bluuebunny/crossref_metadata_2025_split huggingface dataset:
Characteristics of Liver Disease in 100 Individuals With Joubert Syndrome Prospectively Evaluated at a Single Center
Authors: Strongin Anna , Heller Theo , Doherty Dan , Glass Ian A. , Parisi Melissa A. , Bryant Joy , Choyke Peter , Turkbey Baris , Daryanani Kailash , Yildirimli Deniz , Vemulapalli Meghana , Mullikin Jim C. ,   NISC Comparative Sequencing Program, Malicdan May C. , Vilboux Thierry , Gahl William A. , Gunay‐Aygun Meral 
Published: November 2017

ABSTRACTBackground and Aims:Joubert Syndrome (JS) is a rare, inherited, ciliopathy defined by cerebellar and brainstem malformations and is variably associated with liver, kidney, and ocular dysfunction. This study characterizes the hepatic findings in JS and identifies factors associated with probable portal hypertension.Methods:Hundred individuals with JS were prospectively evaluated at the National Institutes of Health Clinical Center. Laboratory tests, imaging, and DNA sequencing were performed. Patients were stratified based on the spleen length/patient height ratio as a marker of splenomegaly, used as a surrogate for probable portal hypertension.Results:Forty‐three patients (43%) had liver involvement based on elevated liver enzymes and/or liver hyperechogenicity and/or splenomegaly. None of the patients had macroscopic liver cysts or bile duct dilatation. Based on the spleen length/patient height ratio, 13 patients were stratified into a probable portal hypertension group. We observed significant elevations in alkaline phosphatase (269 vs 169 U/L, P ⩽ 0.001), alanine aminotransferase (92 vs 42 U/L, P = 0.004), aspartate aminotransferase (77 vs 40 U/L, P = 0.002), and gamma‐glutamyl transferase (226 vs 51 U/L, P ⩽ 0.001) in the probable portal hypertension group. Platelets were lower in the probable portal hypertension cohort (229 vs 299 × 103 cells/μL, P = 0.008), whereas synthetic function was intact in both groups. Probable portal hypertension was also more prevalent in patients with kidney disease (P = 0.001) and colobomas (P = 0.02), as well as mutations in the TMEM67 gene (P = 0.001).Conclusions:In JS, probable portal hypertension is associated with abnormal hepatic enzymes, as well as presence of kidney disease, coloboma, and/or mutation in TMEM67. These findings may allow early identification of JS patients who have or are more likely to develop liver disease.
Prevalence of Rectal Chlamydial and Gonococcal Infections: A Systematic Review
Authors: Dewart Courtney M. , Bernstein Kyle T. , DeGroote Nicholas P. , Romaguera Raul , Turner Abigail Norris 
Published: November 2017

Abstract
We undertook a systematic review to examine rectal Chlamydia trachomatis (Ct) and Neisseria gonorrhoeae (Ng) infections in women and men who have sex with men (MSM). English-language publications measuring rectal Ct or Ng prevalence using nucleic acid amplification tests were eligible. Searching multiple electronic databases, we identified 115 eligible reports published between January 2000 and November 2016. Overall, the prevalence of rectal Ct (9%) was higher than that of rectal Ng (4.7%). Rectal Ct prevalence was similar in MSM (9%) and women (9.2%), whereas rectal Ng prevalence was higher in MSM (6.1%) than in women (1.7%). Generally, rectal Ct prevalence was similar in sexually transmitted disease clinics (9.1%) and nonsexual health clinics (8.6%), whereas rectal Ng prevalence was somewhat lower in sexually transmitted disease clinics (4.5%) than in nonsexual health clinics (6%). These infections seem to be relatively common across a range of populations and clinical settings, highlighting the need for additional research on these preventable, treatable conditions.
Short-Term Propofol Infusion and Associated Effects on Serum Lactate in Pediatric Patients
Authors: Indra Sean , Haddad Haitham , O'Riordan Mary Ann 
Published: November 2017

Objectives
This study aimed to determine if short-duration procedural sedation in children with propofol is related to an adverse metabolic stress response measured by serum lactate. Propofol infusion syndrome is associated with high-dose and long-duration infusion. It has not been studied with short-duration, outpatient propofol administration.

Methods
This was a prospective, longitudinal study that included 50 patients between the ages of 2 and 18 years who were undergoing outpatient procedural sedation with propofol at a pediatric sedation unit. Patients received bolus or bolus and continuous infusion doses of propofol. Serum lactate values were obtained before and after the patients' sedated procedures.

Results
The average length of procedure was 40.3 minutes, and the mean dose of propofol per patient was 8.2 mg/kg. The highest measured lactate value was 1.8 mmol/L. The average (SD) preprocedure and postprocedure lactate values were 1.0 (0.3) and 0.7 (0.2) mmol/L, respectively, resulting in an overall significant decrease in lactate of −0.3 (0.3) mmol/L (P < 0.001). There was a significant (P = 0.01) positive relationship between age and postprocedure lactate value, when controlling for the length of the procedure in a multivariable regression. No significant association was found between propofol dosage and length of the procedure with lactate values.

Conclusion
In this study, we did not find a significant increase in metabolic stress, measured by serum lactate. Using propofol for short-duration procedural sedation may not carry similar risks for propofol infusion syndrome to those for long-duration/high-dose infusion therapy.
Onychomadesis as a Late Complication of Hand-Foot-Mouth Disease
Authors: Kuehnel Nicholas A. , Thach Sokunthirith , Thomas Danny G. 
Published: November 2017

Abstract
Hand-foot-mouth disease is a viral illness frequently caused by enterovirus and coxsackievirus. Traditionally, this disease initially causes malaise, fever, and rash with vesicles in the mouth, as well as on the hands and feet. Occasionally, more severe presentations and late postinfectious sequelae occur, including onychomadesis, nail matrix arrest. We describe a series of 4 cases of onychomadesis and its evaluation following recent hand-foot-mouth disease during this current enteroviral season as a way to ensure appropriate clinician diagnosis and guidance.
Congenital Diaphragmatic Hernia in a Child With Abdominal Pain and Respiratory Distress
Authors: Sanh William , Langer Jacob C. , Ratnapalan Savithiri 
Published: November 2017

Abstract
We report a case of late presentation of congenital diaphragmatic hernia in a child who presented to the emergency department with abdominal pain and respiratory distress. The usual and unusual presentations of congenital diaphragmatic hernia in older children and their possible complications are discussed.
Thrombelastography early amplitudes in bleeding and coagulopathic trauma patients: Results from a multicenter study
Authors: Laursen Thomas H. , Meyer Martin A.S. , Meyer Anna Sina P. , Gaarder Tina , Naess Paal A. , Stensballe Jakob , Ostrowski Sisse R. , Johansson Pär I. 
Published: November 2017

BACKGROUND
Early amplitudes in the viscoelastic hemostatic assays, thrombelastography (TEG) and rotation thromboelastometry (ROTEM), provide fast results, which is critical in the resuscitation of bleeding patients. This study investigated associations between TEG early amplitudes and standard TEG variables in a large multicenter cohort of moderately to severely injured trauma patients admitted at three North European Level I Trauma Centers.

METHODS
Prospective observational study of 404 trauma patients with clinical suspicion of severe injury from London, UK, Copenhagen, Denmark and Oslo, Norway. Biochemistry and clinical data including outcome and TEG parameters were recorded upon arrival. Kaolin TEG, Rapid TEG, and TEG functional fibrinogen curves were extracted, and early amplitudes A5 and A10 (amplitude at 5 and 10 minutes) were registered. Patients were stratified according to international normalized ratio of 1.2 or less or greater than 1.2, as well as transfusion requirements (nontransfused, 1–9 red blood cell units and ≥10 red blood cell units in 12 hours).

RESULTS
In total, 404 patients were included, median Injury Severity Score was 13. There were strong positive correlations between A5/A10 and maximum amplitude in all investigated assays. All TEG values except rTEG maximum amplitude and kTEG maximum amplitude correlated significantly with mortality in transfused patients. Time from initiation of assay to A5 and A10 were lowest for rapid TEG and TEG functional fibrinogen compared with kaolin TEG. Rapid TEG A5 reduced time to result with greater than 50% compared with rapid TEG maximum amplitude.

CONCLUSION
We found strong associations between TEG early amplitudes A5/A10 and maximum amplitude in rapid TEG, kaolin TEG, and TEG functional fibrinogen across trauma patients with coagulopathy and massive transfusion requirements. Introducing the use of early amplitudes can reduce time to diagnosis of coagulopathy and may be used in TEG monitoring of trauma patient. Further randomized controlled trials evaluating the role of TEG in guiding hemostatic resuscitation are warranted.

Level of evidence
Prognostic study, level III.
Ocular Motor Dysfunction Due to Brainstem Disorders
Authors: Lee Seung-Han , Kim Hyo-Jung , Kim Ji-Soo 
Published: November 2017

Background:The brainstem contains numerous structures including afferent and efferent fibers that are involved in generation and control of eye movements.Evidence Acquisition:These structures give rise to distinct patterns of abnormal eye movements when damaged. Defining these ocular motor abnormalities allows a topographic diagnosis of a lesion within the brainstem.Results:Although diverse patterns of impaired eye movements may be observed in lesions of the brainstem, medullary lesions primarily cause various patterns of nystagmus and impaired vestibular eye movements without obvious ophthalmoplegia. By contrast, pontine ophthalmoplegia is characterized by abnormal eye movements in the horizontal plane, while midbrain lesions typically show vertical ophthalmoplegia in addition to pupillary and eyelid abnormalities.Conclusions:Recognition of the patterns and characteristics of abnormal eye movements observed in brainstem lesions is important in understanding the roles of each neural structure and circuit in ocular motor control as well as in localizing the offending lesion.
Spatial Topography of Individual-Specific Cortical Networks Predicts Human Cognition, Personality and Emotion
Authors: Kong Ru , Li Jingwei , Orban Csaba , Sabuncu Mert R , Liu Hesheng , Schaefer Alexander , Sun Nanbo , Zuo Xi-Nian , Holmes Avram J. , Eickhoff Simon B. , Yeo B.T. Thomas 
Published: November 2017

AbstractResting-state functional magnetic resonance imaging (rs-fMRI) offers the opportunity to delineate individual-specific brain networks. A major question is whether individual-specific network topography (i.e., location and spatial arrangement) is behaviorally relevant. Here, we propose a multi-session hierarchical Bayesian model (MS-HBM) for estimating individual-specific cortical networks and investigate whether individual-specific network topography can predict human behavior. The multiple layers of the MS-HBM explicitly differentiate intra-subject (within-subject) from inter-subject (between-subject) network variability. By ignoring intra-subject variability, previous network mappings might confuse intra-subject variability for inter-subject differences. Compared with other approaches, MS-HBM parcellations generalized better to new rs-fMRI and task-fMRI data from the same subjects. More specifically, MS-HBM parcellations estimated from a single rs-fMRI session (10 minutes) showed comparable generalizability as parcellations estimated by two state-of-the-art methods using five sessions (50 minutes). We also showed that behavioral phenotypes across cognition, personality and emotion could be predicted by individual-specific network topography with modest accuracy, comparable to previous reports predicting phenotypes based on connectivity strength. Network topography estimated by MS-HBM was more effective for behavioral prediction than network size, as well as network topography estimated by other parcellation approaches. Thus, similar to connectivity strength, individual-specific network topography might also serve as a fingerprint of human behavior.
Complementary metrics of human auditory nerve function derived from compound action potentials
Authors: Harris Kelly C. , Vaden Kenneth I. , McClaskey Carolyn M. , Dias James W. , Dubno Judy R. 
Published: November 2017

AbstractDeclines in auditory nerve (AN) function contribute to suprathreshold auditory processing and communication deficits in individuals with normal hearing, hearing loss, hyperacusis, and tinnitus. Procedures to characterize AN loss or dysfunction in humans are limited. We report several novel complementary metrics to characterize AN function noninvasively in humans using the compound action potential (CAP), a direct measure of summated AN activity. We examined how these metrics change with stimulus intensity, and interpreted these changes within a framework of known physiological properties of the basilar membrane and AN. Our results reveal how neural synchrony and the recruitment of AN fibers with later first-spike latencies likely contribute to the CAP, affect auditory processing, and differ with noise exposure history in younger adults despite normal pure-tone thresholds. Moving forward, these new metrics, when applied to patient populations, can provide a means to characterize cochlear synaptopathy and other deficits in AN function in humans.New and noteworthyLoss or inactivity of auditory nerve (AN) fibers is thought to contribute to suprathreshold auditory processing deficits, but evidence-based methods to assess these effects are not available. We describe several novel metrics that may be used to quantify neural synchrony and characterize AN function.
The latent geometry of the human protein interaction network
Authors: Alanis-Lobato Gregorio , Mier Pablo , Andrade-Navarro Miguel A. 
Published: November 2017

AbstractTo mine valuable information from the complex architecture of the human protein interaction network (hPIN), we require models able to describe its growth and dynamics accurately. Here, we present evidence that uncovering the latent geometry of the hPIN can ease challenging problems in systems biology. We embedded the hPIN to hyperbolic space, whose geometric properties reflect the characteristic scale invariance and strong clustering of the network. Interestingly, the inferred hyperbolic coordinates of nodes capture biologically relevant features, like protein age, function and cellular localisation. We also realised that the shorter the distance between two proteins in the embedding space, the higher their connection probability, which resulted in the prediction of plausible protein interactions. Finally, we observed that proteins can efficiently communicate with each other via a greedy routeing process, guided by the latent geometry of the hPIN. When analysed from the appropriate biological context, these efficient communication channels can be used to determine the core members of signal transduction pathways and to study how system perturbations impact their efficiency.
Effects of the veterinary anthelmintic moxidectin on dung beetle survival and ecosystem functioning
Authors: Manning Paul , Lewis Owen T. , Beynon Sarah A. 
Published: November 2017

AbstractMacrocyclic lactones (MLs) are a class of chemical compounds administered to livestock for parasite control. These compounds are poorly metabolized and are predominately excreted in dung.When coprophagous insects such as dung beetles (Coleoptera: Scarabaeoidea) are exposed to ML residues, lethal and sublethal effects are often observed. Indirectly this can lead to ML residues impairing ecosystem functions that underpin production. A strategy to reduce these negative effects involves selecting compounds that offer lower risk to non-target invertebrates such as the ML moxidectin.Considering two dung beetle species with differing sensitivities to agricultural intensification, we asked whether exposure to moxidectin residues influenced survival, reproductive output, and functioning (short- and long-term estimates of dung removal).When exposed to moxidectin, adults of the sensitive species (Geotrupes spinigerMarsham) experienced a 43% reduction in survival. In contrast, survival of the non-sensitive species (Aphodius rufipesL.) was unaffected. We were unable to determine whether exposure affected reproductive output of either species.We found little evidence to suggest moxidectin impaired dung removal. We found however, that high densities of a species with relatively low functional importance (A. rufipes) can compensate for the loss of a functionally dominant species (G. spiniger). Over a longer timeframe, earthworms fully decomposed dung irrespective of moxidectin residues.
The Improvement of TSP Fertilizer Production & Quality
Authors: Chaouqi N. , Gharous M. El , Bouzziri M. 
Published: November 2017

ABSTRACTResults obtained showed that some essential manufacturing factors needs to be respected throughout the production loop. These factors will help obtaining a slurry exits with the desired chemical characteristics of P2O5SE (33%), Al (10%), P2O5SE + Citrate (34.5%), H2O (22%), P2O5total (39%)). It is necessary to keep a temperature within 100 ± 5 ° C., with a vapor pressure of 6 to 7 bars, an average density of H3PO4 (1470 g/l) and the solid rate in the feed acid less than 2%. After the reaction phase, the slurry is conveyed to the granulator where it is mixed with the recycled product at a recycling rate (RT) close to 3 and a K ratio between 0.1 and 0.2. This parameter is very important to be monitored to ensure a Good penetration of heat into the next phase in a relatively short time. During the drying process, the product humidity at the outlet must be in the range of 3.5% to 5% to allow the reaction to continue during the developing period. At the exit of the drying tube, the product is in very variable grains diameter (D), the objective of the following step is to extract the grain size range between 2 and 4 mm. To do so we changed the mesh size of the lower webs to 3/50 and 3.2 / 30 mm, which reduces the quantity of particle size less than 2%. Thus, the particles of diameter more than 3.15 mm must be continuously controlled to reach 64%. This portion will ensure conformity of the product for D50 and for the two intervals [2-3.15 mm] and [3.15-4 mm]. In the Storage Hall the reaction continues, the unconverted phosphate will be attacked by the unreacted H3PO4, and according to a monitoring of the evolution of the chemical composition of the final product (TSP). The required ripening time is limited to 21 days. During this study, we were able to identify and modify a number of parameters in different production phases to improve the quality of TSP according to AFCOME, knowing that each phase of the process is a client of the previous phase and supplier of the next one.
Evidence that PV+ cells enhance temporal population codes but not stimulus-related timing in auditory cortex
Authors: Krause Bryan M. , Murphy Caitlin A. , Uhlrich Daniel J. , Banks Matthew I. 
Published: November 2017

AbstractSpatio-temporal cortical activity patterns relative to both peripheral input and local network activity carry information about stimulus identity and context. GABAergic interneurons are reported to regulate spiking at millisecond precision in response to sensory stimulation and during gamma oscillations; their role in regulating spike timing during induced network bursts is unclear. We investigated this issue in murine auditory thalamo-cortical (TC) brain slices, in which TC afferents induced network bursts similar to previous reports in vivo. Spike timing relative to TC afferent stimulation during bursts was poor in pyramidal cells and SOM+ interneurons. It was more precise in PV+ interneurons, consistent with their reported contribution to spiking precision in pyramidal cells. Optogenetic suppression of PV+ cells unexpectedly improved afferent-locked spike timing in pyramidal cells. In contrast, our evidence suggests that PV+ cells do regulate the spatio-temporal spike pattern of pyramidal cells during network bursts, whose organization is suited to ensemble coding of stimulus information. Simulations showed that suppressing PV+ cells reduces the capacity of pyramidal cell networks to produce discriminable spike patterns. By dissociating temporal precision with respect to a stimulus versus internal cortical activity, we identified a novel role for GABAergic cells in regulating information processing in cortical networks.
Continuum Approximation of Invasion Probabilities
Authors: Borchering Rebecca K. , McKinley Scott A. 
Published: November 2017

AbstractIn the last decade there has been growing criticism of the use of Stochastic Differential Equations (SDEs) to approximate discrete state-space, continuous-time Markov chain population models. In particular, several authors have demonstrated the failure of Diffusion Approximation, as it is often called, to approximate expected extinction times for populations that start in a quasi-stationary state.In this work we investigate a related, but distinct, population dynamics property for which Diffusion Approximation fails: invasion probabilities. We consider the situation in which a few individual are introduced into a population and ask whether their collective lineage can successfully invade. Because the population count is so small during the critical period of success or failure, the process is intrinsically stochastic and discrete. In addition to demonstrating how and why the Diffusion Approximation fails in the large population limit, we contrast this analysis with that of a sometimes more successful alternative WKB-like approach. Through numerical investigations, we also study how these approximations perform in an important intermediate regime. In a surprise, we find that there are times when the Diffusion Approximation performs well: particularly when parameters are near-critical and the population size is small to intermediate.
Effective leadership development in information technology: building transformational and emergent leaders
Authors: Hickman Louis , Akdere Mesut 
Published: November 2017

Purpose
Effective leadership has been the focus of much research in recent years, but leadership development is still understudied. Information technology (IT) continues to grow in importance due to the industries IT creates, the industries IT disrupts, and the potential IT holds for all companies. Because leadership is highly context bound, the purpose of this paper is to examine leadership development in the IT context to take first steps toward establishing best practices for IT leadership development.

Design/methodology/approach
This conceptual paper reviews leadership in general before performing an integrated literature review of leadership as it has been studied in the IT context. Then the paper presents three propositions regarding effective IT leadership development.

Findings
IT leadership development should involve formal mentoring, robust feedback that is integrated into the development plan, and should be treated as a core process for long-term success. Emergent and transformational leadership are important for IT.

Practical implications
It behooves IT departments to implement the leadership development programs proposed here because leadership has been identified as one of the most difficult skills to find in IT employees. The findings can inform training professionals exploring ways to improve the leadership capacity and leadership development in the IT units of their organizations.

Originality/value
The paper’s literature review uncovered no quantitative peer-reviewed research on leadership development in the IT context, suggesting an area of need for further empirical studies. Researchers and practitioners alike will benefit from a greater understanding of leadership development in IT.