ODK rate (OR 0.68, CI 0.47–0.98, p = 0.038). However, no significant association was found between CKD severity and masticatory or swallowing function.

Conclusion
We found a high prevalence of frailty in patients with CKD and a significant association between frailty and oral motor skills, affecting the swallowing function of patients with nondialysis CKD. The high prevalence of frailty among patients with CKD suggests that routine assessment of frailty is necessary to prevent the development of severe complications. In addition, oral and kidney function should be carefully evaluated, and oral health education and interventions should be performed for patients with CKD.
Effectiveness of extracorporeal blood purification (hemoadsorption) in patients with severe coronavirus disease 2019 (COVID-19)
Authors: Asgharpour Masoumeh , Mehdinezhad Hamed , Bayani Masoumeh , Zavareh Mahmoud Sadeghi Haddad , Hamidi Seyed Hossein , Akbari Roghayeh , Ghadimi Reza , Bijani Ali , Mouodi Simin 
Published: August 2020

Abstract
Background
Extracorporeal blood purification has been proposed as one of the therapeutic approaches in patients with coronavirus infection, because of its beneficial impact on elimination of inflammatory cytokines.

Methods
This controlled trial has been conducted on critically ill COVID-19 patients admitted in the state hospital affiliated to Babol University of Medical Sciences, Iran who received different antiviral and antibacterial drugs, and different modalities of respiratory treatments and did not have positive clinical improvement. No randomization and blindness was considered. All of the participants underwent three sessions of resin-directed hemoperfusion using continuous renal replacement therapy with a mode of continuous venovenous hemofiltration (CVVH).

Results
Five men and five women with a mean age of 57.30 ± 18.07 years have been enrolled in the study; and six of them have improved after the intervention. Peripheral capillary oxygen saturation (SpO2) changed after each session. Mean SpO2 before the three sessions of hemoperfusion was 89.60% ± 3.94% and increased to 92.13% ± 3.28% after them (p < 0.001). Serum IL-6 showed a reduction from 139.70 ± 105.62 to 72.06 ± 65.87 pg/mL (p = 0.073); and c-reactive protein decreased from 136.25 ± 84.39 to 78.25 ± 38.67 mg/L (P = 0.016).

Conclusions
Extracorporeal hemoadsorption could improve the general condition in most of recruited patients with severe coronavirus disease; however, large prospective multicenter trials in carefully selected patients are needed to definitely evaluate the efficacy of hemoperfusion in COVID-19 patients.

Trial registration
The research protocol has been registered in the website of Iranian Registry of Clinical Trials with the reference number IRCT20150704023055N2.
Aortic pulse wave velocity, central pulse pressure, augmentation index and chronic kidney disease progression in individuals with type 2 diabetes: a 3- year prospective study
Authors: Liu Jian-Jun , Liu Sylvia , Lee Janus , Gurung Resham L. , Yiamunaa M. , Ang Keven , Shao Yi Ming , Choo Robin W. M. , Tavintharan Subramaniam , Tang Wern Ee , Sum Chee Fang , Lim Su Chi 
Published: August 2020

Abstract
Background
Pulse wave velocity (PWV), central pulse pressure and augmentation index are arterial stiffness- related hemodynamic parameters but their associations with renal outcome are still controversial. We hereby aim to study, 1) which hemodynamic parameter is independently associated with progressive chronic kidney disease (CKD), 2) the association of 3-year change in PWV with CKD progression and, 3) the additive predictive value of PWV for progressive CKD.

Methods
Carotid- femoral PWV, central pulse pressure and augmentation index were measured in 1444 participants with type 2 diabetes at baseline and 3 years apart. Progressive CKD was defined as confirmed eGFR decline 40% or greater.

Results
In the follow-up, 102 participants experienced progressive CKD. All 3 hemodynamic parameters were significantly associated with progressive CKD In univariable analysis. However, only PWV remained statistically significant after adjustment for known clinical risk factors and the other 2 hemodynamic parameters (OR 1.14 [95% CI 1.01–1.29] per m/s increment). One m/s regression (decrement) in PWV in the 3-year follow-up was associated with 26% lower adjusted- risk of progressive CKD (OR 0.74, 95% CI 0.56–0.97). Adding PWV onto traditional risk factor- based model significantly improved classification (net reclassification improvement 0.25, 95% CI 0.05–0.45, P = 0.01) and positive prediction rate (24.5 to 32.3%).

Conclusions
Of 3 arterial stiffness- related hemodynamic parameters, only PWV is independently associated with progressive CKD. PWV may be a potential intervention target to mitigate risk of CKD progression and also a biomarker to improve risk-stratification of adverse renal outcome in individuals with type 2 diabetes.
Gestational weight gain and body composition of full-term newborns and infants: a cohort study
Authors: Nehab Sylvia R. , Villela Letícia D. , Soares Fernanda V. M. , Abranches Andrea D. , Araújo Daniele M. R. , da Silva Leila M. L. , Amaral Yasmin N. V. , Junior Saint Clair G. , Meio Maria Dalva B. B. , Moreira Maria Elisabeth 
Published: August 2020

AbstractBackgroundThe association between gestational weight gain and neonatal body composition has been inconsistent, exposing the need for further research. The aim of this study was to evaluate whether gestational weight gain influences the body composition of full-term newborns and infants up to 4 months old.MethodsA cohort study was performed with 124 participants divided into categories of gestational weight gain according to the 2009 Institute of Medicine guidelines. The anthropometric and body composition data of newborns and infants acquired using air displacement plethysmography (PeaPod®) were collected at 96 h, 1 month, 2 months and 4 months of life. In the statistical analysis, the chi-square test was used to analyze categorical variables, and ANOVA was used to analyze numerical variables. Univariate analysis was performed, and the absolute and relative frequencies of the categorical variables, as well as mean and standard deviation of the numerical variables, were obtained. Bivariate analysis was performed for the categories of gestational weight gain and gestational and neonatal characteristics. When adjustments to gestational hypertension, gestational diabetes, and pregestational body mass index (BMI) were analyzed by linear regression, gestational weight gain remained a significant variable for newborn percent fat mass. For all analyses, a significance level of 5% was adopted.ResultsGestational weight gain was adequate in 33.8% of the participants, excessive in 41.1% and insufficient in 25%. Women with excessive weight gain had higher pregestational BMIs and a higher incidence of gestational hypertension. Their newborns had a higher body mass, body fat mass in grams and percent fat mass than the infants born to mothers with adequate or insufficient gestational weight gain. No significant differences were observed in body composition at 1, 2 and 4 months of life during infant follow-up.ConclusionExcessive gestational weight gain may alter the body composition of newborns at birth. Further studies are required to better evaluate infant follow-up.Trial registrationClinical Trial Registry:NCT00875251on April 3, 2009.
Effectiveness of community-based folate-oriented tertiary interventions on incidence of fetus and birth defects: a protocol for a single-blind cluster randomized controlled trial
Authors: Li Mengru , Zhang Yi , Chen Xiaotian , Wang Dingmei , Ji Mi , Jiang Yuan , Dou Yalan , Ma Xiaojing , Sheng Wei , Yan Weili , Huang Guoying 
Published: August 2020

Abstract
Background
Birth defects are the main cause of fetal death, infant mortality and morbidity worldwide. However, the etiology of birth defects remains largely unknown. Maternal folate status during periconception plays an important role in organogenesis and folic acid supplement reduces the risk of neural tube defects, congenital heart diseases, and several other birth defects. This trial seeks to evaluate the effectiveness of folate-oriented tertiary interventions during periconception on the incidence of fetus and birth defects.

Methods
This is a single-blind, two-arm cluster randomized controlled trial in Shanghai, China. Eligible women from 22 clusters are recruited at pre-pregnancy physical examinations clinical settings. Compared to the routine perinatal care group (control arm), folate-oriented tertiary interventions will be provided to the intervention arm. The core interventions consist of assessments of folate status and metabolism, folate intake guidance, and re-evaluation of folate status to ensure red blood cell folate level above 400 ng/ml (906 nmol/L) before pregnancy. Screening and consulting of fetus and birth defects, and treatments of birth defects during pregnancy and afterward will be provided to both arms. The primary outcome is a composite incidence of fetus defects, stillbirth, and neonatal birth defects identified from the confirmation of pregnancy to 28 days after birth. Secondary outcomes include maternal and offspring adverse complications and cost-effectiveness of folate-oriented tertiary interventions. This protocol adheres to the SPIRIT Checklist.

Discussion
To achieve the recommended folate status before or during pregnancy is still a challenge worldwide. This community-based cluster-randomized controlled intervention trial will evaluate the effectiveness of a package of interventions aiming at achieving recommended maternal folate status covering pre- and during pregnancy in reducing fetus and birth defects. Our study has the potential to improve the community-based practice of reducing modifiable risk factors of disease and improving primary prevention of the defects in China. The procedures would formulate the policy on folic acid supplementation during periconception against birth defects in primary care settings.

Trial registration
Clinical Trial Registry, NCT03725878. Prospectively registered on 31 October 2018.