bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long‐term outcome of vein of Galen malformation
Authors: Taffin Hélène , Maurey Hélène , Ozanne Augustin , Durand Philippe , Husson Béatrice , Knebel Jean‐François , Adamsbaum Catherine , Deiva Kumaran , Saliou Guillaume 
Published: November 2019

AimTo describe the long‐term outcomes of children by the time they reached school age with vein of Galen aneurysmal malformation (VGAM).MethodThis was a retrospective observational study on a consecutive cohort of patients with VGAM. We included patients with at least one Francophone parent, aged between 6 and 11 years at the time of long‐term evaluation. The neurological outcome was assessed with the King’s Outcome Scale for Childhood Injury score and eight neurological and behavioural items from the Rivermead Postconcussion Symptoms questionnaire.ResultsAll 52 patients (17 females, 32 males [data missing for n=3]) with at least one Francophone parent (5 fetuses and 47 children) were included. At the long‐term evaluation time‐point, 33 patients were alive and 19 patients had died. Risk of postnatal death was associated with severe neonatal cardiac failure (p=0.007) or isosystemic or suprasystemic pulmonary hypertension (p=0.014). Among survivors, 19 had a good outcome with normal schooling and 14 had a poor outcome. Moreover, among the good outcome patients, a large proportion had neurodevelopmental alterations.InterpretationLong‐term outcome of patients with VGAM appears to be less favourable than outcome described at the short‐ and medium‐term, even in the absence of encephalomalacia at birth. Even patients with good outcome often have neuropsychological disorders that may have repercussions on learning and requiring appropriate rehabilitation or medical management.What this paper adds

Long‐term outcome appears to be less favourable than described at short‐ and medium‐term follow‐up.

Even patients with good outcome at these time‐points often have minor neuropsychological disorders.
Treatment of refractory and superrefractory status epilepticus with topiramate: A cohort study of 106 patients and a review of the literature
Authors: Fechner Anne , Hubert Kristina , Jahnke Kolja , Knake Susanne , Konczalla Jürgen , Menzler Katja , Ronellenfitsch Michael W. , Rosenow Felix , Strzelczyk Adam 
Published: November 2019

AbstractObjectiveNovel treatments are needed to control treatment‐resistant status epilepticus (SE). We present a summary of clinical cases where oral topiramate (TPM) was used in refractory SE (RSE) and superrefractory SE (SRSE).MethodsA review of medical records was carried out to detect TPM administration in SE patients treated in Frankfurt and Marburg between 2011 and 2016. The primary outcome question concerned SE resolution after TPM initiation.ResultsIn total, TPM was used in 106 of 854 patients having a mean age of 67.4 ± 18.1 years, 61 of whom were female (57.5%). The median latency from SE onset to TPM initiation was 8.5 days. Patients with SE had previously failed a median of five other antiepileptic drugs. The median initial TPM dose was 100 mg/d, which was uptitrated to a median maintenance dose of 400 mg/d. Treatment with TPM was continued for a median time of 12 days. TPM was the last drug provided to 42 of 106 (39.6%) patients, with a resultant response attributed to TPM observed in 29 of 106 (27.4%) patients. A response was attributed to TPM in 21 (31.8%) of 66 RSE cases and eight (20%) of 40 SRSE cases. Treatment‐emergent adverse events were attributed to TPM usage in two patients, one each with pancreatitis and hyperchloremic acidosis, and in 38 patients (35.8%), hyperammonemia was seen. Thirty‐four of these patients received a combination of TPM and valproate and/or phenobarbital. The intrahospital mortality rate was 22.6% (n = 24).SignificanceThe rate of SE cessation attributed to TPM treatment (27.4%) represents a relevant response given the late treatment position of TPM and the treatment latency of more than 8 days. Based on these results and in line with the findings of other case series, TPM can be considered an alternative option for treating RSE and SRSE.
Direct regulation of Chk1 protein stability by E3 ubiquitin ligase HUWE1
Authors: Cassidy Katelyn B. , Bang Scott , Kurokawa Manabu , Gerber Scott A. 
Published: November 2019

The HECT E3 ubiquitin ligase HUWE1 is required for a wide array of important functions in cell biology. Although HUWE1 is known to play a role in DNA damage signaling, the mechanism(s) that underlie this function remain elusive. HUWE1 regulates effectors of DNA replication and genotoxic stress tolerance. However, the loss of HUWE1 can also result in the accrual of significant endogenous DNA damage due to insufficient remediation of replication stress induced by an overabundance of key substrates. We discovered that HUWE1 depletion leads to a significant increase in levels of the single‐strand break effector kinase Chk1, independent of the DNA damage response, activation of apical DNA damage repair (DDR) signaling kinases (ATM and ATR), and the tumor suppressor p53. We also identified multiple lysine residues on Chk1 that are polyubiquitinated by HUWE1 in vitro, many of which are within the kinase domain. HUWE1 knockdown also markedly prolonged the protein half‐life of Chk1 in steady‐state conditions and resulted in greater stabilization of Chk1 protein than depletion of Cul4A, an E3 ubiquitin ligase previously described to control Chk1 abundance. Moreover, prolonged replication stress induced by hydroxyurea or camptothecin resulted in a reduction of Chk1 protein levels, which was rescued by HUWE1 knockdown. Our study indicates that HUWE1 plays a significant role in the regulation of the DDR signaling pathway by directly modulating the abundance of Chk1 protein.
A global test of the subsidized island biogeography hypothesis
Authors: Menegotto André , Rangel Thiago Fernando , Schrader Julian , Weigelt Patrick , Kreft Holger 
Published: November 2019

AbstractAimThe decreasing capacity of area to predict species richness on small islands (the small‐island effect; SIE) seems to be one of the few exceptions of the species–area relationship. While most studies have focused on how to detect the SIE, the underlying ecological factors determining this pattern remain largely unexplored. Here, we evaluate one of the few mechanisms proposed to explain the SIE, the subsidized island biogeography hypothesis, which posits that marine productivity around small islands may alter their expected species richness.LocationSeven hundred and ninety islands worldwide, including 420 islands < 1 km2.Time periodPresent.Major taxa studiedAngiosperms.MethodsWe applied iterative partial regression to determine the effects of island area and marine productivity on plant species richness for islands of varying sizes. We also employed geographically weighted regression to account for non‐stationarity in the marine productivity effects. Lastly, we used estimates of ammonia emissions based on nutrient excretion by seabird colonies from a subset of 66 islands to evaluate the effects of marine resources deposition on angiosperm species richness.ResultsWe found no effect of marine productivity on insular species richness, at both regional and global scales. In all models, area emerged as the only predictor of plant species richness. A weak contribution of marine productivity was only detectable in models with a low number of islands, but this effect was independent of island size. Although nutrient deposition significantly contributes to explaining plant diversity, this effect was also independent of island size.Main conclusionsOur study demonstrates that marine productivity has no general effect on plant species richness of small islands worldwide. Although marine‐derived resources may still contribute to species richness variation, this effect does not seem to be restricted to small islands. Overall, our results do not provide support for the subsidized island biogeography hypothesis.
Role of strontium substitution in spray drying of hydroxyapatite: A comparative study on physical properties
Authors: Bastan Fatih E. , Erdogan Garip , Ustel Fatih 
Published: November 2019

AbstractSpray drying (SD) has the ability to tailor granule size and morphology, thus, it is used for producing hydroxyapatite (HA) granules. Recently, Sr functionalized HA (SrHA) has been recommended owing to its improved biological properties. The aim of this article was to produce SrHA granules with the combination of nanoparticle synthesis and spray‐drying. The effects of substitution on the physical properties of SrHA nanoparticles and spray‐dried granules were investigated. TEM analyzes revealed that Sr substitution reduced the mean size of HA nanoparticles from 4.59 nm to 2.31 nm. Besides, Sr substitution increased the viscosity of the prepared slurry for spray drying, which may be attributed to the smaller nanoparticle sizes. The reduced nanoparticle size caused to the agglomeration of the SrHA more than the pure HA nanoparticles. Moreover the 16 mol % Sr substituted HA (16SrHA) slurry were quickly hardened, which hampered the feeding of the slurry to the SD system; eventually the atomizer was blocked. The increase in the viscosity increased the mean granule size of HA from 41.53 µm to 49.18 µm. HA and 8SrHA granules presented HA phase dominantly after the heat treatment at 1000°C, while, 16SrHA decomposed to TCP according to RAMAN and XRD investigations.
Systematic review and narrative synthesis of pharmacist provided medicines optimisation services in care homes for older people to inform the development of a generic training or accreditation process
Authors: Wright David John , Maskrey Vivienne , Blyth Annie , Norris Nigel , Alldred David P , Bond Christine M , Desborough James , Hughes Carmel M , Holland Richard Charles 
Published: November 2019

AbstractObjectivesTo develop a training programme to enable pharmacists with prescribing rights to assume responsibility for the provision of pharmaceutical care within care homes, a systematic review and narrative synthesis was undertaken to identify reported approaches to training pharmacists and use this literature to identify potential knowledge requirements.MethodsA PROSPERO-registered systematic review was performed using key search terms for care homes, pharmacist, education, training and pharmaceutical care. Papers reporting primary research focussed on care of the older person within the care home setting were included. No restrictions were placed on methodology. Two researchers independently reviewed titles, abstracts and papers. Agreement on inclusion was reached through consensus. Data on titles, training and activities undertaken were extracted and knowledge requirements identified. Findings were synthesised and reported narratively.Key findingsFifty-nine papers were included, most of which were uncontrolled service evaluations. Four papers reported an accreditation process for the pharmacist. Thirteen papers reported providing tools or specific training on a single topic to pharmacists. The main clinical and therapeutic areas of activity (requiring codified knowledge) were dementia, pain, antipsychotic and cardiovascular medication. Provision of pharmaceutical care, effective multidisciplinary working and care home staff training represented the main areas of practical knowledge.ConclusionsInformation regarding training and accreditation processes for care home pharmacists is limited. This study provides insight into potential codified and practical knowledge requirements for pharmacists assuming responsibility for the provision of pharmaceutical care within care homes. Further work involving stakeholders is required to identify the cultural knowledge requirements and to develop a training and accreditation process.
Assessment of agreement and time in therapeutic range of capillary versus venous international normalised ratio in frail elderly people in a nursing home
Authors: Sikirdji Chloé , Costa David , Alonso Sandrine , Clape Jean‐François , Amouyal Michel , de Waziere Benoît , Fabbro‐Peray Pascale 
Published: November 2019

AbstractVitamin K antagonists are widely used, yet have a slim therapeutic margin and high iatrogenicity. Patients are monitored through international normalised ratio (INR) by venipuncture, but coagulometers could measure INR by capillary puncture. This prospective study evaluated the clinical concordance of capillary INR versus venous INR in 31 nursing home patients. Concordance was good and mean time in therapeutic range (TTR) markedly increased. Capillary INR is thus reliable, could improve TTR and decrease iatrogenicity.
Are external management consultancies effective in healthcare improvement, do they reflect value for money and what are the alternative models?
Authors: Skouteris Helen , Kirkpatrick Ian , Currie Graeme , Braithwaite Jeffrey , Teede Helena 
Published: November 2019

AbstractDespite the increasing use and costs associated with external management consultancy for healthcare improvement, there is a paucity of formal evaluations examining impact. This paper aims to: (i) discuss the potential benefits and disadvantages of external consultancies in addressing complex healthcare challenges and delivering healthcare improvement in Australia; and (ii) explore potential alternative models, including internal consultancy and hybrid models delivered through platforms of collaborative expertise. We propose that the substantive reliance on high cost external management consultancies without demonstrating value or benefit, is unsustainable. An integrative approach that embeds research and capacity building within healthcare services may be of value.
The significance of anti‐granulocyte‐macrophage colony‐stimulating factor antibodies in cryptococcal infection: case series and review of antibody testing
Authors: Stevenson Brittany , Bundell Christine , Mulrennan Siobhain , McLean‐Tooke Andrew , Murray Ronan , Brusch Anna 
Published: November 2019

AbstractWe report two cases of cryptococcosis, associated with anti‐granulocyte‐macrophage colony‐stimulating factor antibodies. We review this recently identified acquired form of autoimmune immune deficiency and discuss the potential applications of granulocyte‐macrophage colony‐stimulating factor antibody testing by enzyme‐linked immunosorbent assay.
Translation, cross‐cultural adaptation and validation of the “Cardiff wound impact schedule,” a wound‐specific quality of life instrument, to the native Spanish of Mexican patients
Authors: Lozano‐Platonoff Adriana , Contreras‐Ruiz Jose , Dominguez‐Cherit Judith , Cardenas‐Sanchez Andrea , Alvarez‐Rivero Valeria , Martínez‐Regalado Joel A. 
Published: November 2019

AbstractThe aim of this study was to translate into Mexican Spanish, cross‐culturally adapt and validate the wound‐specific quality of life (QoL) instrument Cardiff wound impact schedule (CWIS) for Mexican patients. This instrument went through the full linguistic translation process based on the guidelines of Beaton et al (Beaton DE, Bombardier C, Guillemin F, Ferraz MB, Guidelines for the process of cross‐cultural adaptation of self‐report measures, Spine Phila Pa, 1976, 2000, 318‐391). We included a total of 500 patients with chronic leg ulcers. The expert committee evaluated the Face validity and they agreed unanimously that the instrument was adequate to assess the QoL of these patients, covering all relevant areas presented by them. The content validity index obtained was of 0.95. The construct validity demonstrated moderately significant correlations between related sub‐scales of CWIS and SF‐36 (P = .010 to P < .001). The instrument was able to discriminate between healed and unhealed ulcers. The instrument obtained an overall Cronbach's alpha of .952, corresponding to an excellent internal consistency (.903‐.771 alpha range for domains). The CWIS can be appropriately used to assess the health‐related QoL of Mexican patients with chronic leg ulcers.
Atmospheric humidity and genotype are key determinants of the diurnal stomatal conductance pattern
Authors: Zhang Xinying , Wang Yajing , Huang Guirong , Feng Fu , Liu Xiaoying , Guo Rui , Gu Fengxue , Hu Xin , Yang Ziguang , Zhong Xiuli , Mei Xurong 
Published: November 2019

AbstractGreat understanding of the genotypic difference in diurnal stomatal conductance (gs) pattern and the key determinants of the pattern is important for saving water by adopting cultivars appropriately. Fifteen wheat genotypes were studied under different soil conditions and various meteorological conditions with pot cultivation in rain shelter for two years. Genotype and air humidity were found to be key determinants of diurnal gs pattern. All genotypes under low relative humidity (LRH) and most genotypes under high relative humidity (HRH) displayed a gradual decline pattern from morning through the afternoon. Under moderate relative humidity (MRH), all genotypes present a single‐peak curve pattern, but they differed in peak time, which may lead to unreliable gs comparison between genotypes and get ostensible contrasting materials. The stomatal conductance was significantly different among genotypes under LRH and the increased gs magnitude is also significantly different when it was compared between LRH and HRH. The present results provide new thinking for selecting and adopting appropriate cultivars with specific stomata traits for the area with various meteorological conditions.
The concept of effective population size loses its meaning in the context of optimal management of diversity using molecular markers
Authors: Toro Miguel A. , Villanueva Beatriz , Fernández Jesús 
Published: November 2019

AbstractEffective population size is a key parameter in conservation genetics. In the management of conservation programs using pedigree information, there is a consensus that the optimal method for maximizing effective population size is to calculate the contribution of each potential parent (the number of offspring that each individual leaves to the next generation) by minimizing the global pedigree‐based coancestry between potential parents weighted by their contributions. When using molecular data, the optimal method for managing genetic diversity will remain the same but now the molecular coancestry calculated from markers will replace the pedigree‐based coancestry. However, in this situation, the concept of effective population size loses its meaning because with optimal molecular management, genetic diversity increases in early generations and therefore effective population size takes negative values. Furthermore, in the long term, the molecular effective population size does not attain an asymptotic value but it shows an unpredictable behaviour.
Climate change and biogeographic connectivity across the Brazilian cerrado
Authors: De Oliveira Paulo E. , Raczka Marco , McMichael Crystal N. H. , Pinaya Jorge L. D. , Bush Mark B. 
Published: November 2019

AbstractAimTo investigate cerrado responses to glacial–interglacial climate change and the potential for connective rain forest corridors between the Atlantic Coastal Forest and Amazonian rain forest.LocationThe crater lake of Serra Negra (18 °S, 46 °W) in Minas Gerais, Brazil.Taxon117 fossil pollen types, 22 non‐pollen palynomorphs were documented.MethodsWe recovered 7.82 m of sediment from the lake, and analysed fossil pollen at 62 depth intervals throughout the core. We derived a chronology based on radiocarbon dating with simple rate extrapolation to the base of the core.ResultsThe c. 90,000‐year fossil record showed a trend towards cooler climates at the Last Glacial Maximum (LGM), but interstadial warming coupled with reduced evaporative stress allowed the expansion of woodlands under cool, moist conditions. Cool‐adapted trees were most abundant between c. 67,000 and 48,000 years ago. A cool cerrado‐like environment marked full glacial conditions between c. 48,000 and 34,000 years ago. The peak of the LGM between c. 34,000 and 17,000 years ago is inferred to have been dry as no sediment accumulated in the system.Main conclusionsExpanded ranges of cold‐tolerant forest taxa led to establishment of a series of assemblages without modern analogue. A system characteristic of modern cerrado was rare in the history of this site. Multiple forest expansions were observed, each differing in composition. The periods of forest abundance at Serra Negra were not temporally aligned with forest expansion in the Atlantic Coastal Forest, and did not provide a continuous corridor of similar forest that connected the cerrado to the Atlantic Coastal Forest.
The role of Persian‐language word exercise games in improving spelling of students with dyslexia: Word exercise games in improving spelling
Authors: Zare Mahbobe , Amani Malahat , Sadoughi Majid 
Published: November 2019

AbstractChildren with dyslexia have reduced sensitivity to phonological sounds and words, and this deficiency in lexical processing causes many problems for them. Word exercise games based on phonological awareness have emphasized the mistakes of students with dyslexia, attempting to help children avoid these mistakes. This study was conducted to investigate the effect of Persian‐language word exercise games on the spelling of students with dyslexia. The design of the present study was quasi‐experimental, with a pretest and posttest of an experimental group and a control group. Participants were 30 students with dyslexia from second grade to fifth grade in elementary schools in Bojnord, who completed the spelling test as a pretest and posttest. The experimental group played eleven 40‐min sessions of word exercise games. The results showed that the word exercise programme improved the spelling of the children with dyslexia. This suggests that basing training on the phonological mistakes of students with dyslexia and using the word exercise games can improve their spelling.
Live interactive teledermatology compared to in‐person care – a systematic review
Authors: Andrees V. , Klein T.M. , Augustin M. , Otten M. 
Published: November 2019

AbstractTeledermatology is a rapidly developing field of dermatological care, giving the opportunity to deliver more efficient healthcare to patients in remote areas. Live interactive (LI) teledermatology uses videoconferencing and, hence, allows for direct communication. A current overview on effectiveness, costs, feasibility and accuracy of LI applications compared to standard care is missing. The present systematic review provides this overview on LI teledermatology. Two databases were searched until April 2019, followed by title, abstract and full‐text screening. Additionally, reference lists of the detected eligible articles were screened for further eligible studies. Studies comparing LI applications with standard care were included. Data on study design, sample size, country, objectives, main findings and characteristics of LI applications were extracted. Results on time effectiveness, costs, accuracy and feasibility of LI applications were synthesized. Additionally, the quality of included studies was assessed. Twenty‐three publications were included in the final analysis: seventeen case–control studies and six randomized controlled trials. Included studies were published between 1997 and 2017. Study quality differed across studies. The studies were carried out in eight different countries. Eleven studies focused on patient consultation, three on patient organization and nine on combined applications of the aforementioned. Nine studies investigated applications facilitating patient–provider interaction. Fourteen studies evaluated applications combining patient–provider and provider–provider interaction, meaning the patient sits next to one provider while using LI applications to interact with another provider. This review reveals that LI applications can be a time effective substitute of or supplement to standard dermatological care. Results demonstrated that LI and standard care are comparable with regard to feasibility and accuracy. No clear tendencies can be reported with regard to costs. However, there is a lack of current comparative studies.