bluuebunny/crossref_metadata_2025_split huggingface dataset:
Association of Blood Component Ratios With 24-Hour Mortality in Injured Children Receiving Massive Transfusion
Authors: Butler Elissa K. , Mills Brianna M. , Arbabi Saman , Bulger Eileen M. , Vavilala Monica S. , Groner Jonathan I. , Stansbury Lynn G. , Hess John R. , Rivara Frederick P. 
Published: June 2019

Objectives:
To determine if higher fresh frozen plasma and platelet to packed RBC ratios are associated with lower 24-hour mortality in bleeding pediatric trauma patients.

Design:
Retrospective cohort study using the Pediatric Trauma Quality Improvement Program Database from 2014 to 2016.

Setting:
Level I and II pediatric trauma centers participating in the Trauma Quality Improvement Program

Patients:
Injured children (≤ 14 yr old) who received massive transfusion (≥ 40 mL/kg total blood products in 24 hr). Of 123,836 patients, 590 underwent massive transfusion, of which 583 met inclusion criteria.

Interventions:
None.

Measurements and Main Results:
Ratios of fresh frozen plasma:packed RBC and platelet:packed RBC. Of the 583 patients, 60% were male and the median age was 5 years (interquartile range, 2–10 yr). Overall mortality was 19.7% (95% CI, 16.6–23.2%) at 24 hours. There was 51% (adjusted relative risk, 0.49; 95% CI, 0.27–0.87; p = 0.02) and 40% (adjusted relative risk, 0.60; 95% CI, 0.39–0.92; p = 0.02) lower risk of death at 24 hours for the high (≥ 1:1) and medium (≥ 1:2 and < 1:1) fresh frozen plasma:packed RBC ratio groups, respectively, compared with the low ratio group (< 1:2). Platelet:packed RBC ratio was not associated with mortality (adjusted relative risk, 0.94; 95% CI, 0.51–1.71; p = 0.83).

Conclusions:
Higher fresh frozen plasma ratios were associated with lower 24-hour mortality in massively transfused pediatric trauma patients. The platelet ratio was not associated with mortality. Although these findings represent the largest study evaluating blood product ratios in pediatric trauma patients, prospective studies are necessary to determine the optimum blood product ratios to minimize mortality in this population.
PREDICTIVE ACTIVATION BIOMARKERS OF TREATMENT-NAIVE ASYMPTOMATIC CHOROIDAL NEOVASCULARIZATION IN AGE-RELATED MACULAR DEGENERATION
Authors: Serra Rita , Coscas Florence , Boulet Jean François , Cabral Diogo , Lupidi Marco , Coscas Gabriel J. , Souied Eric H. 
Published: June 2019

Purpose:
To assess the long-term evolution of treatment-naive quiescent choroidal neovascularization (CNV), in age-related macular degeneration (AMD), to identify predictive activation biomarkers.

Methods:
Patients with quiescent CNV underwent a comprehensive ophthalmological examination, including fluorescein and indocyanine green angiographies, structural optical coherence tomography (OCT), and OCT angiography. Qualitative and quantitative analyses of structural OCT and OCT angiography images were performed during the study period. At the last follow-up evaluation, the enrolled eyes were divided into two groups: eyes with quiescent CNV converting to exudative AMD (eAMD) and those not progressing to eAMD.

Results:
Sixty-eight eyes of 68 patients were enrolled in the study. Mean follow-up duration was 40 ± 28 months using multimodal imaging and 22 ± 13 months using OCT angiography. On structural OCT, quiescent CNV not converting to eAMD showed a preferential growth of the pigment epithelium detachment greatest linear diameter (P = 0.009), whereas the eAMD group presented a preferential growth of the pigment epithelium detachment maximal height (P < 0.0001) during the study period. Quantitative analysis of choriocapillaris OCT angiograms confirmed the CNV area growth during follow-up (from 4.18 ± 4.77 mm2 at baseline to 5.10 ± 5.06 mm2 at the last follow-up visit; P = 0.02).

Conclusion:
A close follow-up is recommended to early identify predictive activation biomarkers of treatment-naive quiescent CNV.
A Path Analysis of Effects of Patients’ Underlying Conditions, Treatment Satisfaction, and Adherence on Quality of Life Among Korea Glaucoma Patients
Authors: Kim Chan Yun , Park Ki Ho , Ahn Jaehong , Ahn Myung-Douk , Cha Soon Cheol , Kim Ho-Soong , Kim Joon Mo , Kim Moon Jung , Kim Tae-Woo , Kim Yong Yeon , Lee Ji Woong , Park Sang-Woo , Sohn Yong Ho , Sung Kyung Rim , Yoo Chungkwon , Cha Jin-Hye , Kim Young-Joo , Lee Junyoung , Choi Jimi 
Published: June 2019

Précis:This observed patient-specific features and treatment-driven conditions that were significantly associated with the impairment of health-related quality of life (HRQOL) among glaucoma patients. The results suggest that more systematic strategies should be implemented to improve an HRQOL.Purpose:To determine factors contributing to an HRQOL and assess total, direct and indirect, effects that may exist between the determinants using path analysis.Materials and Methods:Data were derived from previous Korea glaucoma outcomes research. A total of 847 glaucoma outpatients with ≤2 years of eye-drop use were included in the present study. Including factors and their hypothetical pathways to an HRQOL were based on the literature review and consultation with specialists. Age, sex, comorbidities, and education levels were included as underlying conditions and treatment patterns were defined by the number of daily instillations of prescribed eye-drops. Treatment satisfaction and adherence were estimated using a Treatment Satisfaction Questionnaire for Medication, which assessed side-effects, effectiveness, convenience, global satisfaction, and pill count. An HRQOL was assessed by EQ-5D and EQ-visual analogue scale (EQ-VAS).Results:In EQ-5D analysis, male individuals showed total effects (β=0.102,P=0.0026), including both direct (β=0.076,P=0.0095) and indirect effects (β=0.0253,P=0.0020), which were mediated by treatment satisfaction in the side effects and global satisfaction categories. Higher education (β=0.197,P=0.0020) and satisfaction in the side effect (β=0.095,P=0.0076) and global satisfaction (β=0.075,P=0.0414) categories were found to have direct effects on EQ-5D. For EQ-VAS, higher education levels had significant total effects (β=0.153,P=0.0020) accounting for direct effects (β=0.131,P=0.0030) and indirect effects (β=0.0223,P=0.0531) through global satisfaction. In addition, male individuals had indirect effects (β=0.274,P=0.0165), which was indirectly mediated by satisfaction in the global satisfaction category. Comorbidity (β=−0.096,P=0.0090) and satisfaction in the effectiveness (β=0.094,P=0.0302), convenience (β=0.076,P=0.0390), and global satisfaction (β =0.144,P=0.0020) categories showed indirect effect on EQ-VAS.Conclusion:Higher education and better treatment satisfaction had positive effects on HRQOL both in direct and indirect ways whereas comorbidities indirectly showed negative associations with HRQOL.
Argon Laser Iridoplasty For Plateau Iris Syndrome: Long-Term Outcomes of 48 Eyes
Authors: Romito Norman , Bluwol Elisa , Graber Martin , Le Pape Clémence , Lachkar Yves 
Published: June 2019

Precis:
Argon laser peripheral iridoplasty (ALPI) was performed on 48 eyes with plateau iris syndrome (PIS). Indentation gonioscopy was used to monitor the opening of the iridocorneal angle. Mean intraocular pressure (IOP) at 5 years decreased from 15.9 to 14.4 mm Hg. None of the eyes required trabeculectomy.

Purpose:
The purpose of this article was to report the long-term outcomes of ALPI for PIS.

Materials and Methods:
A retrospective chart review was performed on all patients with PIS treated with ALPI from 2001 to 2012. The study included 48 eyes from 28 patients with PIS after peripheral iridotomy, with a follow-up of at least 5 years. Patients with advanced glaucoma requiring initial surgical treatment (pathologic discs and IOP above the target IOP despite medical treatment) were excluded. The primary outcomes were the effect on the number of IOP medications, and the need for complementary selective laser trabeculoplasty (SLT) or surgery (trabeculectomy and/or phacoemulsification). Secondary outcomes were optic nerve head changes and adverse events.

Results:
The mean IOP statistically decreased after ALPI (15.91±2.62 vs. 14.35±2.18 mm Hg, P>0.001). The mean number of IOP-lowering medications statistically increased after ALPI (0.81±0.94 vs. 1.2±1.04, P>0.001). Mean follow-up was 92.4±26.5 months. At the end of the follow-up, 12 (25%) eyes had no medications, 20 (42%) had 1 medication, 11 (23%) had 2 medications, 3 (6%) had 3 medications, and 2 (4%) had 4. Ten (21%) eyes underwent SLT, 6 (10%) underwent phacoemulsification, and no trabeculectomy was necessary during follow-up. The change in cup to disc ratio from pre-ALPI to latest follow-up was not statistically significant, and no adverse events were reported.

Conclusions:
ALPI is relatively effective and safe to prevent angle-closure glaucoma and avoid trabeculectomy in patients with PIS. This procedure often helps to control IOP, although SLT and additional medical treatments are frequently necessary to maintain the target IOP.
Influence of Ocular Dimensional Change on 24-Hour Intraocular Pressure Measurement With Contact Lens Sensor
Authors: Tojo Naoki , Hayashi Atsushi 
Published: June 2019

Purpose:
We measured changes in the corneal morphology and intraocular pressure (IOP) before and after the use of contact lens sensor (CLS). Then, we investigated whether the ocular dimensional change affected the measured IOP value with CLS.

Material and Methods:
In this prospective study, we examined 112 eyes of glaucoma patients. We measured the IOP fluctuation using a Sensimed Triggerfish CLS. We measured the corneal morphology before and after making measurements using a CLS in anterior segment optic coherent optical coherence tomography and measured the IOP with a Goldmann applanation tonometer. We investigated the correlation between these ocular dimensional changes and the last IOP value with CLS.

Results:
Measurement with the CLS significantly increased the IOP (P=0.0305), made the corneal thickness (CT) significantly thicker (P<0.001), and increased the steeper meridian (P=0.0026). The last value of IOP-patterns recorded with CLS was significantly correlated with the IOP immediately after the measurement with Goldmann applanation tonometer (P=0.0134), was not significantly correlated with central and peripheral CT. The difference of steeper and flatter curvature between before and after measurement significantly correlated with the last value of IOP-patterns recorded with CLS.

Conclusions:
Measurement with a CLS affects the IOP, CT, and corneal steeper meridian. From the last value of IOP-related patterns recorded with CLS, CLS could perceive accurate IOP. The change of corneal curvature might affect CLS output values.
A Case of Murine Typhus Presenting With Hemophagocytic Lymphohistiocytosis
Authors: Poulos Samuel , Grundy Brain , Al Mouslmani Mohammed , Bellur Sunil , Goodgame Boone , Douglass Elizabeth , Mondy Kristin 
Published: June 2019

Abstract
Murine typhus, also known as endemic typhus, is a rickettsial illness transmitted by the flea Xenopsylla cheopis, usually in contact with rats. Symptoms of the illness can be nonspecific, and murine typhus has been considered an underrecognized entity as it is sometimes confused with viral illnesses. Although hemophagocytic lymphohistiocytosis (HLH), a rare inflammatory condition characterized by activation of macrophages and phagocytosis of hematopoietic cells in the bone marrow, liver, and spleen, has been reported to occur in other rickettsial illnesses, to our knowledge it has not been reported as a complication of murine typhus. We report the case of a 39-year-old man for whom murine typhus was diagnosed with serological evaluation and HLH was diagnosed via bone marrow biopsy and other studies. Although HLH has been previously described with scrub typhus, and iron overload has been described with murine typhus infection, our case represents the first report of HLH diagnosed concurrently with a murine typhus infection.
Electrocardiographic and Echocardiographic Findings in Black Athletes: A General Review
Authors: Pambo Prince , Scharhag Jürgen 
Published: June 2019

Background:
Participation in regular physical activity produces electrophysiological and structural cardiac changes in electrocardiograms (ECGs) and echocardiographs (ECHOs) of athletes, and the term “athlete's heart” is used to describe these physiological cardiovascular adaptations. Extent and type of sporting discipline, age, sex, body dimensions, and ethnicity have an influence on cardiac remodeling.

Objective:
As the recent scientific literature increasingly reports on ethnicity-specific ECG and ECHO findings in black athletes, it is the aim of this review to provide an overview of ECG and ECHO findings among athletes of black African/Afro-Caribbean descent.

Data Sources:
A systematic search of PubMed and MEDLINE databases up to and including August 2017 was conducted using the following terms/phrases “black OR African OR Afro-Caribbean athlete heart,” “black OR African OR Afro-Caribbean athlete electrocardiogram,” and “black OR African OR Afro-Caribbean athlete echocardiogram.” The search generated a total of 130 papers, out of which 16 original articles fitted our criteria and were selected for this review.

Main Results:
The various studies reviewed revealed that about 10% to 30% of black African/Afro-Caribbean athletes had abnormal ECG. R/S voltage criteria exceeding hypertrophic indices were found in about 60% to 89% of black African/Afro-Caribbean athletes. ST-segment elevation (17%-90%) and T-wave inversions were also common findings among this ethnicity. About 10% to 12% of black African/Afro-Caribbean athletes had a left ventricular wall thickness ranging from 13 to 15 mm. Cavity dimensions ranged from 40 to 66 mm in black African/Afro-Caribbean athletes with a relative wall thickness >0.44.

Conclusions:
Updated ethnic-specific guidelines are required to discriminate physiological from pathologic hypertrophy and repolarization changes. Future studies should focus on homogeneous cohorts of African athletes.
Switching to Bortezomib may Improve Recovery From Severe Vincristine Neuropathy in Pediatric Acute Lymphoblastic Leukemia
Authors: Joshi Jaitri , Tanner Lynn , Gilchrist Laura , Bostrom Bruce 
Published: June 2019

Purpose:
The purpose of this study was to evaluate the impact of switching patients being treated for acute lymphoblastic leukemia (ALL) from vincristine to bortezomib.

Patients and Methods:
A total of 20 patients with ALL were switched from vincristine to bortezomib (1.3 mg/m2/dose) because of worsening neuropathy despite physical therapy interventions (n=18) or at increased risk of neuropathy (n=2). Relapse rates were compared with 56 vincristine-only patients matched by prognostic factors. Maintenance blood counts in bortezomib patients were compared with cooperative group data using vincristine during maintenance. In addition, 6 evaluable patients were assessed for neuropathy using the pediatric-modified total neuropathy score. Neuropathy scores were collected during treatment with vincristine and after switching to bortezomib.

Results:
After a median follow-up of 3.5 years the relapse rate in patients switched to bortezomib was nonsignificantly different than those remaining on vincristine. Patients on monthly bortezomib had statistically significantly lower platelet counts that did not require transfusions or dose adjustment. Total neuropathy for all 6 cases decreased significantly when switched to bortezomib from vincristine (P=0.015), with motor neuropathy declines in 5 of 6 subjects.

Conclusions:
Bortezomib substitution for vincristine in ALL treatment is a potential strategy to mitigate severe vincristine neuropathy. These findings should be confirmed in a randomized clinical trial to further assess benefits and risks of this approach.
Obesity is associated with postinjury hypercoagulability
Authors: Samuels Jason M. , Moore Ernest E. , Coleman Julia R. , Sumislawski Joshua J. , Cohen Mitchell J. , Silliman Christopher C. , Banerjee Anirban , Ghasabyan Arsen , Chandler James , Sauaia Angela 
Published: June 2019

BACKGROUND
Obesity is linked to hypercoagulability with an increased risk of venous thromboembolic events (VTE) in the uninjured population. Therefore, we hypothesize that obesity (body mass index [BMI] ≥30 kg/m2 [BMI30]) is associated with a hypercoagulable state postinjury characterized by increased clot strength and resistance to fibrinolysis.

METHODS
Our prospective Trauma Activation Protocol database includes all trauma activations patients for whom a rapid thrombelastography is obtained within 60 minutes postinjury prior to any transfusions. The data set was then stratified by BMI and subjects with BMI30 were compared with those with BMI less than 30 kg/m2). The following thrombelastography measurements were obtained: activated clotting time, clot formation rate (angle), maximum clot strength (MA), and % clot lysis 30 minutes after MA (LY30, %). Fibrinolysis shutdown (SD) was defined as LY30 < 0.6% and hyperfibrinolysis (HF) as LY30 greater than 7.6%. Continuous variables are expressed as median (interquartile range).

RESULTS
Overall, 687 patients were included of whom 161 (23%) had BMI30. The BMI30 group was older, had a lower proportion of males and of blunt trauma, and was less severely injured. After adjustment for confounders, BMI30 was independently associated with lower odds of MA less than 55 mm (odds ratio [OR], 0.28; 95% confidence interval [CI], 0.13–0.60) and of HF (OR, 0.31; 95% CI, 0.10–0.97) and higher odds of SD (OR, 1.82; 95% CI, 1.09–3.05). No independent association was observed with angle less than 65° (OR 0.57 95% CI 0.30–1.05). While VTEs were more frequent among BMI30 patients (5.0 vs. 3.3%), this did not reach significance after confounding adjustment (p = 0.11).

CONCLUSION
Obesity was protective against diminished clot strength and hyperfibrinolysis, and obesity was associated with an increased risk of fibrinolytic SD in severely injured patients. These findings suggest a relative hypercoagulability. Although no difference in VTEs was noted in this study, these findings may explain the higher rate of VTEs reported in other studies.

LEVEL OF EVIDENCE
Prognostic and Epidemiological, level III.
Redlisting the redlist: a global analysis of species distributions and biodiversity
Authors: Hughes Alice C. 
Published: June 2019

AbstractGlobal conservation and research has come to rely on the IUCN species range-maps to direct research, allocate funding and define and design protected areas. However, IUCN species range-maps may be created on the basis of little actual data. Though the IUCN methods may be suitable for well known species, they may be liable unreliable for lesser known species or areas. In such cases, human biases may limit the usefulness of the output maps and potentially misdirect conservation funding and protection. Possible errors in these IUCN maps has global implications for the preservation of biodiversity, as flawed data leads to flawed decisions, which are of critical importance to the protection of the biodiversity of this planet. Ultimately we show that the current IUCN “expert-assessment based” range-maps may hinder rather than assist global conservation, as errors ranged from tens to thousands of kilometres (between recorded distributions and IUCN-maps), and for some taxa under 50% of records fell within their mapped-ranges. IUCN maps are being used globally to evaluate global conservation and protection, yet up to 85.4% of IUCN species-range boundaries follow political-boundaries, making using IUCN maps to evaluate the protected-area system and it’s efficacy impossible. The current availability of data, methods and computer-power has relegated these maps in the wake of more empirically based methods, and better use of available data provides the ability to make better conservation decisions, especially for highly threatened species and regions.
TGAC Browser: An open-source genome browser for non-model organisms
Authors: Thanki Anil S. , Bian Xingdong , Davey Robert P. 
Published: June 2019

Genome browsers play a vital role to provide visualisation for genomic data. It is often the case that bespoke genome browser customisations are required between different research groups, with an obvious necessity to update, upgrade and tailor tracks and features on a potentially frequent basis. However, most of the current genome browsers require highly curated data held in public repositories. Besides, these genome browsers often rely on particular dependencies, where writing plug-in or modifying existing code can be troublesome and resource expensive.We present TGAC Browser, a new open-source web-based genome browser designed to overcome shortcomings in available approaches. It uses a locally installed Ensembl Core Database schema and is also able to visualise data from well-known NGS data formats. We also added simple analysis functionality to perform BLAST searches within TGAC Browser. TGAC Browser also allows uploading your genomic data. TGAC Browser is an open-source, easy to set up, and user-friendly genome browser with minimal, lightweight configuration details.
Human and mouse essentiality screens as a resource for disease gene discovery
Authors: Cacheiro Pilar , Muñoz-Fuentes Violeta , Murray Stephen A. , Dickinson Mary E. , Bucan Maja , Nutter Lauryl M.J. , Peterson Kevin A. , Haselimashhadi Hamed , Flenniken Ann M. , Morgan Hugh , Westerberg Henrik , Konopka Tomasz , Hsu Chih-Wei , Christiansen Audrey , Lanza Denise G. , Beaudet Arthur L. , Heaney Jason D. , Fuchs Helmut , Gailus-Durner Valerie , Sorg Tania , Prochazka Jan , Novosadova Vendula , Lelliott Christopher J. , Wardle-Jones Hannah , Wells Sara , Teboul Lydia , Cater Heather , Stewart Michelle , Hough Tertius , Wurst Wolfgang , Sedlacek Radislav , Adams David J. , Seavitt John R. , Tocchini-Valentini Glauco , Mammano Fabio , Braun Robert E. , McKerlie Colin , Herault Yann , de Angelis Martin Hrabě , Mallon Ann-Marie , Lloyd K.C. Kent , Brown Steve D.M. , Parkinson Helen , Meehan Terrence F. , Smedley Damian ,   on behalf of the Genomics England Research Consortium and the International Mouse Phenotyping Consortium
Published: June 2019

ABSTRACTAlthough genomic sequencing has been transformative in the study of rare genetic diseases, identifying causal variants remains a considerable challenge that can be addressed in part by new gene-specific knowledge. Here, we integrate measures of how essential a gene is to supporting life, as inferred from the comprehensive viability and phenotyping screens performed on knockout mice by the International Mouse Phenotyping Consortium and from human cell line essentiality screens. We propose a novel, cross-species gene classification across theFullSpectrum of Intolerance toLoss-of-function (FUSIL) and demonstrate that genes in five mutually exclusive FUSIL categories have differing characteristics in the biological processes they regulate, tissue expression levels and human mutation rates. Most notably, Mendelian disease genes, particularly those associated with developmental disorders, are highly overrepresented in the developmental lethal category, representing genes not essential for cell survival but required for organism development. Exploiting this finding, we have screened developmental disorder cases from three independent disease sequencing consortia and identified potentially pathogenic,de novovariants shared in different patients for several developmental lethal genes that have not previously been associated with rare disease. We therefore propose FUSIL as an efficient resource for disease gene discovery.