bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and genetic features of congenital dyserythropoietic anemia (CDA)
Authors: Moreno‐Carralero María‐Isabel , Horta‐Herrera Saul , Morado‐Arias Marta , Ricard‐Andrés María‐Pilar , Lemes‐Castellano Angelina , Abio‐Calvete Mariola , Cedena‐Romero María‐Teresa , González‐Fernández Fernando‐Ataulfo , Llorente‐González Laura , Periago‐Peralta Adela‐María , de‐la‐Iglesia‐Íñigo Silvia , Méndez Manuel , Morán‐Jiménez María‐José 
Published: June 2018

AbstractIntroductionCongenital dyserythropoietic anemias (CDA) are characterized by hyporegenerative anemia with inadequate reticulocyte values, ineffective erythropoiesis, and hemolysis. Distinctive morphology of bone marrow erythroblasts and identification of causative genes allow classification into 4 types caused by variants in CDAN1, c15orf41, SEC23B, KIF23, and KLF1 genes.ObjectiveIdentify pathogenic variants in CDA patients.MethodsMassive parallel sequencing with a targeted gene panel, Sanger sequencing, Comparative Genome Hybridization (CGH), and in silico predictive analysis of pathogenicity.ResultsPathogenic variants were found in 21 of 53 patients studied from 44 unrelated families. Six variants were found in CDAN1: two reported, p.Arg714Trp and p.Arg725Trp and, four novel, p.Arg623Trp, p.Arg946Trp, p.Phe1125Ser and p.Ser1227Gly. Twelve variants were found in SEC23B: seven reported, p.Arg14Trp, p.Glu109Lys, p.Arg217Ter, c.835‐2A>G, p.Arg535Ter, p.Arg550Ter and p.Arg718Ter and, five novel, p.Val164Leu, p.Arg190Gln, p.Gln521Ter, p.Arg546Trp, and p.Arg611Gln. The variant p.Glu325Lys in KLF1 was found in one patient and p.Tyr365Cys in ALAS2 in an other. Moreover, we identified genomic rearrangements by CGH in some SEC23B‐monoallelic patients.ConclusionsNew technologies for genetic studies will help to find variants in other genes, in addition to those known, that contribute to or modulate the CDA phenotype or support the correct diagnosis.
Effect of modified Atkins diet in adults with drug‐resistant focal epilepsy: A randomized clinical trial
Authors: Kverneland Magnhild , Molteberg Ellen , Iversen Per O. , Veierød Marit B. , Taubøll Erik , Selmer Kaja K. , Nakken Karl O. 
Published: June 2018

SummaryObjectiveKetogenic diets reduce seizures in children with drug‐resistant epilepsy. Whether adults benefit from similar treatment has not been clarified. We therefore examined the efficacy of the modified Atkins diet in adults with drug‐resistant focal epilepsy.MethodsWe performed a randomized clinical trial (RCT) with patients >16 years who had at least 3 seizures per month despite having tried at least 3 antiepileptic drugs. They were randomized to either 12 weeks on the modified Atkins diet (diet group) or habitual diet (control group). Primary endpoint was a change in seizure frequency from baseline to the intervention period, comparing those on diet with controls.ResultsWe assigned 37 patients to the diet group and 38 to the control group. Nine of the patients in the diet group and 4 controls were excluded. Of those who completed the dietary intervention (n = 24), median seizure change was −1.0 (interquartile range [IQR] −13.7‐8.8), while in the control group (n = 32) the median change was 4.5 (IQR −4.8‐33.5). The median difference between the groups was −7.0 (95% confidence interval [CI] −37.0‐3.0; P = .21). In the intention‐to‐treat analysis, the relative risk (RR) for achieving >50% seizure reduction was 1.8 (95% CI 0.3‐10.2; P = .65), while for achieving >25% seizure reduction RR was 2.43 (95% CI 0.94‐6.28; P = .06). We observed no serious adverse events.SignificanceIn this RCT investigating the effect of an adjunctive modified Atkins diet on seizure frequency in adults with difficult‐to‐treat focal epilepsy, we found a significant reduction in seizure frequency in the diet group compared to the controls, but only for moderate benefit (>25% seizure reduction) among those who completed the intervention. However, seizure response varied considerably between individuals, perhaps negatively influenced by a drop in serum concentrations of antiepileptic drugs.
Traits drive global wood decomposition rates more than climate
Authors: Hu Zhenhong , Michaletz Sean T. , Johnson Daniel J. , McDowell Nate G. , Huang Zhiqun , Zhou Xuhui , Xu Chonggang 
Published: June 2018

AbstractWood decomposition is a major component of the global carbon cycle. Decomposition rates vary across climate gradients, which is thought to reflect the effects of temperature and moisture on the metabolic kinetics of decomposers. However, decomposition rates also vary with wood traits, which may reflect the influence of stoichiometry on decomposer metabolism as well as geometry relating the surface areas that decomposers colonize with the volumes they consume. In this paper, we combined metabolic and geometric scaling theories to formalize hypotheses regarding the drivers of wood decomposition rates, and assessed these hypotheses using a global compilation of data on climate, wood traits, and wood decomposition rates. Our results are consistent with predictions from both metabolic and geometric scaling theories. Approximately half of the global variation in decomposition rates was explained by wood traits (nitrogen content and diameter), whereas only a fifth was explained by climate variables (air temperature, precipitation, and relative humidity). These results indicate that global variation in wood decomposition rates is best explained by stoichiometric and geometric wood traits. Our findings suggest that inclusion of wood traits in global carbon cycle models can improve predictions of carbon fluxes from wood decomposition.
N‐terminal dual lipidation‐coupled molecular targeting into the primary cilium
Authors: Kumeta Masahiro , Panina Yulia , Yamazaki Hiroya , Takeyasu Kunio , Yoshimura Shige H. 
Published: June 2018

AbstractThe primary cilium functions as an “antenna” for cell signaling, studded with characteristic transmembrane receptors and soluble protein factors, raised above the cell surface. In contrast to the transmembrane proteins, targeting mechanisms of nontransmembrane ciliary proteins are poorly understood. We focused on a pathogenic mutation that abolishes ciliary localization of retinitis pigmentosa 2 protein and revealed a dual acylation‐dependent ciliary targeting pathway. Short N‐terminal sequences which contain myristoylation and palmitoylation sites are sufficient to target a marker protein into the cilium in a palmitoylation‐dependent manner. A Golgi‐localized palmitoyltransferase DHHC‐21 was identified as the key enzyme controlling this targeting pathway. Rapid turnover of the targeted protein was ensured by cholesterol‐dependent membrane fluidity, which balances highly and less‐mobile populations of the molecules within the cilium. This targeting signal was found in a set of signal transduction molecules, suggesting a general role of this pathway in proper ciliary organization, and dysfunction in ciliary disorders.
Low bone mineral density and associated factors in patients with haemophilia in Colombia
Authors: Sossa Melo C. L. , Wandurraga E. A. , Peña A. M. , Jiménez S. I. , Salazar L. A. , Ochoa M. E. , Luna‐Gonzalez M. L. , Ortiz M. L. , Morales K. , Ayala‐Castillo M. , Reyes D. L. , Chalela C. M. 
Published: June 2018

IntroductionPatients with haemophilia may have lower levels of bone mineral density (BMD) compared with the general population. Moreover, haemophilic patients have increased risk factors for low bone mineral density (LBMD) such as arthropathy and resulting immobility, increasing their risk for osteoporosis and fractures.AimTo assess the prevalence of LBMD and associated risk factors among a group of Colombian haemophilic patients.MethodsIn this case‐control study, 90 patients with haemophilia A and B, over the age of five, were recruited. Controls were healthy participants matched by age, gender, body mass index (BMI), socioeconomic status, and race. All participants underwent dual energy X‐ray absorptiometry (DXA) and the Global Physical Activity Questionnaire. Blood tests were collected to evaluate LBMD determinants in cases.ResultsBMD was lower in cases than in the control group. BMD of femoral necks was 0.907 g/cm2 in cases vs. 1.020 g/cm2 in controls (P = .019), and BMD of hips 0.930 g/cm2 in cases vs. 1030 g/cm2 in controls (P = .019). The greater the severity of haemophilia, the lower BMD in spine, femoral neck, and hips. Elevated C‐protein levels were found in 44.1% of patients with LBMD and 14.8% with normal BMD (P = .003). The study found an adjusted prevalence ratio of 2.11, indicating that haemophilic patients are two times more likely to have LBMD (CI95% = 1.43‐3.11 P < .001).ConclusionResults from the present study showed that haemophilia was associated with a higher frequency of LBMD. Severity of haemophilia, haemophilic arthropathy, and elevated C‐reactive protein levels was directly associated with LBMD.
One generalist or several specialist species? Wide host range and diverse manipulations of the hosts’ web‐building behaviour in the true spider parasitoidZatypota kauros(Hymenoptera: Ichneumonidae)
Authors: Korenko Stanislav , Spasojevic Tamara , Pekár Stano , Walter Gimme H. , Korenková Vlasta , Hamouzová Kateřina , Kolářová Michaela , Kysilková Kristýna , Klopfstein Seraina 
Published: June 2018

AbstractParasitoid wasps of thePolysphinctagenus‐group are highly specialised on their spider hosts, and most of them are known to manipulate their hosts into building a special web in which the parasitoid pupates. Trophic niche and the plasticity of host use were investigated in the koinobiont parasitoidZatypota kaurosGauld from Queensland, Australia.We found thatZ. kaurosattacks spider hosts from different families, each differing widely in their web‐building behaviours, which makes it unique in the breadth of its host range. Molecular analyses revealed that the taxonZ. kauroscontains three divergent mitochondrial lineages. Lineage A was associated exclusively with spiders of the genusAnelosimus(Theridiidae), which builds tangle webs; lineage B was associated with the genusCyrtophora(Araneidae), which weaves tent webs; and lineage C was associated with a broad range of hosts, including spiders of both the families Araneidae and Theridiidae. Unique host manipulations could be observed in the web‐building behaviours of the different host groups. Nevertheless, nuclear data from two ribosomal genes and three introns did not add any support to the existence of different evolutionary lineages, nor did they coincide with the different host groups.The partial correspondence of mitochondrial lineage and host use, together with an apparent mito‐nuclear conflict might indicate maternal effects or very recent and/or incomplete speciation in this taxon. Given their wide host range and intriguing interactions with their hosts, theZ. kauroscomplex represents a promising system for studying parasitoid specialisation and its potential impact on speciation.
3D printed replicas for endodontic education
Authors: Reymus M. , Fotiadou C. , Kessler A. , Heck K. , Hickel R. , Diegritz C. 
Published: June 2018

AbstractAimTo assess the feasibility of producing artificial teeth for endodontic training using 3D printing technology, to analyse the accuracy of the printing process, and to evaluate the teeth by students when used during training.MethodologySound extracted human teeth were selected, digitalized by cone beam computed tomography (CBCT) and appropriate software and finally reproduced by a stereolithographic printer. The printed teeth were scanned and compared with the original ones (trueness) and to one another (precision). Undergraduate dental students in the third and fourth years performed root canal treatment on printed molars and were subsequently asked to evaluate their experience with these compared to real teeth.ResultsThe workflow was feasible for manufacturing 3D printed tooth replicas. The absolute deviation after printing (trueness) ranged from 50.9 to 104.3 μm. The values for precision ranged from 43.5 to 68.2 μm. Students reported great benefits in the use of the replicated teeth for training purposes.ConclusionThe presented workflow is feasible for any dental educational institution who has access to a CBCT unit and a stereolithographic printer. The accuracy of the printing process is suitable for the production of tooth replicas for endodontic training. Undergraduate students favoured the availability of these replicas and the fairness they ensured in training due to standardization.
Turkey's Education Policies towards Syrian Refugees: A Macro‐level Analysis
Authors: Unutulmaz K. Onur 
Published: June 2018

AbstractThis study discusses the transformation of Turkey's education policies towards Syrian refugees in three major stages. It argues that education policies in these different stages reflect the general perception of and political vision for Syrian refugees by the Turkish state, and that they are also instruments through which this political vision is materialized and declared. The remarkable evolution of Turkey's education policies towards Syrian refugees, from early policies that aimed at temporary accommodation to later policies that have aimed at full integration, needs to be understood in this framework of a changing vision in addition to the security concerns. Lastly, the article argues that, while the current strategy of complete integration of Syrians into formal education system is certainly positive, it also brings about a number of significant challenges related to the political and legal context in Turkey that will have to be handled in its realization.
Facile high‐performance film thermocouple made of strontium lanthanum chromate for temperature sensing in air
Authors: Liu Dan , Shi Peng , Ren Wei , Liu Yantao , Liu Ming , Niu Gang , Lin Qijing , Tian Bian , Jiang Zhuangde , Ye Zuo‐Guang 
Published: June 2018

AbstractFilm thermocouples as the most effective tool are used to monitor the real‐time and accurate in situ temperature in harsh environment, which will demand their electrodes with high thermal‐resistant properties, high sensitivity, and stability. In this work, a series of solid solutions based on strontium lanthanum chromate were systematically investigated as new electrodes. LaCrO3 and La0.8Sr0.2CrO3 with some glass additives were chosen to fabricate high‐performance thick‐film thermocouple due to a bigger Fermi level difference between them. The thermocouple shows a high sensitivity (107.8 μV/°C) during static calibration, and an excellent reproducibility and stability during multicycle calibrations carried out up to 1550°C, resulting in a record ultra‐high temperature sensing in film thermocouples. This facile thermocouple may open up a new way to measure high temperature in air or oxidation atmosphere accurately.
First report of the fall armyworm, Spodoptera frugiperda (Lepidoptera: Noctuidae), natural enemies from Africa
Authors: Sisay Birhanu , Simiyu Josephine , Malusi Peter , Likhayo Paddy , Mendesil Esayas , Elibariki Nsami , Wakgari Mulatu , Ayalew Gashawbeza , Tefera Tadele 
Published: June 2018

AbstractThe fall armyworm (FAW), Spodoptera frugiperda, is a major pest of maize in North and South America. It was first reported from Africa in 2016 and currently established as a major invasive pest of maize. A survey was conducted to explore for natural enemies of the fall armyworm in Ethiopia, Kenya and Tanzania in 2017. Smallholder maize farms were randomly selected and surveyed in the three countries. Five different species of parasitoids were recovered from fall armyworm eggs and larvae, including four within the Hymenoptera and one Dipteran. These species are new associations with FAW and were never reported before from Africa, North and South America. In Ethiopia, Cotesia icipe was the dominant larval parasitoid with parasitism ranging from 33.8% to 45.3%, while in Kenya, the tachinid fly, Palexorista zonata, was the primary parasitoid with 12.5% parasitism. Charops ater and Coccygidium luteum were the most common parasitoids in Kenya and Tanzania with parasitism ranging from 6 to 12%, and 4 to 8.3%, respectively. Although fall armyworm has rapidly spread throughout these three countries, we were encouraged to see a reasonable level of biological control in place. This study is of paramount importance in designing a biological control program for fall armyworm, either through conservation of native natural enemies or augmentative release.
COPF: Citrus orchard perimeter fencing as a strategy for reducing Asian citrus psyllid (Hemiptera:Liviidae) infestation
Authors: Sétamou Mamoudou , Alabi Olufemi J. , Tofangsazi Nastaran , Grafton‐Cardwell Elizabeth 
Published: June 2018

AbstractCitrus grove colonization by the Asian citrus psyllid (Diaphorina citri) starts from border trees. This behaviour can be exploited by placing barriers along grove borders to prevent psyllid incursion and thwart population establishment and growth. We tested the effects of psyllid‐resistant mesh fencing along grove borders for season‐long control of D. citri populations. Deployment of sticky cards at different heights revealed that most D. citri (>99%) were collected on traps placed at or below 3.7 m along grove border. Thus, psyllid‐resistant mesh fencing (3.7 m in height) was installed along the eastern and southern borders of a 3‐year‐old grapefruit grove to protect against D. citri infestations, while the west and north edges were left unprotected. A similar adjacent grove with no border fencing served as untreated control. A reduction of 55%–98% of D. citri numbers was observed in the grove with border fences relative to the untreated control. D. citri density reductions were more pronounced on the sides with border fences suggesting that the observed effects were due to the mesh fencing. Thus, border mesh fencing as a standalone approach or coupled with other management strategies, such as border sprays, could be an effective psyllid management strategy. The season‐long benefits of border fences are not limited to D. citri population control, but also significant reductions of insecticide input and nontarget effects, thus substantially mitigating the risks of secondary pest outbreaks and ensuring the sustainability of D. citri management in citrus groves.
Pediatric Elective Diagnostic Procedure Complications: A Multicenter Cohort Analysis
Authors: Attard Thomas M , Miller Mikaela , Lee Brian , Champion Thomas W , Thomson Mike 
Published: June 2018

AbstractBackground and AimIncreased access to endoscopic procedures have entrenched these investigative tools in routine pediatric gastroenterology practice. Patient outcomes following endoscopy therefore are topical in the decision toward endoscopy. We studied the likelihood and patient characteristics of children admitted following ambulatory endoscopy.MethodsHospitalization data were obtained from the Pediatric Hospital Information System including 49 tertiary children's hospitals in the USA. Children who underwent ambulatory diagnostic endoscopy between October 1, 2005 and September 25, 2015 were included. The primary outcomes were post‐procedure events resulting in unplanned admission (not for inflammatory bowel disease management) or emergency room visit within 5 days. Unadjusted, univariate analyses were followed by multivariable analysis of the associations between patient characteristics and outcome using the R statistical package, v. 3.2.3.ResultsDuring the study period, 217 817 patients underwent diagnostic endoscopy; 101 (0.05%) patients were admitted directly; 1314 (0.60%) were admitted to the same facility's emergency department with either a respiratory or a gastrointestinal complication as a primary diagnosis within 5 days. None of the procedures resulted in death; female patients were more likely to experience adverse outcomes (P < 0.001), as were patients from an urban setting (P = 0.0004), whereas White, non‐Hispanic patients were less likely to represent (P < 0.0001). Patients with chronic comorbidities were more likely to experience complications. The most frequent diagnoses at admission were abdominal pain (30.5%), other gastroenterologic processes (26.8%), respiratory disorders (17.1%), gastrointestinal hemorrhage (8.3%), and fever (4.5%).ConclusionsAmbulatory pediatric endoscopy is safe; significant adverse outcomes are rare but more likely in female, non‐White or Hispanic patients and in patients with significant chronic comorbidities.
Varices on computed tomography are surrogate of clinically significant portal hypertension and can predict survival in compensated cirrhosis patients
Authors: Lee Dong Ho , Ahn Jhii‐Hyun , Chung Jin Wook , Kim Young Ju , Cha Seung‐Whan , Kim Moon Young , Baik Soon Koo 
Published: June 2018

AbstractBackground and AimTo investigate prognostic value of varices on computed tomography (CT) and redefine surrogate criteria for clinically significant portal hypertension (CSPH).MethodsWe retrospectively enrolled 241 patients with compensated cirrhosis who underwent hepatic venous pressure gradient (HVPG) measurement from 2008 to 2013. Using CT and upper endoscopy findings obtained within 3 months from HVPG measurement, patients were classified into three groups: presence of standard surrogate for CSPH, defined as presence of varices on upper endoscopy and/or splenomegaly associated with thrombocytopenia (Group 1, n = 139); varices on CT without standard surrogate for CSPH (Group 2, n = 41); and free from both (Group 3, n = 61). HVPG value and overall survival (OS) rates were compared among three patient groups. Revised surrogate for CSPH was defined as presence of standard surrogate and/or presence of varices on CT (i.e. both Group 1 and Group 2).ResultsMean HVPG value in Group 2 was significantly higher than that in Group 3 (10.3 mmHg vs 6.5 mmHg, P < 0.001), but significantly lower than that in Group 1 (10.3 mmHg vs 13.1 mmHg, P < 0.001). Seven‐year OS rates in Group 2 was similar to those in Group 1 (57.0% vs 62.7%, P = 0.591), but significantly poorer than those in Group 3 (57.0% vs 84.0%, P = 0.015). The presence of revised surrogate for CSPH was a significant predictive factor for OS (P = 0.025, Hazard ratio = 2.71 [1.14–6.45]) on multivariate analysis whereas standard surrogate for CSPH was not (P = 0.849).ConclusionThe presence of varices on CT was a significant sign for CSPH, predicting poor OS outcome in patients with compensated cirrhosis.