bluuebunny/crossref_metadata_2025_split huggingface dataset:
Exon-specific U1 snRNAs improve ELP1 exon 20 definition and rescue ELP1 protein expression in a familial dysautonomia mouse model
Authors: Donadon Irving , Pinotti Mirko , Rajkowska Katarzyna , Pianigiani Giulia , Barbon Elena , Morini Elisabetta , Motaln Helena , Rogelj Boris , Mingozzi Federico , Slaugenhaupt Susan A , Pagani Franco 
Published: April 2018

Abstract
Familial dysautonomia (FD) is a rare genetic disease with no treatment, caused by an intronic point mutation (c.2204+6T&gt;C) that negatively affects the definition of exon 20 in the elongator complex protein 1 gene (ELP1 also known as IKBKAP). This substitution modifies the 5′ splice site and, in combination with regulatory splicing factors, induces different levels of exon 20 skipping, in various tissues. Here, we evaluated the therapeutic potential of a novel class of U1 snRNA molecules, exon-specific U1s (ExSpeU1s), in correcting ELP1 exon 20 recognition. Lentivirus-mediated expression of ELP1-ExSpeU1 in FD fibroblasts improved ELP1 splicing and protein levels. We next focused on a transgenic mouse model that recapitulates the same tissue-specific mis-splicing seen in FD patients. Intraperitoneal delivery of ELP1-ExSpeU1s-adeno-associated virus particles successfully increased the production of full-length human ELP1 transcript and protein. This splice-switching class of molecules is the first to specifically correct the ELP1 exon 20 splicing defect. Our data provide proof of principle of ExSpeU1s-adeno-associated virus particles as a novel therapeutic strategy for FD.
Citizen science records describe the distribution and migratory behaviour of a piscivorous predator, Pomatomus saltatrix
Authors: Brodie S , Litherland L , Stewart J , Schilling H T , Pepperell J G , Suthers I M 
Published: April 2018

Abstract
Understanding the processes driving species distributions is a key question in ecology, yet obtaining pertinent data for many species can be challenging. Tag-recapture data from voluntary recreational fisheries programmes is an undervalued citizen science data resource and can provide valuable observation records for data-poor species. Here, we highlight the utility of such data by compiling four tag-recapture datasets (n = 20 878 tags) to describe the movements and distribution of a piscivorous predator, tailor Pomatomus saltatrix, with respect to their environment. We compared species distribution models that explored a trade-off between temporal duration and spatial extent. Both models revealed sea surface temperature (SST) was an important factor in describing the distribution and density of tailor. However, the model with broader spatial extent indicated a non-linear response to SST, peaking at 21.5°C, while the model with temporal longevity showed a linear SST response. This disjunct showcases the importance of building models across an adequate geographic extent to ensure covariate responses are not truncated. This is the first study to comprehensively describe the distribution and migrations of adult tailor across their eastern Australian distribution. The results are discussed in light of ocean warming along eastern Australia, and the subsequent implications for using tag-recapture data in fisheries management.
Clinical Updates in Women’s Health Care Summary: Asthma
Authors: Namazy Jennifer A. , Schatz Michael , Dombrowski Mitchell 
Published: April 2018

Abstract
Asthma is a common and potentially life-threatening lung disease. The prevalence of asthma is higher in women than in men. This monograph provides an overview of the pathophysiology, evaluation, and management of asthma in women. Management approaches in reproductive-aged, pregnant, and postmenopausal patients are addressed.
How to Optimally Fit a Hearing Aid for Bimodal Cochlear Implant Users: A Systematic Review
Authors: Vroegop Jantien L. , Goedegebure André , van der Schroeff Marc P. 
Published: April 2018

Objectives:
Bimodal hearing has shown to improve speech recognition in quiet and in noise and to improve sound localization compared with unilateral cochlear implant (CI) use alone. Fitting the CI and hearing aid (HA) separately has been described well, but HA fitting procedures for bimodal CI users are not well researched or widely accepted. The aim of the present study was to systematically review the literature on the effect of different HA fitting strategies on auditory performance in bimodal CI users.

Design:
Original articles, written in English, were identified through systematic searches in Medline (OvidSP), Embase, Web of Science, Scopus, CINAHL, Cochrane, PubMed publisher, and Google Scholar. The quality of the studies was assessed on five aspects: methodologic quality (with the methodological index for nonrandomized studies score), number of subjects, quality of the description of contralateral hearing loss, quality of HA verification, and direct comparison of HA fitting procedures based on auditory performance.

Results:
A total of 1665 records were retrieved, of which 17 were included for systematical reviews. Critical appraisal led to three high-quality studies, 10 medium-quality studies, and four low-quality studies. The results of the studies were structured according to four topics: frequency response, frequency translation/transposition, dynamic range compression, and loudness. In general, a bimodal benefit was found in most studies, using various strategies for the HA fitting. Using a standard prescription rule such as National Acoustics Laboratory formula-non-linear 1, National Acoustics Laboratory formula-non-linear 2, or desired sensation level is a good starting point in children and adults.

Conclusions:
Although a bimodal benefit was found in most studies, there is no clear evidence how certain choices in HA fitting contribute to optimal bimodal performance. A generally accepted HA prescription rule is an essential part of most fitting procedures used in the studies. Current evidence suggests that frequency lowering or transposition is not beneficial. Individual fine tuning based on loudness or general preference is often applied, but its additional value for auditory performance should be investigated more thoroughly. Good quality comparative studies are needed to further develop evidence-based fitting procedures in case of bimodal listening.
Comparison of the Clinical Accuracy Between Point-to-Point Registration and Auto-Registration Using an Active Infrared Navigation System
Authors: Zhao Jingwei , Liu Yajun , Fan Mingxing , Liu Bo , He Da , Tian Wei 
Published: April 2018

Study Design.
A model experiment.

Objective.
To measure and compare the clinical accuracy of point-to-point registration (PR) and auto-registration (AR) in an operative set using an active infrared navigation system.

Summary of Background Data.
PR and AR are two major registration methods of navigation assisted spinal surgery. No previous study compared the difference between the two methods with respect to clinical accuracy.

Methods.
A novel method was used to measure the clinical accuracy of the navigation system under an operative set using a Sawbone model with titanium beads on the surface, which was essential to measure the accuracy numerically, instead of a real patient. Both the operative set and the procedure mimicked a regular surgery. The clinical accuracy was defined as the average distance between the “navigation coordinate” and the “image coordinate.” The clinical accuracy of the PR using preoperative computed tomography (CT) images and the AR using intraoperative CT images was measured and compared.

Results.
The average clinical accuracy of PR was different among different segments. The accuracy of the most accurate segment, which provided the reference points during the PR, was 1.10 mm. In the two segments adjacent to the reference segment, the clinical accuracy deteriorated to 1.37 and 1.50 mm. The accuracy of the farther segments was worse. In comparison, the clinical accuracy of different segments of AR was of no significant difference. The average accuracy of AR was 0.74 mm, which was significantly better than the best accuracy of PR.

Conclusion.
AR is better than PR with respect to clinical accuracy in navigation assisted spinal surgery.

Level of Evidence: N/A
Lordosis Recreation in Transforaminal and Posterior Lumbar Interbody Fusion
Authors: Robertson Peter A. , Armstrong William A. , Woods Daniel L. , Rawlinson Jeremy J. 
Published: April 2018

Study Design.
Controlled cadaveric study of surgical technique in transforaminal and posterior lumbar interbody fusion (TLIF and PLIF)

Objective.
To evaluate the contribution of surgical techniques and cage variables in lordosis recreation in posterior interbody fusion (TLIF/PLIF).

Summary of Background Data.
The major contributors to lumbar lordosis are the lordotic lower lumbar discs. The pathologies requiring treatment with segmental fusion are frequently hypolordotic or kyphotic. Current posterior based interbody techniques have a poor track record for recreating lordosis, although recreation of lordosis with optimum anatomical alignment is associated with better outcomes and reduced adjacent segment change needing revision. It is unclear whether surgical techniques or cage parameters contribute significantly to lordosis recreation.

Methods.
Eight instrumented cadaveric motion segments were evaluated with pre and post experimental radiological assessment of lordosis. Each motion segment was instrumented with pedicle screw fixation to allow segmental stabilization. The surgical procedures were unilateral TLIF with an 18° lordotic and 27 mm length cage, unilateral TLIF (18°, 27 mm) with bilateral facetectomy, unilateral TLIF (18°, 27 mm) with posterior column osteotomy (PCO), PLIF with bilateral cages (18°, 22 mm), and PLIF with bilateral cages (24°, 22 mm). Cage insertion used and “insert and rotate” technique.

Results.
Pooled results demonstrated a mean increase in lordosis of 2.2° with each procedural step (lordosis increase was serially 1.8°, 3.5°, 1.6°, 2.5°, and 1.6° through the procedures). TLIF and PLIF with PCO increased lordosis significantly compared with unilateral TLIF and TLIF with bilateral facetectomy. The major contributors to lordosis recreation were PCO, and PLIF with paired shorter cages rather than TLIF.

Conclusion.
This study demonstrates that the surgical approach to posterior interbody surgery influences lordosis gain and PCO optimizes lordosis gain in TLIF. The bilateral cages used in PLIF are shorter and associated with further gain in lordosis. This information has the potential to aid surgical planning when attempting to recreate lordosis to optimize outcomes.

Level of Evidence: N/A
COMBINATION THERAPY OF INTRAVITREAL RANIBIZUMAB AND SUBTHRESHOLD MICROPULSE PHOTOCOAGULATION FOR MACULAR EDEMA SECONDARY TO BRANCH RETINAL VEIN OCCLUSION
Authors: Terashima Hiroko , Hasebe Hiruma , Okamoto Fumiki , Matsuoka Naoki , Sato Yayoi , Fukuchi Takeo 
Published: April 2018

Purpose:
To determine the efficacy of the combination therapy of intravitreal ranibizumab (IVR) and 577-nm yellow laser subthreshold micropulse laser photocoagulation (SMLP) for macular edema secondary to branch retinal vein occlusion cystoid macular edema.

Methods:
Retrospective, consecutive, case–control study. Forty-six eyes of 46 patients with treatment-naive branch retinal vein occlusion cystoid macular edema were enrolled. The IVR + SMLP group consisted of 22 patients who had undergone both SMLP and IVR. Intravitreal ranibizumab group consisted of 24 patients who had undergone IVR monotherapy. Intravitreal ranibizumab therapy was one initial injection and on a pro re nata in both groups, and SMLP was performed at 1 month after IVR in the IVR + SMLP group. Preoperatively and monthly, best-corrected visual acuity and central retinal thickness were evaluated using swept source optical coherence tomography.

Results:
Best-corrected visual acuity and central retinal thickness significantly improved at 6 months in IVR + SMLP and IVR groups. Best-corrected visual acuity and central retinal thickness were not significantly different between the two groups at any time points. The number of IVR injections during initial 6 months in IVR group (2.3 ± 0.9) was significantly greater (P = 0.034) than that in IVR + SMLP group (1.9 ± 0.8).

Conclusion:
The combination therapy of IVR and SMLP can treat branch retinal vein occlusion cystoid macular edema effectively, by decreasing the frequency of IVR injections while maintaining good visual acuity.
DETECTION OF TYPE 1 CHOROIDAL NEOVASCULAR MEMBRANES USING OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY IN TUBERCULAR POSTERIOR UVEITIS
Authors: Aggarwal Kanika , Agarwal Aniruddha , Sharma Aman , Sharma Kusum , Gupta Vishali ,   for the OCTA Study Group
Published: April 2018

Purpose:
To study optical coherence tomography angiography (OCTA) and multimodal imaging features of Type 1 inflammatory choroidal neovascularization (CNV) in tubercular serpiginous-like choroiditis and response to anti–vascular endothelial growth factor therapy.

Methods:
In this study, multimodal imaging was performed using OCTA, enhanced-depth imaging optical coherence tomography, fluorescein angiography, and indocyanine green angiography. Correlation of OCTA with other imaging modalities in the detection of CNV was performed. The changes in CNV configuration after anti–vascular endothelial growth factor therapy were assessed.

Results:
In this study, nine eyes (8 patients; 5 females; mean age: 32.5 ± 11.57 years) with diagnosis of tubercular serpiginous-like choroiditis were included. All the eyes had presence of low-lying pigment epithelial detachments on enhanced-depth imaging optical coherence tomography. Using OCTA, it was possible to detect Type 1 CNV in all eyes. Type 1 CNV networks comprised fine anastomotic network of vessels, some of which had a hairpin loop configuration. After anti–vascular endothelial growth factor therapy, there was a decrease in branching and anastomosis. The visual acuity significantly improved from 0.49 ± 0.26 (20/60 Snellen equivalent) at baseline to 0.26 ± 0.17 (20/36 Snellen equivalent) (P = 0.03) in all eyes.

Conclusion:
Type 1 CNV can occur among patients with tubercular serpiginous-like choroiditis, leading to significant visual loss even in the healed stages of the disease. Optical coherence tomography angiography can help in the detection of Type 1 CNV where conventional multimodal imaging, including fluorescein angiography and OCT, fails to make a definitive diagnosis and thereby guide the initiation of anti–vascular endothelial growth factor therapy.
Robotic Beating Heart Totally Endoscopic Coronary Artery Bypass in Higher-Risk Patients
Authors: Balkhy Husam H. , Nisivaco Sarah , Kitahara Hiroto , McCrorey Mackenzie , Patel Brooke 
Published: April 2018

Objective Patients with a high Society of Thoracic Surgeons (STS) predicted risk of mortality undergoing coronary artery bypass surgery are known to have worse outcomes. Less invasive approaches have been shown to improve morbidity and mortality for these patients. In this study, we examined perioperative outcomes in higher-risk patients undergoing robotic totally endoscopic beating heart coronary artery bypass surgery. Methods The STS predicted risk of mortality was reviewed for patients undergoing robotic totally endoscopic beating heart coronary artery bypass surgery at our institution between January 2013 and May 2017. We identified a higher-risk cohort (n = 50) and compared them to a lower-risk cohort (n = 220) during the same period. The higher-risk group was formed from patients with the 50 highest STS scores. Perioperative data were collected retrospectively. Results There were 50 patients in the higher-risk group (mean STS score = 7.05 ± 4.9, mean age = 73 years) and 220 patients in the lower-risk group (mean STS score = 0.89 ± 0.6, mean age = 64 years). The higher-risk group had significantly greater rates of renal insufficiency, peripheral vascular disease, and lower ejection fraction. The incidence of postoperative re-exploration for bleeding, stroke, myocardial infarction, and prolonged ventilation was similar. Perioperative blood transfusion and hospital length of stay were greater in the higher-risk group. Mortality was lower in the higher-risk group (0% vs 1.8% P = 0.045). Cardiac-related mortality was similar at midterm follow-up. Conclusions We conclude that beating heart totally endoscopic beating heart coronary artery bypass surgery can be performed in patients with a higher STS predicted risk of mortality with excellent outcomes. Further studies are warranted to evaluate long-term results of totally endoscopic beating heart coronary artery bypass surgery in this challenging group of patients.
An Integrative Review of Nurse-Authored Research to Improve Health Equity and Human Rights for Criminal-Justice-Involved People
Authors: Goshin Lorie S. , Colbert Alison M. , Carey John F. 
Published: April 2018

ABSTRACT

Background
An estimated 10 million people are incarcerated internationally, including 2.1 million people in the United States. Criminal justice involvement is a social determinant of individual and family health disparities. Health care in correctional and forensic psychiatric facilities is nurse driven. The unique contributions of nurse authors to the research literature on health equity and humane conditions for this population have not been aggregated, nor have the nursing research gaps in this area been systematically identified. This article determines the volume and country location of nurse-authored research in this area and analyzes the research by population (prisons, jails, community supervision, forensic psychiatry), clinical foci, and research methods.

Methods
We conducted an integrative review of research literature published in English between 1990 and 2017.

Results
Our search revealed 283 data-based articles written by 349 nurse authors, representing Asia, Australia, Europe, and North and South Americas. Most research occurred in prisons (53.3%) and focused on mental health/substance use (32.5%), infectious disease (21.6%), or correctional health services (15.6%). Almost one third of articles across clinical foci sampled nurses, not patients.

Implications
Although there is clearly a cadre of nurse researchers working in this area, it remains a relatively small group focused most closely on mental health, infectious disease, and the correctional nursing experience. Gaps and opportunities for important nursing contributions remain, especially in reproductive health, meeting the needs of community-supervised people, and explicitly connecting this work with its human rights implications.
Longitudinal Evaluation of Bone-Anchored Hearing Aid Implant Stability Using the Advanced System for Implant Stability Testing (ASIST)
Authors: Westover Lindsey , Faulkner Gary , Hodgetts William , Kamal Fraaz , Lou Edmond , Raboud Don 
Published: April 2018

Objective:
This study aims to provide a clinical evaluation of the Advanced System for Implant Stability Testing (ASIST) for assessment of implant stability for bone-anchored hearing aid patients. We evaluate the longitudinal changes in implant interface stability during the first year following surgery.

Methods:
ASIST measurements were collected for 39 patients selected to receive a bone anchored hearing aid for hearing loss. Measurements were collected at the time of surgery and at 3 days, 2 weeks, 1 month, 3 months, 6 months, and 12 months following surgery. Longitudinal changes in ASIST Stability Coefficient (ASC) were determined for each patient. Correlations were investigated between initial implant stability as measured by the ASC and clinical parameters such as operating surgeon, patient age at surgery, and implant type.

Results:
ASC values ranged from 11.9 to 137.0 (31.9 ± 18.0). On average, there was a slight decrease in ASC up to 3 months after surgery followed by an increase up to 1 year. Preliminary results presented in this study suggest that there may be differences in the initial stability between operating surgeons (p = 0.0012; p = 0.0049) and there was a trend toward possible differences between different implant types.

Conclusion:
We have shown promising results using the ASIST in a clinical setting for longitudinal evaluation of bone–implant interface integrity. Isolating the interface properties from the implant–abutment system allows for objective comparisons across patients that are not possible with other stability measurement systems.
Immunogenicity of a Brucella abortus 55-kDa recombinant surface protein conjugated with detoxified lipopolysaccharide in black and albino type C mouse model
Authors: Yahyaie Sara , Nojoomi Farshad , Shafiei Morvarid , Ghasemian Abdolmajid 
Published: April 2018

Objective:Brucellaspp. causes zoonotic infections. Lipopolysaccharide (LPS) is a major antigenicBrucellastructure stimulating a T-independent response. For eliciting cell-mediated immunity against LPS, it should be conjugated with a polypeptide antigen. The aim of this study was to evaluate the immunogenicity ofBrucella abortus55-kDa recombinant surface protein (BC55) conjugated with detoxified LPS in black and albino type C (BALB/c) mouse model.Materials and methods:TheB. abortus bc55gene was amplified and cloned into pET32a (+) plasmid. The recombinant vectors were transferred intoEscherichia coliGM2163 and the expression of the recombinant protein was induced by 1-mmol/l isopropyl β-d-1-thiogalactopyranoside. The BC55 protein was purified using a nickel resin column and, next, conjugated with detoxified LPS and injected subcutaneously to BALB/c mice including five groups.B. abortus544 and PBS were used as positive and negative controls, respectively. Recovered bacteria from spleen of mice were compared with those of members of the control group.Results:The BC55 with 57-kDa size was purified by resin–nickel column method. The conjugation of BC55 + dLPS induced major immune response, significantly higher than each of the dLPS, PBS, and BC55 protein components.Conclusion:The conjugation of BC55 + dLPS induced responses significantly higher than each of the dLPS, PBS, and BC55 protein components. In addition, the dLPS + BC55 conjugate not only had efficient results in the induction of immune responses as a new vaccination strategy, it (also) can reduce the number ofB. abortusin spleen.
Physician Confidence in Dental Trauma Treatment and the Introduction of a Dental Trauma Decision-Making Pathway for the Pediatric Emergency Department
Authors: Cully Matthew , Cully Jennifer , Nietert Paul J. , Titus M. Olivia 
Published: April 2018

Objectives
The objectives of this study were to (1) survey and report the awareness and confidence of pediatric emergency medicine physicians in the management of dental trauma and (2) determine the prevalence of dental trauma decision-making pathway utilization in the pediatric emergency department.

Methods
A survey was distributed through e-mail to the pediatric emergency medicine discussion list via Brown University LISTSERV. The survey study included 10 questions and was multiple-choice. The survey contained questions about physician confidence and their use of a dental trauma decision-making pathway.

Results
A total of 285 individuals responded to the survey. Somewhat confident was the most common response (61%) followed by not confident (20%) and confident (19%) by respondents in treating dental trauma. Forty-one percent of respondents felt comfortable, 39% somewhat comfortable, 19% not comfortable, and 1% not sure in replanting an avulsed tooth. Only 6% of respondents reported that their pediatric emergency department always or sometimes uses a dental trauma decision-making pathway, whereas 78% of pediatric emergency departments do not.

Conclusions
We believe that the adoption of a decision-making pathway will provide timely management, improve emergency physician comfort, and enhance outcomes for pediatric patients presenting with a dental trauma. A future multicenter review will aim to evaluate these goals based on the utilization of our dental trauma decision-making pathway.