bluuebunny/crossref_metadata_2025_split huggingface dataset:
Young Children's Reliance on Information From Inaccurate Informants
Authors: Kim Sunae , Paulus Markus , Kalish Chuck 
Published: December 2016

AbstractPrior work shows that children selectively learn from credible speakers. Yet little is known how they treat information from non‐credible speakers. This research examined to what extent and under what conditions children may or may not learn from problematic sources. In three studies, we found that children displayed trust toward previously inaccurate speakers. Children were equally likely to extend labels from previously accurate and inaccurate speakers to novel objects. Moreover, they expected third parties to share labels provided by previously inaccurate speakers. Only when there was clear evidence that the speakers' information was wrong (as in the case when speakers' perceptual access to the information was blocked), did young children reject the label. Together, the findings provide evidence that young children do not completely ignore the labels supplied by non‐credible speakers unless there is strong reason to do so.
Antibody depletion strategy for the treatment of suspected antibody‐mediated rejection in lung transplant recipients: Does it work?
Authors: Vacha Mary , Chery Godefroy , Hulbert Amanda , Byrns Jennifer , Benedetti Clark , Finlen Copeland Catherine Ashley , Gray Alice , Onwuemene Oluwatoyosi , Palmer Scott M. , Snyder Laurie D. 
Published: December 2016

AbstractBackgroundDonor‐specific antibodies (DSAs) after lung transplantation correlate with poor outcomes. The ideal treatment strategy for antibody‐mediated rejection AMR is not defined. Our institution implemented an aggressive multimodality protocol for the treatment of suspected AMR.MethodsLung transplant recipients with suspected AMR were treated with a standardized protocol of plasma exchange, steroids, bortezomib, rituximab, and intravenous immune globulin. Primary outcome was DSA clearance at 6 months in those alive. Secondary endpoints included preserved allograft function at 6 months, survival at 6 and 12 months and complications due to the protocol.ResultsSixteen lung transplant recipients with documented DSA and allograft dysfunction were included in the analysis. Of the 16 patients, 11 survived to 6 months. Three of those 11 patients (27%) cleared all DSAs within 6 months of the protocol. Four of the 11 patients (36%) had preserved allograft function at 6 months. Overall 12‐month patient survival was 56%. Complications included thrombocytopenia (50%) and abdominal pain or gastrointestinal discomfort (18.7%).ConclusionsThis multimodality protocol resulted in clearance of DSAs and preserved lung function in a minority of lung transplant recipients with suspected AMR surviving to 6 months after therapy. There were significant side effects of the protocol.
Single‐center outcomes of combined heart and liver transplantation in the failing Fontan
Authors: D'Souza Benjamin A. , Fuller Stephanie , Gleason Lacey P. , Hornsby Nicole , Wald Joyce , Krok Karen , Shaked Abraham , Goldberg Lee R. , Pochettino Alberto , Olthoff Kim M. , Kim Yuli Y. 
Published: December 2016

AbstractLong‐term outcomes of the Fontan operation include Fontan failure and liver disease. Combined heart‐liver transplantation (CHLT) is an option for select patients although limited data exist on this strategy. A retrospective review of Fontan patients 18 years or older referred for cardiac transplant evaluation between 2000 and 2013 at the Hospital of the University of Pennsylvania was performed. All patients were considered for potential CHLT. Clinical variables such as demographics, perioperative factors, and short‐term outcomes were reviewed. Of 17 referrals for cardiac transplantation, seven Fontan patients underwent CHLT. All patients who underwent CHLT had either advanced fibrosis or cirrhosis on liver biopsy. There were no perioperative deaths. The most common postoperative morbidity was acute kidney injury. Short‐term complications include one episode of acute liver rejection but no cardiac rejection greater than 1R. CHLT is an acceptable therapeutic option for patients with failing Fontan physiology who exhibit concomitant advanced liver fibrosis. However, optimal patient selection is currently undefined, and long‐term outcomes are not known.
Pruritus and quality of life in renal transplant patients
Authors: Panuccio Vincenzo , Tripepi Rocco , Bellantoni Marianna , Saporito Licia , Quattrone Serena , Lacava Viviana , Parlongo Giovanna , Tripepi Giovanni , Mallamaci Francesca , Zoccali Carmine 
Published: December 2016

AbstractBackgroundPruritus has a negative impact on quality of life (QoL) in dialysis patients. The reversibility of this symptom after renal transplantation and its impact upon QoL has scarcely been studied in these patients.MethodsPruritus was evaluated by the Visual Analogue Scale (VAS), the Visual Rating Scale (VRS), and the Numerical Rating Scale (NRS) in 133 unselected renal transplant patients, 62 healthy subjects, and 29 hemodialysis patients. QoL was assessed by KDQOL‐SF™1.3. The reversibility of pruritus was studied by applying retrospectively the VRS.ResultsThe prevalence of pruritus by the VRS was 62% in hemodialysis patients, 32% in renal transplant patients, and 11% in healthy subjects (P<.001). The prevalence of pruritus among transplant patients was 32% by VRS and 38% by VAS and NRS. The prevalence of pretransplantation pruritus (68%) by the VRS recall questionnaire was higher than the prevalence of pruritus in the same patients after renal transplantation (32%, P<.01). Pruritus in transplant patients was associated with important dimensions of QoL, including social, emotional, and working limitations (P<.05 for the three comparisons).ConclusionsThe prevalence of pruritus markedly reduces after renal transplantation but remains substantially higher than in the general population and impacts upon quality of life in these patients.
Environmental impact on young children's participation in home‐based activities
Authors: Albrecht Erin C , Khetani Mary A 
Published: December 2016

AimTo test the effect of child, family, and environmental factors on young children's participation in home‐based activities.MethodCaregivers of young children were recruited using convenience and snowball sampling. Participants were 395 caregivers of children (222 males, 173 females) aged from 1 month to 5 years and 11 months. Demographic items and the home section of the Young Children's Participation and Environment Measure were administered online, followed by completion of the daily activities, mobility, and social/cognitive domains of the Pediatric Evaluation of Disability Inventory Computer Adaptive Test by telephone interview.ResultsA structural equation model fitted the data well (comparative fit index=0.91) and explained 31.2% of the variance in perceived environmental support and 42.5% of the variance in home involvement. Functional limitations and performance had an indirect effect on young children's participation through their effect on perceived environmental support. Specifically, fewer functional limitations and higher task performance were associated with greater environmental support, which in turn predicted higher levels of home involvement.InterpretationResults suggest the importance of a young child's functional abilities and task performance on caregiver perceptions of environmental support at home, and the impact of environmental support on a child's participation in home‐based activities during the early childhood period. Results warrant replication with more diverse samples to evaluate model generalizability.
Behaviour in Cornelia de Lange syndrome: a systematic review
Authors: Mulder Paul A , Huisman Sylvia A , Hennekam Raoul C , Oliver Chris , van Balkom Ingrid D C , Piening Sigrid 
Published: December 2016

AimCareful study and accurate description of behaviour are important to understand developmental challenges for individuals with Cornelia de Lange syndrome (CdLS). Here we present a systematic review of current understanding of behaviour in CdLS.MethodA systematic search was performed for articles published between January 1946 and December 2015 evaluating autism, self‐injury, and/or cognition in CdLS. After study‐selection, 43 papers were included. The Cochrane quality criteria were adjusted to assign quality scores to the included studies.ResultsParticipants were mostly categorized in the severe/profound developmental level. Methodology and quality were very heterogeneous, as well as reporting occurrence of autism. Self‐injurious behaviour was reported in 15 papers. Physical conditions were reported in 21 studies, mostly related to hearing and vision. Only nine studies mentioned details about medication.InterpretationComparison of presented results was hindered by heterogeneous assessment methods. Improving our understanding of behavioural characteristics in CdLS requires more uniform methodology. We propose a criterion standard of instruments that can ideally be used in assessment of behaviour and development. This will improve understanding of behaviour in the context of developmental level and daily functioning.
Premature mortality of epilepsy in low‐ and middle‐income countries: A systematic review from the Mortality Task Force of the International League Against Epilepsy
Authors: Levira Francis , Thurman David J. , Sander Josemir W. , Hauser W. Allen , Hesdorffer Dale C. , Masanja Honorati , Odermatt Peter , Logroscino Giancarlo , Newton Charles R. ,   the Epidemiology Commission of the International League Against Epilepsy
Published: December 2016

SummaryTo determine the magnitude of risk factors and causes of premature mortality associated with epilepsy in low‐ and middle‐income countries (LMICs). We conducted a systematic search of the literature reporting mortality and epilepsy in the World Bank‐defined LMICs. We assessed the quality of the studies based on representativeness; ascertainment of cases, diagnosis, and mortality; and extracted data on standardized mortality ratios (SMRs) and mortality rates in people with epilepsy. We examined risk factors and causes of death. The annual mortality rate was estimated at 19.8 (range 9.7–45.1) deaths per 1,000 people with epilepsy with a weighted median SMR of 2.6 (range 1.3–7.2) among higher‐quality population‐based studies. Clinical cohort studies yielded 7.1 (range 1.6–25.1) deaths per 1,000 people. The weighted median SMRs were 5.0 in male and 4.5 in female patients; relatively higher SMRs within studies were measured in children and adolescents, those with symptomatic epilepsies, and those reporting less adherence to treatment. The main causes of death in people with epilepsy living in LMICs include those directly attributable to epilepsy, which yield a mean proportional mortality ratio (PMR) of 27.3% (range 5–75.5%) derived from population‐based studies. These direct causes comprise status epilepticus, with reported PMRs ranging from 5 to 56.6%, and sudden unexpected death in epilepsy (SUDEP), with reported PMRs ranging from 1 to 18.9%. Important causes of mortality indirectly related to epilepsy include drowning, head injury, and burns. Epilepsy in LMICs has a significantly greater premature mortality, as in high‐income countries, but in LMICs the excess mortality is more likely to be associated with causes attributable to lack of access to medical facilities such as status epilepticus, and preventable causes such as drowning, head injuries, and burns. This excess premature mortality could be substantially reduced with education about the risk of death and improved access to treatments, including AEDs.
Perampanel effects in the WAG/Rij rat model of epileptogenesis, absence epilepsy, and comorbid depressive‐like behavior
Authors: Citraro Rita , Leo Antonio , Franco Valentina , Marchiselli Roberto , Perucca Emilio , De Sarro Giovambattista , Russo Emilio 
Published: December 2016

SummaryObjectivePerampanel (PER), a selective non‐competitive α‐amino‐3‐hydroxy‐5‐methyl‐4‐isoxazolepropionic acid (AMPA)‐receptor antagonist, exhibits broad‐spectrum anticonvulsant activity in several seizure models, but its potential disease‐modifying effects have not been investigated. Because of the relevance of AMPA receptors in epileptogenesis and psychiatric comorbidities, we studied the effects of PER in the WAG/Rij rat model of epileptogenesis, absence epilepsy, and depressive‐like comorbidity.MethodsWe investigated the effects of acute, subchronic, and chronic treatment with PER (0.25–3 mg/kg) on absence seizures, their development, and related psychiatric/neurologic comorbidity in WAG/Rij rats. Depression‐related behavior was studied by using the forced swimming and the sucrose preference test; anxiety‐related behavior by using the open field and elevated plus maze test; and memory by using the passive avoidance test.ResultsPER (3 mg/kg/day orally for 17 weeks starting from P30) significantly reduced the development of absence seizures at 6 months of age (1 month after treatment withdrawal), but this effect was not maintained when reassessed 4 months later. Attenuated absence seizure development was accompanied by reduced depressive‐like behavior in the forced swimming test (FST), whereas no effects were observed on anxiety‐related behavior and memory. Subchronic (1 and 3 mg/kg/day orally for 1 week) and acute PER (0.25–1 mg/kg, i.p.) dosing did not affect established absence seizures and behavior.SignificanceThese results suggest that AMPA receptors are involved in mechanisms of epileptogenesis in an established model of absence epilepsy, and that these mechanisms differ from those responsible for seizure generation and spread when epilepsy has become established.
Time to onset of sustained ≥50% responder status in patients with focal (partial‐onset) seizures in three phase III studies of adjunctive brivaracetam treatment
Authors: Klein Pavel , Johnson Martin E. , Schiemann Jimmy , Whitesides John 
Published: December 2016

SummaryTime to onset of sustained ≥50% responder status (SRS) was assessed for the pooled patient population receiving brivaracetam (BRV) 50, 100, or 200 mg/day or placebo in three randomized phase III studies (NCT00464269, NCT00490035, and NCT01261325). Patients were aged ≥16 years with well‐characterized focal (partial‐onset) seizures (FS) uncontrolled by 1–2 concomitant antiepileptic drugs. After an 8‐week baseline period, patients received study drug without up‐titration for a 12‐week (84‐day) treatment period. A patient was a sustained ≥50% responder on a particular day if they completed the entire treatment period through day 84 and was a ≥50% responder (based on percent reduction in FS frequency from baseline) both on that day and every successive day until day 84 (end of treatment period). In the pooled efficacy population (N = 1,160), 15.5%, 18.1%, and 19.4% of patients taking BRV 50, 100, or 200 mg/day, respectively, achieved SRS on day 1 versus 6.7% for placebo (p < 0.001). Statistically significant SRS was also achieved for most of the BRV‐treated groups in the three separate studies. This suggests that BRV has an early, sustained onset of action in a subset of responders. The incidence of adverse events during the first week was similar to that in the overall treatment period.
Ictal asystole: A systematic review
Authors: Tényi Dalma , Gyimesi Csilla , Kupó Péter , Horváth Réka , Bóné Beáta , Barsi Péter , Kovács Norbert , Simor Tamás , Siegler Zsuzsa , Környei László , Fogarasi András , Janszky József 
Published: December 2016

SummaryObjectiveTo comprehensively analyze ictal asystole (IA) on a large number of subjects.MethodsWe performed a systematic review of case report studies of patients diagnosed with IA (1983–2016). Each included case was characterized with respect to patient history, IA seizure characteristics, diagnostic workup, and therapy. In addition, comparative analyses were also carried out: two alignments were developed based on the delay between epilepsy onset and IA onset (“new‐onset” if <1 year, “late‐onset” if ≥1 year) and asystole duration (asystole was “very prolonged” if lasted >30 s).ResultsOne hundred fifty‐seven cases were included. All patients had focal epilepsy. In 7% of cases IA developed during a secondary generalized tonic–clonic seizure. Both the seizure‐onset zone and the focal seizure activity at asystole beginning were usually temporal (p < 0.001 and p = 0.001, respectively) and were lateralized to the left hemisphere in 62% (p = 0.005 and p = 0.05, respectively). Asystole duration was 18 ± 14 s (mean±SD) (range 3–96 s); 73% of patients had late‐onset, 27% had new‐onset IA. Compared to late‐onset IA, new‐onset IA was associated with female gender (p = 0.023), preexisting heart condition (p = 0.014), focal seizure activity at asystole beginning (p = 0.012), normal neuroimaging (p = 0.013), normal interictal EEG (p < 0.001), auditory aura (p = 0.012), and drug‐responsive epilepsy (p < 0.001). “Very prolonged” asystole was associated with secondary generalized tonic–clonic seizures (p = 0.003) and tended to occur in extratemporal lobe seizures (p = 0.074). No IA‐related death was reported.SignificanceCharacteristics considered to be typical of IA (focal, left temporal seizures appearing on grounds of a long‐lasting, intractable epilepsy) seem only partially legitimate. We suggest that in new‐onset IA, female gender and a preexisting heart condition could serve as predispositions in an otherwise benign epilepsy. We speculate that in late‐onset IA, male‐predominant changes in neuronal networks in chronic, intractable epilepsy and an accompanying autonomic dysregulation serve as facilitating factors.
Contributions of Observed Parent Socialization of Coping and Skin Conductance Level Reactivity to Childhood Adjustment
Authors: Stanger Sarah , Abaied Jamie , Wagner Caitlin , Sanders Wesley 
Published: December 2016

This research examined the longitudinal association between parent socialization of coping and child adjustment, as well as the moderating role of children's skin conductance level reactivity (SCLR). Participants were a community sample of children (n = 64, M age = 9.02, 54.5% females, 93.2% Caucasian) and their parent(s). Parent coping suggestions were observed while their child engaged in a stressful challenge task, during which the child's SCLR, a measure of children's physiological reactivity to stress, was also measured. Parent(s) completed the Child Behavior Checklist (Achenbach & Rescorla, 2001) at baseline and a 6‐month follow‐up to assess internalizing and externalizing problems. Results revealed that secondary control engagement suggestions predicted fewer internalizing problems over time. In addition, disengagement suggestions predicted fewer externalizing problems over time among children with high SCLR. This study provides evidence that parent coping suggestions serve as a resource that protects youth from developing adjustment problems.
Discovery of short linear motif‐mediated interactions through phage display of intrinsically disordered regions of the human proteome
Authors: Davey Norman E. , Seo Moon‐Hyeong , Yadav Vikash Kumar , Jeon Jouhyun , Nim Satra , Krystkowiak Izabella , Blikstad Cecilia , Dong Debbie , Markova Natalia , Kim Philip M. , Ivarsson Ylva 
Published: December 2016

The intrinsically disordered regions of eukaryotic proteomes are enriched in short linear motifs (SLiMs), which are of crucial relevance for cellular signaling and protein regulation; many mediate interactions by providing binding sites for peptide‐binding domains. The vast majority of SLiMs remain to be discovered highlighting the need for experimental methods for their large‐scale identification. We present a novel proteomic peptide phage display (ProP‐PD) library that displays peptides representing the disordered regions of the human proteome, allowing direct large‐scale interrogation of most potential binding SLiMs in the proteome. The performance of the ProP‐PD library was validated through selections against SLiM‐binding bait domains with distinct folds and binding preferences. The vast majority of identified binding peptides contained sequences that matched the known SLiM‐binding specificities of the bait proteins. For SHANK1 PDZ, we establish a novel consensus TxF motif for its non‐C‐terminal ligands. The binding peptides mostly represented novel target proteins, however, several previously validated protein–protein interactions (PPIs) were also discovered. We determined the affinities between the VHS domain of GGA1 and three identified ligands to 40–130 μm through isothermal titration calorimetry, and confirmed interactions through coimmunoprecipitation using full‐length proteins. Taken together, we outline a general pipeline for the design and construction of ProP‐PD libraries and the analysis of ProP‐PD‐derived, SLiM‐based PPIs. We demonstrated the methods potential to identify low affinity motif‐mediated interactions for modular domains with distinct binding preferences. The approach is a highly useful complement to the current toolbox of methods for PPI discovery.
Response of vegetation phenology to urbanization in the conterminous United States
Authors: Li Xuecao , Zhou Yuyu , Asrar Ghassem R. , Mao Jiafu , Li Xiaoma , Li Wenyu 
Published: December 2016

AbstractThe influence of urbanization on vegetation phenology is gaining considerable attention due to its implications for human health, cycling of carbon and other nutrients in Earth system. In this study, we examined the relationship between change in vegetation phenology and urban size, an indicator of urbanization, for the conterminous United States. We studied more than 4500 urban clusters of varying size to determine the impact of urbanization on plant phenology, with the aids of remotely sensed observations since 2003–2012. We found that phenology cycle (changes in vegetation greenness) in urban areas starts earlier (start of season, SOS) and ends later (end of season, EOS), resulting in a longer growing season length (GSL), when compared to the respective surrounding urban areas. The average difference of GSL between urban and rural areas over all vegetation types, considered in this study, is about 9 days. Also, the extended GSL in urban area is consistent among different climate zones in the United States, whereas their magnitudes are varying across regions. We found that a tenfold increase in urban size could result in an earlier SOS of about 1.3 days and a later EOS of around 2.4 days. As a result, the GSL could be extended by approximately 3.6 days with a range of 1.6–6.5 days for 25th ~ 75th quantiles, with a median value of about 2.1 days. For different vegetation types, the phenology response to urbanization, as defined by GSL, ranges from 1 to 4 days. The quantitative relationship between phenology and urbanization is of great use for developing improved models of vegetation phenology dynamics under future urbanization, and for developing change indicators to assess the impacts of urbanization on vegetation phenology.