bluuebunny/crossref_metadata_2025_split huggingface dataset:
The natural course and complications of alpha‐mannosidosis—a retrospective and descriptive study
Authors: Malm Dag , Riise Stensland Hilde Monica Frostad , Edvardsen Øyvind , Nilssen Øivind 
Published: June 2013

AbstractMost alpha‐mannosidosis patients described have been children and information on the natural course of the disorder has been based on a very limited number of observations. In order to assess the disease presentation in detail and to study disease characteristics, a study was started in 1991 and has been ongoing for over 20 years. Patients with confirmed alpha‐mannosidosis were recruited through The International Society for Mannosidosis and Related Diseases (ISMRD) where families affected with alpha‐mannosidosis received a questionnaire on general clinical information to be filled out by the responsible physician. The questionnaire was returned by 125 patients (64 %). Of these, 45 patients were 15 years old or older at the time of evaluation. The questionnaire allowed us to assess the following features: Facial dysmorphism, columnar disease, arthritis, myopathy, hearing impairment, mental impairment, psychosis, bone disease and motor function as well as general health. This study describes the progression of alpha‐mannosidosis and may be helpful in determining the clinical characteristics for assessments of prognosis.
Cysteine‐rich protein 61 (CCN1) and connective tissue growth factor (CCN2) at the crosshairs of ocular neovascular and fibrovascular disease therapy
Authors: Yan Lulu , Chaqour Brahim 
Published: June 2013

AbstractThe vasculature forms a highly branched network investing every organ of vertebrate organisms. The retinal circulation, in particular, is supported by a central retinal artery branching into superficial arteries, which dive into the retina to form a dense network of capillaries in the deeper retinal layers. The function of the retina is highly dependent on the integrity and proper functioning of its vascular network and numerous ocular diseases including diabetic retinopathy, age‐related macular degeneration and retinopathy of prematurity are caused by vascular abnormalities culminating in total and sometimes irreversible loss of vision. CCN1 and CCN2 are inducible extracellular matrix (ECM) proteins which play a major role in normal and aberrant formation of blood vessels as their expression is associated with developmental and pathological angiogenesis. Both CCN1 and CCN2 achieve disparate cell‐type and context‐dependent activities through modulation of the angiogenic and synthetic phenotype of vascular and mesenchymal cells respectively. At the molecular level, CCN1 and CCN2 may control capillary growth and vascular cell differentiation by altering the composition or function of the constitutive ECM proteins, potentiating or interfering with the activity of various ligands and/or their receptors, physically interfering with the ECM‐cell surface interconnections, and/or reprogramming gene expression driving cells toward new phenotypes. As such, these proteins emerged as important prognostic markers and potential therapeutic targets in neovascular and fibrovascular diseases of the eye. The purpose of this review is to highlight our current knowledge and understanding of the most recent data linking CCN1 and CCN2 signaling to ocular neovascularization bolstering the potential value of targeting these proteins in a therapeutic context.
Measures of Balance Performance Are Affected by a Rested Versus Fatigued Testing Condition in People With Multiple Sclerosis
Authors: Jackson Kurt , Bigelow Kimberly Edginton 
Published: June 2013

ObjectiveTo investigate the effects of a rested and fatigued testing condition on measures of balance in people with multiple sclerosis (MS).DesignProspective observational study.SettingAcademic research laboratory.ParticipantsFifteen ambulatory adults with relapsing remitting or secondary progressive MS with moderate disability.MethodsMeasures of postural control, dynamic balance, and fatigue were assessed during a “rested” and “fatigued” testing condition on separate days.Main Outcome MeasurementsStatic posturography was used to assess postural control. The Mini‐Balance Evaluation Systems Test and Dynamic Gait Index were used to assess dynamic balance. The chronic fatigue level was measured by using the Fatigue Severity Scale, and acute fatigue was assessed by using a visual analog scale at multiple time points during testing.ResultsDuring the fatigued condition, the participants reported a significant (P < .05) increase in acute fatigue levels (visual analog scale) and demonstrated a significant (P < .05) decrease in performance on measures of postural control and dynamic balance when compared with the rested condition. However, no significant relationship was identified between the changes in fatigue and changes in balance performance.ConclusionsMeasures of balance performance and acute fatigue may be affected by rested versus fatigued testing conditions in people with MS. However, no significant relationships between changes in balance and fatigue were identified. Therefore, clinicians should use caution when making assumptions about the influence of fatigue on balance performance and should test for individual responses. Clinicians and researchers should also consider controlling for factors such as time of day and prior physical activity when administering standardized measures of balance in people with MS.
Vasoactive intestinal peptide (VIP) and pituitary adenylate cyclase-activating polypeptide (PACAP) in the circulation after sumatriptan
Authors: Hansen Jakob Møller , Fahrenkrug Jan , Petersen Jesper , Wienecke Troels , Olsen Karsten Skovgaard , Ashina Messoud 
Published: June 2013

Abstract

Background and purpose
The origin of migraine pain is still elusive, but increasingly researchers focus on the neuropeptides in the perivascular space of cranial vessels as important mediators of nociceptive input during migraine attacks. The parasympathetic neurotransmitters, pituitary adenylate cyclase activating peptide-38 (PACAP38) and vasoactive intestinal peptide (VIP) may be released from parasympathetic fibres and activate sensory nerve fibres during migraine attacks. Triptans are effective and well tolerated in acute migraine management but the exact mechanism of action is still debated. Triptans might reduce circulating neuropeptides. To examine this question, we examined the effect of sumatriptan on VIP and PACAP levels in vivo, under conditions without trigeminovascular system activation.

Methods
In 16 healthy volunteers we measured VIP and PACAP levels before and after administration of subcutaneous sumatriptan. We simultaneously collected blood samples from the internal and external jugular, the cubital veins and the radial artery, thereby covering both the cerebral and systemic circulation. VIP and PACAP determinations were assayed blindly with respect to timing and vascular compartments, but with all samples of a patient in the same assay, to minimize the influence of interassay variation.

Results
We found no difference in VIP and PACAP concentrations between the internal and external jugular, the cubital veins and the radial artery, (P>0.05), and the circulating levels of VIP and PACAP did not change over time (P>0.05). We found excellent agreement between neuropeptide levels in the internal and the external jugular system.

Conclusion
Sumatriptan did not change the levels of circulating VIP and PACAP in the intra or extra cerebral circulation in healthy volunteers. Under baseline conditions, without trigeminovascular activation, sumatriptan does not affect the release of neuropeptides VIP and PACAP.

Implications
Our results indicate no effect of 5-HT1B/D receptor activation on circulating levels of VIP and PACAP in humans without trigeminovascular activation. Given that neuropeptides play an important role for migraine it would be interesting to conduct a similar study in a migraine population.
Diophantine type of interval exchange maps
Authors: KIM DONG HAN 
Published: June 2013

AbstractRoth type irrational rotation numbers have several equivalent arithmetical characterizations as well as several equivalent characterizations in terms of the dynamics of the corresponding circle rotations. In this paper we investigate how to generalize Roth-like Diophantine conditions to interval exchange maps. If one considers the dynamics in parameter space one can introduce two non-equivalent Roth type conditions, the first (condition (Z)) by means of the Zorich cocycle [Finite Gauss measure on the space of interval exchange transformations. Lyapunov exponents. Ann. Inst. Fourier 46(2) (1996), 325–370], the second (condition (A)) by means of a further acceleration of the continued fraction algorithm by Marmi–Moussa–Yoccoz introduced in [The cohomological equation for Roth type interval exchange maps, J. Amer. Math. Soc. 18 (2005), 823–872]. A third very natural condition (condition (D)) arises by considering the distance between the discontinuity points of the iterates of the map. If one considers the dynamics of an interval exchange map in phase space then one can introduce the notion of Diophantine type by considering the asymptotic scaling of return times pointwise or with respect to uniform convergence (respectively conditions (R) and (U)). In the case of circle rotations all the above conditions are equivalent. For interval exchange maps of three intervals we show that (D) and (A) are equivalent and imply (Z), (U) and (R), which are equivalent among them. For maps of four intervals or more we prove several results; the only relation that we cannot decide is whether (Z) implies (R) or not.
The ghost of parasites past: eggs of the blood fluke Cardicola chaetodontis (Aporocotylidae) trapped in the heart and gills of butterflyfishes (Perciformes: Chaetodontidae) of the Great Barrier Reef
Authors: YONG R. Q-Y. , CUTMORE S. C. , MILLER T. L. , ADLARD R. D. , CRIBB T. H. 
Published: June 2013

SUMMARYWe explored the distribution of Cardicola chaetodontis in chaetodontid fishes from the Great Barrier Reef. We found just four infections of adult worms in 238 individuals of 26 chaetodontid species. By contrast, eggs were present in hearts of 75 fishes (31·5%) and 19 of 26 chaetodontid species (all Chaetodon species). In 10 cases eggs contained moving miracidia; all the others were dead and degenerating. Eggs were sought in the gills of 51 individual fish. There were 17 cases of eggs being present in gills while present in the heart, but also 13 cases where eggs were absent from gills but present in the heart, suggesting that eggs remain longer in heart tissue than in gills. ITS2 rDNA sequences from two adult worms and eggs extracted from gills of five fishes (all different species) were identical to previously reported sequences of C. chaetodontis except for a single base-pair difference in two samples. We conclude that aporocotylid eggs trapped in fish heart tissues may inform understanding of the distributions and host ranges of aporocotylids, especially where adult prevalence is low. The low host-specificity of C. chaetodontis contrasts with higher specificity of trematodes of chaetodontids that have trophic transmission.
Challenges and opportunities for faecal microbiota transplantation therapy
Authors: ROGERS G. B. , BRUCE K. D. 
Published: June 2013

SUMMARYThe incidence, morbidity, and mortality associated withClostridium difficilegastrointestinal infections has increased greatly over recent years, reaching epidemic proportions; a trend due, in part, to the emergence of hypervirulent and antibiotic-resistant strains. The need to identify alternative, non-antibiotic, treatment strategies is therefore urgent. The ability of bacteria in faecal matter transplanted from healthy individuals to displace pathogen populations is well recognized. Further, there is growing evidence that such faecal microbiota transplantation can be of benefit in a wide range of conditions associated with gut dysbiosis. Recent technical advances have greatly increased our ability to understand the processes that underpin the beneficial changes in bacterial community composition, as well as to characterize their extent and duration. However, while much of the research into faecal microbiota transplantation focuses currently on achieving clinical efficacy, the potential for such therapies to contribute to the transmission of infective agents also requires careful consideration.
Nonvolatile liquid anthracenes for facile full-colour luminescence tuning at single blue-light excitation
Authors: Babu Sukumaran Santhosh , Hollamby Martin J. , Aimi Junko , Ozawa Hiroaki , Saeki Akinori , Seki Shu , Kobayashi Kenji , Hagiwara Keita , Yoshizawa Michito , Möhwald Helmuth , Nakanishi Takashi 
Published: June 2013

AbstractNonvolatile room-temperature luminescent molecular liquids are a new generation of organic soft materials. They possess high stability, versatile optical properties, solvent-free fluid behaviour and can effectively accommodate dopant dye molecules. Here we introduce an approach to optimize anthracene-based liquid materials, focussing on enhanced stability, fluorescence quantum yield, colour tunability and processability, with a view to flexible electronic applications. Enveloping the anthracene core in low-viscosity branched aliphatic chains results in stable, nonvolatile, emissive liquid materials. Up to 96% efficient energy-transfer-assisted tunable emission is achieved by doping a minute amount of acceptor dye in the solvent-free state. Furthermore, we use a thermoresponsive dopant to impart thermally controllable luminescence colours. The introduced strategy leading to diverse luminescence colours at a single blue-light excitation can be an innovative replacement for currently used luminescent materials, providing useful continuous emissive layers in developing foldable devices.
NGS++: a library for rapid prototyping of epigenomics software tools
Authors: Nordell Markovits Alexei , Joly Beauparlant Charles , Toupin Dominique , Wang Shengrui , Droit Arnaud , Gevry Nicolas 
Published: June 2013

Abstract
Motivation: The development of computational tools to enable testing and analysis of high-throughput–sequencing data is essential to modern genomics research. However, although multiple frameworks have been developed to facilitate access to these tools, comparatively little effort has been made at implementing low-level programming libraries to increase the speed and ease of their development.
Results: We propose NGS++, a programming library in C++11 specialized in manipulating both next-generation sequencing (NGS) datasets and genomic information files. This library allows easy integration of new formats and rapid prototyping of new functionalities with a focus on the analysis of genomic regions and features. It offers a powerful, yet versatile and easily extensible interface to read, write and manipulate multiple genomic file formats. By standardizing the internal data structures and presenting a common interface to the data parser, NGS++ offers an effective framework for epigenomics tool development.
Availability: NGS++ was written in C++ using the C++11 standard. It requires minimal efforts to build and is well-documented via a complete docXygen guide, online documentation and tutorials. Source code, tests, code examples and documentation are available via the website at http://www.ngsplusplus.ca and the github repository at https://github.com/NGS-lib/NGSplusplus.
Contact: nicolas.gevry@usherbrooke.ca or arnaud.droit@crchuq.ulaval.ca
The Anesthesia in Abdominal Aortic Surgery (ABSENT) Study
Authors: Lindholm Espen E. , Aune Erlend , Norén Camilla B. , Seljeflot Ingebjørg , Hayes Thomas , Otterstad Jan E. , Kirkeboen Knut A. 
Published: June 2013

Abstract

Background:
On the basis of data indicating that volatile anesthetics induce cardioprotection in cardiac surgery, current guidelines recommend volatile anesthetics for maintenance of general anesthesia during noncardiac surgery in hemodynamic stable patients at risk for perioperative myocardial ischemia. The aim of the current study was to compare increased troponin T (TnT) values in patients receiving sevoflurane-based anesthesia or total intravenous anesthesia in elective abdominal aortic surgery.

Methods:
A prospective, randomized, open, parallel-group trial comparing sevoflurane-based anesthesia (group S) and total intravenous anesthesia (group T) with regard to cardioprotection in 193 patients scheduled for elective abdominal aortic surgery. Increased TnT level on the first postoperative day was the primary endpoint. Secondary endpoints were postoperative complications, nonfatal coronary events and mortality.

Results:
On the first postoperative day increased TnT values (>13 ng/l) were found in 43 (44%) patients in group S versus 41 (43%) in group T (P = 0.999), with no significant differences in TnT levels between the groups at any time point. Although underpowered, the authors found no differences in postoperative complications, nonfatal coronary events or mortality between the groups.

Conclusions:
In elective abdominal aortic surgery sevoflurane-based anesthesia did not reduce myocardial injury, evaluated by TnT release, compared with total intravenous anesthesia. These data indicate that potential cardioprotective effects of volatile anesthetics found in cardiac surgery are less obvious in major vascular surgery.
Surgery at the End of Life
Authors: Barnet Caryn S. , Arriaga Alexander F. , Hepner David L. , Correll Darin J. , Gawande Atul A. , Bader Angela M. 
Published: June 2013

Abstract

Background:
More than a quarter of medical costs for Medicare beneficiaries are incurred in the last year of life; surgical intensity during this time is significant. This study was performed to determine types of operations patients undergo in their terminal year, and compare characteristics of decedents with those of survivors.

Methods:
Population of 747 consecutive all-payer patients seen at the preoperative assessment center of a tertiary care hospital. Patient characteristics were obtained from the electronic medical record. Surgical indication (palliative, curative, diagnostic, elective) was assessed based on procedure performed and underlying diagnosis. Vital status was determined using the electronic medical record with confirmation via social security national death master file. Descriptive statistics were performed to compare patient characteristics and procedures performed on those who died within 1 yr of procedure with those of survivors.

Results:
Thirty-seven patients (5%) were confirmed dead at 1 yr. Ten (27%) of these had palliative procedures, 11 (30%) diagnostic, 14 (38%) curative, and 2 (5%) elective. Decedents were more likely to have undergone a palliative (27 vs. 3%) or diagnostic (30 vs. 14%) procedure and less likely to have undergone an elective procedure (5 vs. 42%) than survivors (P < 0.0001). Nearly half of decedents did not have an advanced directive by the date of surgical intervention.

Conclusions:
Nearly 1 in 20 patients seen at the preoperative assessment clinic of a tertiary care hospital died within 1 yr of their procedure. Patient characteristics and procedure indication for decedents differed from those of survivors. Similar analyses based on institution and region may provide methodologies to compare variation in surgical intensity and assist preoperative care providers in evaluating appropriateness of resource allocation.
Arabidopsis Enhanced Drought Tolerance1/HOMEODOMAIN GLABROUS11 Confers Drought Tolerance in Transgenic Rice without Yield Penalty
Authors: Yu Linhui , Chen Xi , Wang Zhen , Wang Shimei , Wang Yuping , Zhu Qisheng , Li Shigui , Xiang Chengbin 
Published: June 2013

Abstract
Enhancing drought tolerance without yield decrease has been a great challenge in crop improvement. Here, we report the Arabidopsis (Arabidopsis thaliana) homodomain-leucine zipper transcription factor Enhanced Drought Tolerance/HOMEODOMAIN GLABROUS11 (EDT1/HDG11) was able to confer drought tolerance and increase grain yield in transgenic rice (Oryza sativa) plants. The improved drought tolerance was associated with a more extensive root system, reduced stomatal density, and higher water use efficiency. The transgenic rice plants also had higher levels of abscisic acid, proline, soluble sugar, and reactive oxygen species-scavenging enzyme activities during stress treatments. The increased grain yield of the transgenic rice was contributed by improved seed setting, larger panicle, and more tillers as well as increased photosynthetic capacity. Digital gene expression analysis indicated that AtEDT1/HDG11 had a significant influence on gene expression profile in rice, which was consistent with the observed phenotypes of transgenic rice plants. Our study shows that AtEDT1/HDG11 can improve both stress tolerance and grain yield in rice, demonstrating the efficacy of AtEDT1/HDG11 in crop improvement.
Dermatological side‐effects of telaprevir‐based triple therapy for chronic hepatitis C in phase III trials in Japan
Authors: Torii Hideshi , Sueki Hirohiko , Kumada Hiromitsu , Sakurai Yuko , Aoki Keiji , Yamada Ichimaro , OHtsuki Mamitaro 
Published: June 2013

AbstractTelaprevir‐based triple therapy is highly effective for chronic hepatitis C. However, concern has been expressed over the high frequency and severity of its dermatological side‐effects compared with those associated with peginterferon (PEG‐IFN) and ribavirin (RBV) therapy. Thus, here, we evaluated the dermatological adverse reactions of telaprevir‐based triple therapy in Japanese multicenter phase III clinical trials in an attempt to characterize the dermatological side‐effects and establish appropriate management plans. In these trials, 126 treatment‐naïve patients and 141 treatment‐failure patients were administrated telaprevir, PEG‐IFN‐α‐2b and RBV for 12 weeks followed by PEG‐IFN‐α‐2b and RBV for another 12 weeks (T12/PR24 group), and 63 treatment‐naïve patients were administrated PEG‐IFN‐α‐2b and RBV for 48 weeks (PR48 group). Dermatological adverse reactions developed in over 80% patients in both groups, and most of them were grade 1 or 2. In the T12/PR24 group, there were more grade 2 or grade 3 events, and the time to onset was earlier than that in the PR48 group. Most reactions could be managed with topical corticosteroids and oral antihistamines, and the rates of discontinuation due to dermatological reactions were not high even in the T12/PR24 group. In the T12/PR24 group, however, two cases of Stevens–Johnson syndrome and one case of drug rash with eosinophilia and systemic symptoms, which corresponds to drug‐induced hypersensitivity syndrome in Japan, were reported. For appropriate treatments of individual dermatological adverse reactions, the judgment of discontinuation of antiviral drugs and treatment based on the severity are extremely important in this triple therapy.