bluuebunny/crossref_metadata_2025_split huggingface dataset:
Paediatric pulmonary Langerhans cell histiocytosis
Authors: Barclay Mhairi , Devaney Rebecca , Bhatt Jayesh. M. 
Published: July 2020

Paediatric pulmonary Langerhans cell histiocytosis (pPLCH) is a rare diffuse cystic lung disease. Unlike pulmonary Langerhans cell histiocytosis (LCH) in adults, which is often seen as an isolated condition with smoking being a major risk factor, isolated pPLCH is vanishingly rare in children and it is most often a component of multisystem LCH. Diagnosis should be based on histological and immunophenotypic examination of affected tissue in addition to clinical and radiological features. It should be considered an important differential for diffuse cystic lung disease in paediatric patients. Recent progress in the biological understanding of the disease supports the classification of LCH as an inflammatory myeloid neoplasia. Chemotherapy and specific management of respiratory complications are the mainstays of treatment. The lungs are no longer considered a “risk organ” in LCH as pulmonary involvement is not associated with a worse prognosis than the involvement of other organs. Multidisciplinary treatment approaches are needed.Prognosis can be good but is adversely influenced by multisystem involvement, and complications such as pneumothoraces and respiratory failure can be life threatening. This review aims to give an overview of this condition, with a focus on the diagnosis, monitoring and management of complications such as pneumothoraces and respiratory failure, which can be challenging for the paediatric respiratory specialist.Educational aimsTo give an overview of paediatric pulmonary LCH.To discuss the differential diagnosis of paediatric cystic lung disease.
Caspase1/11 signaling affects muscle regeneration and recovery following ischemia, and can be modulated by chloroquine
Authors: Sachdev Ulka , Ferrari Ricardo , Cui Xiangdong , Pius Abish , Sahu Amrita , Reynolds Michael , Liao Hong , Sun Ping , Shinde Sunita , Ambrosio Fabrisia , Shiva Sruti , Loughran Patricia , Scott Melanie 
Published: July 2020

Abstract
Background
We previously showed that the autophagy inhibitor chloroquine (CQ) increases inflammatory cleaved caspase-1 activity in myocytes, and that caspase-1/11 is protective in sterile liver injury. However, the role of caspase-1/11 in the recovery of muscle from ischemia caused by peripheral arterial disease is unknown. We hypothesized that caspase-1/11 mediates recovery in muscle via effects on autophagy and this is modulated by CQ.

Methods
C57Bl/6 J (WT) and caspase-1/11 double-knockout (KO) mice underwent femoral artery ligation (a model of hind-limb ischemia) with or without CQ (50 mg/kg IP every 2nd day). CQ effects on autophagosome formation, microtubule associated protein 1A/1B-light chain 3 (LC3), and caspase-1 expression was measured using electron microscopy and immunofluorescence. Laser Doppler perfusion imaging documented perfusion every 7 days. After 21 days, in situ physiologic testing in tibialis anterior muscle assessed peak force contraction, and myocyte size and fibrosis was also measured. Muscle satellite cell (MuSC) oxygen consumption rate (OCR) and extracellular acidification rate was measured. Caspase-1 and glycolytic enzyme expression was detected by Western blot.

Results
CQ increased autophagosomes, LC3 consolidation, total caspase-1 expression and cleaved caspase-1 in muscle. Perfusion, fibrosis, myofiber regeneration, muscle contraction, MuSC fusion, OCR, ECAR and glycolytic enzyme expression was variably affected by CQ depending on presence of caspase-1/11. CQ decreased perfusion recovery, fibrosis and myofiber size in WT but not caspase-1/11KO mice. CQ diminished peak force in whole muscle, and myocyte fusion in MuSC and these effects were exacerbated in caspase-1/11KO mice. CQ reductions in maximal respiration and ATP production were reduced in caspase-1/11KO mice. Caspase-1/11KO MuSC had significant increases in protein kinase isoforms and aldolase with decreased ECAR.

Conclusion
Caspase-1/11 signaling affects the response to ischemia in muscle and effects are variably modulated by CQ. This may be critically important for disease treated with CQ and its derivatives, including novel viral diseases (e.g. COVID-19) that are expected to affect patients with comorbidities like cardiovascular disease.
Integrated assessment of biomarker responses and comparative toxicity pathway in snails Bellamya aeruginosa exposed to tributyltin and triphenyltin in sediments
Authors: Wu Lingling , Chen Ling , Zhu Lingjiao , Chen Xiaoping , Li Qian 
Published: July 2020

Abstract
Background
Sediments function as a secondary and significant source of tributyltin (TBT) and triphenyltin (TPT) in aquatic ecosystems and may pose a potential threat on benthic organisms and human health. The subchronic toxicity of sediment-associated TBT or TPT to snails Bellamya aeruginosa at environmentally relevant concentrations was investigated in this study. Multiple endpoints at the biochemical [ethoxyresorufin-O-deethylase (EROD), superoxide dismutase (SOD), catalase (CAT), protein carbonyl content (PCC) and lipid peroxidation (LPO)] and transcriptomic levels were examined.

Results
TBT or TPT in sediment could induce antioxidant enzymes’ activities and result in oxidative damage in the hepatopancreas of B. aeruginosa after 28-day exposure. A transcriptomic profile of B. aeruginosa exposed to TBT and TPT was reported. CYP genes and EROD activity were sensible and reliable biomarkers for toxicity assessment of TBT or TPT in sediments. Comparative pathway analysis revealed the alteration of steroid hormone biosynthesis and retinol metabolism in B. aeruginosa after 90-day exposure to sediment-associated TBT at the concentration of 2000 ng/g dw, which might affect both reproduction and lipogenesis functions. The ubiquitin proteasome system and immune system might be the toxicity target in B. aeruginosa after exposure to sediment-associated TPT for 90 days.

Conclusions
The results offered new mechanisms underlying the toxicity of sediment-associated tributyltin and triphenyltin.
Production and verification of the first Atlantic salmon (Salmo salar L.) clonal lines
Authors: Hansen Tom Johnny , Penman David , Glover Kevin Alan , Fraser Thomas William Kenneth , Vågseth Tone , Thorsen Anders , Sørvik Anne Grete Eide , Fjelldal Per Gunnar 
Published: July 2020

AbstractBackgroundIn several fish species homozygous and heterozygous clonal lines have been produced using gynogenetic and androgenetic techniques. These lines are standardized and can be reproduced over generations. In rainbow trout such lines have existed for decades and has become important research tools in genome studies as well as in studies of commercially important traits. The Atlantic salmon is one of the best studied fish species globally, but all experiments are done on fish of wild or domesticated origin and access to standardized immortal fish lines would be of great benefit. Here, we describe the protocols developed to produce mitotic gynogenes, and from these the first clonal lines in Atlantic salmon.ResultsAtlantic salmon eggs fertilized with UV irradiated sperm combined with a pressure shock applied at 4700–4800 minC at 8 °C gave all homozygous (doubled haploid) gynogenetic progeny with high survival. From the six first maturing females, five all homozygous clonal lines were produced by meiotic gynogenesis and were verified as clonal and identical to their mother with microsatellite markers.ConclusionsWe have now produced the first documented cloned Atlantic salmon lines. This work demonstrates the potential for production of further Atlantic salmon clonal lines, potentially with distinct characteristics. Such lines will provide an important resource for further elucidation of phenotypic and genetic traits in this globally important species.
Genomic diversity in pearl millet inbred lines derived from landraces and improved varieties
Authors: Kanfany Ghislain , Serba Desalegn D. , Rhodes Davina , St. Amand Paul , Bernardo Amy , Gangashetty Prakash I , Kane Ndjido Ardo , Bai Guihua 
Published: July 2020

Abstract
Background
Genetic improvement of pearl millet is lagging behind most of the major crops. Development of genomic resources is expected to expedite breeding for improved agronomic traits, stress tolerance, yield, and nutritional quality. Genotyping a breeding population with high throughput markers enables exploration of genetic diversity, population structure, and linkage disequilibrium (LD) which are important preludes for marker-trait association studies and application of genomic-assisted breeding.

Results
Genotyping-by-sequencing (GBS) libraries of 309 inbred lines derived from landraces and improved varieties from Africa and India generated 54,770 high quality single nucleotide polymorphism (SNP) markers. On average one SNP per 29 Kb was mapped in the reference genome, with the telomeric regions more densely mapped than the pericentromeric regions of the chromosomes. Population structure analysis using 30,208 SNPs evenly distributed in the genome divided 309 accessions into five subpopulations with different levels of admixture. Pairwise genetic distance (GD) between accessions varied from 0.09 to 0.33 with the average distance of 0.28. Rapid LD decay implied low tendency of markers inherited together. Genetic differentiation estimates were the highest between subgroups 4 and 5, and the lowest between subgroups 1 and 2.

Conclusions
Population genomic analysis of pearl millet inbred lines derived from diverse geographic and agroecological features identified five subgroups mostly following pedigree differences with different levels of admixture. It also revealed the prevalence of high genetic diversity in pearl millet, which is very useful in defining heterotic groups for hybrid breeding, trait mapping, and holds promise for improving pearl millet for yield and nutritional quality. The short LD decay observed suggests an absence of persistent haplotype blocks in pearl millet. The diverse genetic background of these lines and their low LD make this set of germplasm useful for traits mapping.
Proteomic analysis of Rickettsia akari proposes a 44 kDa-OMP as a potential biomarker for Rickettsialpox diagnosis
Authors: Csicsay František , Flores-Ramirez Gabriela , Zuñiga-Navarrete Fernando , Bartošová Mária , Fučíková Alena , Pajer Petr , Dresler Jiří , Škultéty Ľudovít , Quevedo-Diaz Marco 
Published: July 2020

Abstract
Background
Rickettsialpox is a febrile illness caused by the mite-borne pathogen Rickettsia akari. Several cases of this disease are reported worldwide annually. Nevertheless, the relationship between the immunogenicity of R. akari and disease development is still poorly understood. Thus, misdiagnosis is frequent. Our study is aiming to identify immunogenic proteins that may improve disease recognition and enhance subsequent treatment. To achieve this goal, two proteomics methodologies were applied, followed by immunoblot confirmation.

Results
Three hundred and sixteen unique proteins were identified in the whole-cell extract of R. akari. The most represented protein groups were found to be those involved in translation, post-translational modifications, energy production, and cell wall development. A significant number of proteins belonged to amino acid transport and intracellular trafficking. Also, some proteins affecting the virulence were detected. In silico analysis of membrane enriched proteins revealed 25 putative outer membrane proteins containing beta-barrel structure and 11 proteins having a secretion signal peptide sequence. Using rabbit and human sera, various immunoreactive proteins were identified from which the 44 kDa uncharacterized protein (A8GP63) has demonstrated a unique detection capability. It positively distinguished the sera of patients with Rickettsialpox from other rickettsiae positive human sera.

Conclusion
Our proteomic analysis certainly contributed to the lack of knowledge of R. akari pathogenesis. The result obtained may also serve as a guideline for a more accurate diagnosis of rickettsial diseases. The identified 44 kDa uncharacterized protein can be certainly used as a unique marker of rickettsialpox or as a target molecule for the development of more effective treatment.
Flow-cytometry analysis reveals persister resuscitation characteristics
Authors: Mohiuddin Sayed Golam , Kavousi Pouria , Orman Mehmet A. 
Published: July 2020

Abstract
Background
Persisters and viable but non-culturable (VBNC) cells are two phenotypic variants known to be highly tolerant to antibiotics. Although both cell types are stained as live and often appear as nongrowing during antibiotic treatment, the only distinguishing feature is the ability of persisters to recolonize in standard culture media in the absence of antibiotics. Despite considerable progress in the characterization of persister formation mechanisms, their resuscitation mechanisms remain unclear due to technical limitations in detecting and isolating these cell types in culture environments that are highly heterogeneous.

Results
In this study, we used a methodology integrating flow cytometry, fluorescent protein expression systems and ampicillin-mediated cell lysing technique to monitor persister resuscitation at the single-cell level. With this method, we were able to investigate the effects of various culture conditions (e.g., antibiotic treatment time, the length of the stationary phase in overnight pre-cultures, or pretreatment of cells with a metabolic inhibitor) on persister resuscitation. Although we observed long-term pre-cultures have many more VBNC cells compared to short-term pre-cultures, only a small fraction of non-lysed cells was able to resuscitate in all conditions tested. Regardless of pre-culturing and ampicillin treatment times, these persister cells started to resuscitate within 1 hour, after they were transferred to fresh liquid media, with the same doubling time that normal cells have. Our analysis further showed that ampicillin was not able to lyse the cells in the presence of arsenate, a metabolic inhibitor commonly used to increase bacterial persistence. However, the removal of arsenate during antibiotic treatment resulted in cell lysis and a reduction in persister levels despite the significant decrease in ATP levels in the cells.

Conclusions
The strategy presented in this study helps us monitor persister resuscitation at the single-cell level, and simultaneously quantify persister, VBNC and dead cell subpopulations in ampicillin-treated cultures. Our results indicate that the characterization of persister resuscitation with flow cytometry will enhance the current molecular-level understanding of persistence and its evolution.
Do primary and secondary care doctors have a different experience and perception of cross-level clinical coordination? Results of a cross-sectional study in the Catalan National Health System (Spain)
Authors: Esteve-Matalí Laura , Vargas Ingrid , Sánchez Elvira , Ramon Isabel , Plaja Pere , Vázquez María-Luisa 
Published: July 2020

AbstractBackgroundClinical coordination across care levels is a priority for health systems around the world, especially for those based on primary health care. The aim of this study is to analyse the degree of clinical information and clinical management coordination across healthcare levels in the Catalan national health system experienced by primary (PC) and secondary care (SC) doctors and explore the associated factors.MethodsCross-sectional study based on an online survey using the self-administered questionnaire COORDENA-CAT. Data collection: October–December 2017. Study population: PC and SC (acute and long term) doctors of the Catalan national health system. Participation rate was 21%, with a sample of 3308 doctors. Outcome variables: cross-level clinical information coordination, clinical management coordination, and perception of cross-level coordination within the area. Explanatory variables: socio-demographic, employment characteristics, attitude towards job, type of area (according to type of hospital and management), interactional factors, organizational factors and knowledge of existing coordination mechanisms. Stratification variable: level of care. Descriptive and multivariate analysis by logistic regression.ResultsThe degree of clinical coordination experienced across levels of care was high for both PC and SC doctors, although PC doctors experienced greater exchange and use of information and SC doctors experienced greater consistency of care. However, only 32.13% of PC and 35.72% of SC doctors found that patient care was coordinated across care levels within their area. In both levels of care, knowing the doctors of the other level, working in an area where the same entity manages SC and majority of PC, and holding joint clinical case conferences were factors positively associated with perceiving high levels of clinical coordination. Other associated factors were specific to the care level, such as being informed of a patient’s discharge from hospital for PC doctors, or trusting in the clinical skills of the other care level for SC doctors.ConclusionsInteractional and organizational factors are positively associated with perceiving high levels of clinical coordination. Introducing policies to enhance such factors can foster clinical coordination between different health care levels. The COORDENA questionnaire allows us to identify fields for improvement in clinical coordination.
Real-world clinical effectiveness and safety of vedolizumab and anti-tumor necrosis factor alpha treatment in ulcerative colitis and Crohn’s disease patients: a German retrospective chart review
Authors: Helwig Ulf , Mross Michael , Schubert Stefan , Hartmann Heinz , Brandes Alina , Stein Dara , Kempf Christian , Knop Jana , Campbell-Hill Sarah , Ehehalt Robert 
Published: July 2020

Abstract
Background
Real-world comparisons of biologic treatment outcomes for ulcerative colitis (UC) or Crohn’s disease (CD) patients are limited. We sought to evaluate the real-world effectiveness of vedolizumab (VDZ) and anti-tumor necrosis factor alpha (anti-TNFα) in UC and CD patients in Germany.

Methods
A retrospective chart review (15 sites) investigated UC and CD patients who were biologic-treatment naïve (biologic-naïve) or had received no more than one prior anti-TNFα before initiating treatment with VDZ or anti-TNFα between 15 July 2014 and 20 October 2015. Kaplan-Meier analyses assessed time to first chart-documented clinical remission (CR) and symptom resolution (UC: rectal bleeding [RB], stool frequency [SF]; CD: abdominal pain [AP], liquid stools [LS]) and outcome duration.

Results
A total of 133 UC (76 VDZ; 57 anti-TNFα) and 174 CD (69 VDZ; 105 anti-TNFα) patients were included. By Week 26, estimated cumulative rates of patients achieving CR or symptom resolution with VDZ vs anti-TNFα treatment were for UC: CR, 53.7% vs 31.7%; RB, 66.8% vs 55.8%; and SF, 59.8% vs 50.7%, respectively; and for CD: CR, 14.4% vs 32.8%; AP, 62.5% vs 56.0%; and LS, 29.9% vs 50.3%, respectively. Outcomes were sustained similarly between treatments, except RB (VDZ vs anti-TNFα: median 38.1 vs 15.1 weeks, P = 0.03). Treatment-related adverse events occurred in 5.3% vs 7.0% (UC) and 8.7% vs 19.0% (CD) of VDZ vs anti-TNFα patients, respectively.

Conclusions
Although there were differences in CR, symptom resolution, and safety profiles, real-world data support both VDZ and anti-TNFα as effective treatment options in UC and CD.
Client, caregiver, volunteer, and therapist views on a voluntary supported group exercise programme for older adults with dementia
Authors: Taraldsen Kristin , Boulton Elisabeth , Helbostad Jorunn L. , Saltvedt Ingvild , Granbo Randi 
Published: July 2020

Abstract
Background
Existing group exercise programmes, or other services offered to maintain physical activity levels, are typically not developed specifically for older adults with dementia. The aim of this study was to gain knowledge about perceptions of a newly developed volunteer supported group exercise programme for older adults with dementia, and any barriers that may have affected participation and compliance.

Methods
Seven (six face-to-face and one by email) interviews were conducted with (i) older adults and volunteers participating in a pilot 12-week group exercise programme, (ii) caregivers, and (iii) therapists leading the group sessions. Interview transcriptions were systemised by use of NVivo 8 and analysed by use of Systematic Text Condensation method.

Results
The theme “building relationships” represents the reason why attending this group was important for the participants. The findings suggest that how we organize exercise groups is important, with some sort of extra support, to ensure that persons will begin and continue to participate in new activities outside their homes.

Conclusions
This study showed that it is possible to involve home-dwelling persons with cognitive decline and dementia in group exercise sessions. The role of building relationships was the major factor for successful participation. Providing support and ensuring motivation for persons attending the group outside their homes was essential, both for them and their caregivers. Service providers should not underestimate the importance of building relationships between persons involved in service offers.
Association analysis of polymorphisms rs12997 in ACVR1 and rs1043784 in BMP6 genes involved in bone morphogenic protein signaling pathway in primary angle-closure and pseudoexfoliation glaucoma patients of Saudi origin
Authors: Kondkar Altaf A. , Sultan Tahira , Azad Taif A. , Osman Essam A. , Almobarak Faisal A. , Al-Obeidan Saleh A. 
Published: July 2020

Abstract
Background
Glaucoma is a polygenic neurodegenerative disease and the second most common cause of blindness in Saudi Arabia. To test the hypothesis that genetic variants in the genes involved in the bone morphogenic protein (BMP) signaling pathway may be associated with glaucoma, we investigated the association between 3′ untranslated region variants, rs12997 in ACVR1 and rs1043784 in BMP6, and primary angle-closure glaucoma (PACG) and pseudoexfoliation glaucoma (PXG).

Methods
In a case-control study, TaqMan® real-time PCR-based genotyping was done in 444 subjects consisting of 250 controls, 101 PACG and 95 PXG cases, and tested for genetic association with glaucoma-types and other clinical phenotypes.

Results
Rs12997[G] allele in ACVR1 exhibited significant 2-fold increased risk of PACG (p = 0.005) in women but not in men. Similarly, genotype analysis also showed that subjects carrying rs12997[G/G] genotype were at > 2-fold risk of PACG that remained significant after adjustment for age, sex, and Bonferroni correction in the recessive model. Furthermore, this effect was also significant in women only. In PXG, the rs12997[G/G] genotype showed a significant trend towards increased risk of the disease (OR = 2.04, 95% CI = 0.99–4.18, p = 0.049) but did not survive the Bonferroni correction. Regression analysis showed that rs12997[G/G] genotype was a significant predictor of PACG independent of age, sex, and rs1043784 genotypes. Likewise, age and rs12997[G/G] genotype showed significant effect on PXG outcome. The rs12997[A/G] genotype showed significant association with cup/disc ratio as compared to wild-type (p = 0.005) in PXG. Genotype and allele frequencies of rs1043784 in BMP6 did not show any significant association either with PACG or PXG.

Conclusions
Our results suggest that the polymorphism rs12997 in the ACVR1 gene involved in the BMP signaling pathway is significantly associated with PACG and PXG in a Saudi cohort. This is the first study to associate this variant/gene with PACG and PXG. However, further studies would be needed to replicate these findings in a large population-based cohort.