bluuebunny/crossref_metadata_2025_split huggingface dataset:
Strain-specific differences in brain gene expression in a hydrocephalic mouse model with motile cilia dysfunction
Authors: McKenzie Casey W. , Preston Claudia C. , Finn Rozzy , Eyster Kathleen M. , Faustino Randolph S. , Lee Lance 
Published: August 2018

AbstractCongenital hydrocephalus results from cerebrospinal fluid accumulation in the ventricles of the brain and causes severe neurological damage, but the underlying causes are not well understood. It is associated with several syndromes, including primary ciliary dyskinesia (PCD), which is caused by dysfunction of motile cilia. We previously demonstrated that mouse models of PCD lacking ciliary proteins CFAP221, CFAP54 and SPEF2 all have hydrocephalus with a strain-dependent severity. While morphological defects are more severe on the C57BL/6J (B6) background than 129S6/SvEvTac (129), cerebrospinal fluid flow is perturbed on both backgrounds, suggesting that abnormal cilia-driven flow is not the only factor underlying the hydrocephalus phenotype. Here, we performed a microarray analysis on brains from wild type and nm1054 mice lacking CFAP221 on the B6 and 129 backgrounds. Expression differences were observed for a number of genes that cluster into distinct groups based on expression pattern and biological function, many of them implicated in cellular and biochemical processes essential for proper brain development. These include genes known to be functionally relevant to congenital hydrocephalus, as well as formation and function of both motile and sensory cilia. Identification of these genes provides important clues to mechanisms underlying congenital hydrocephalus severity.
Imidazole Compounds for Protecting Choroidal Endothelial Cells from Complement Injury
Authors: Zeng Shemin , Wen Kuo-Kuang , Workalemahu Grefachew , Sohn Elliott H. , Wu Meng , Chirco Kathleen R. , Flamme-Wiese Miles J. , Liu Xiuying , Stone Edwin M. , Tucker Budd A. , Mullins Robert F. 
Published: August 2018

AbstractAge-related macular degeneration (AMD) is a common, blinding disease associated with increased complement system activity. Eyes with AMD show elevated accumulation of the membrane attack complex (MAC) in the choriocapillaris and degeneration of macular choriocapillaris endothelial cells (ECs). Thus, one could reasonably conclude that the endothelial cell death that occurs in AMD is due to injury by the MAC. We therefore sought to identify strategies for protecting ECs against MAC lysis. RF/6A endothelial cells were pre-incubated with a library of FDA-approved small molecules, followed by incubation with complement intact human serum quantification of cell death. Two closely related molecules identified in the screen, econazole nitrate and miconazole nitrate, were followed in validation and mechanistic studies. Both compounds reduced lysis of choroidal ECs treated with complement-intact serum, across a range of doses from 1 to 100 µM. Cell rescue was confirmed in mouse primary choroidal ECs. Both exosome release and cell surface roughness (assessed using a Holomonitor system) were reduced by drug pretreatment in RF/6A cells, whereas endosome formation increased with both drugs, consistent with imidazole-mediated alterations of cell surface dynamics. The results in the current study provide further proof of principle that small molecules can protect choroidal ECs from MAC-induced cell death and suggest that FDA approved compounds may be beneficial in reducing vascular loss and progression of AMD.
Impact of peritoneal carcinomatosis on clinical outcomes of patients receiving self-expandable metal stents for malignant colorectal obstruction
Authors: Park Jae , Rhee Kwangwon , Yoon Jin , Park Soo , Kim Joo , Kim Jie-Hyun , Youn Young , Kim Tae , Park Hyojin , Kim Won , Cheon Jae 
Published: August 2018

Abstract
Background Peritoneal carcinomatosis can influence clinical outcomes of patients receiving self-expandable metal stents (SEMS) for malignant colorectal obstruction, but data regarding this issue are sparse. We analyzed the clinical outcomes of post-SEMS insertion for malignant colorectal obstruction based on carcinomatosis status.
Methods Stent- and patient-related clinical outcomes were compared for carcinomatosis status in a retrospective review involving 323 consecutive patients (colorectal cancer 198 patients; extracolonic malignancy 125 patients) who underwent palliative SEMS placement for malignant colorectal obstruction from January 2005 to March 2012. Severity of carcinomatosis was classified as mild, moderate, or severe.
Results Carcinomatosis was observed in 190 patients (58.8 %). The rates of technical (84.7 vs. 94.7 %; P = 0.005) and clinical (73.2 vs. 83.5 %; P = 0.03) success were lower in patients with vs. without carcinomatosis. Rates of early (2.1 % vs. 3.0 %; P = 0.72) and delayed (1.6 % vs. 6.0 %; P = 0.08) perforation and stent failure (27.9 % vs. 26.3 %; P = 0.75) showed no difference. Technical and clinical success rates were significantly different based on the severity of carcinomatosis (technical success rate: mild 90.7 %, moderate 97.4 %, severe 76.3 %, P = 0.003; clinical success rate: mild 83.3 %, moderate 82.1 %, severe 63.9 %, P = 0.01). In multivariate analysis, severe carcinomatosis was identified as an independent factor related to technical (odds ratio [OR] 0.18, 95 % confidence interval [CI] 0.06 – 0.56) and clinical (OR 0.33, 95 %CI 0.15 – 0.74) success.
Conclusions Peritoneal carcinomatosis was associated with decreased technical and clinical success rates in patients receiving SEMS for malignant colorectal obstruction. Moreover, the presence of severe carcinomatosis was an independent factor determining these clinical outcomes.
New Direct Assay of Free Protein S Antigen Applied to Diagnosis of Protein S Deficiency
Authors: Aillaud M F , Pouymayou K , Brunet D , Alessi M C , Amiral J , Juhan-Vague I 
Published: August 2018

SummaryCongenital deficiencies of protein S (PS) are associated with thrombophilia. Their characterization and classification have been hampered by the complex physiology of the protein C-protein S system and the poor standardization and reliability of laboratory assays. The free active form of protein S is usually determined by immunoassay using polyclonal antibodies in the plasma supemate after polyethyleneglycol (PEG) precipitation. A new one step ELISA using two monoclonal antibodies specific for distinct epitopes of the free form of protein S has been developed for the direct measurement of free PS in untreated plasma.We have tested two ELISA assays for free PS. One assay was based on the PEG precipitation (Asserachrom PS®, Stago, Asnières, France) whereas the other was a one step ELISA assay (Asserachrom® free PS, Stago). Values were obtained in 35 PS deficient patients recruited among 500 consecutive patients evaluated by the laboratory for diagnosis of congenital disorders of coagulation. Values were compared to those obtained in 50 patients with no PS deficiency matched for age and sex with the PS deficient patients as well as in 33 normal subjects and in 12 pregnant women. Strong correlation was found between the two tests (r = 0.81, p<10-5) in the entire population (n = 130), as well as in the separate groups. The new one step ELISA was more accurate than the PEG free PS determination. Determination of PS activity and antigens allowed us to separate quantitative and qualitative deficiencies. Among the qualitative deficiencies, isolated decrease in PS activity was the most frequent defect observed (66%). This fact questions the substitution of PS activity assays by the one step antigenic free PS ELISA assay.
Factor XIa Induced Activation of the Intrinsic Cascade In Vivo
Authors: Cate Hugo ten , Biemond Bart J , Levi Marcel , Wuillemin Walter A , Bauer Kenneth , Barzegar Samad , Buller Harry R , Hack C Erik , ten Cate Jan W , Rosenberg Robert D 
Published: August 2018

SummaryCoagulation factor XI is a glycoprotein of the contact factor system. Its deficiency is associated with a highly variable bleeding tendency, thus a role in relation to hemostasis appears to exist. However, the importance of factor XI for stimulating intrinsic coagulation in vivo has not yet been determined. To study the procoagulant effects of human factor Xla in vivo, we infused the purified enzyme into normal chimpanzees (100 Μg) in the absence or presence of the thrombin inhibitor rec-hirudin (1.0 mg/kg loading dose plus 0.3 mg/kg body wt continuous infusion). Factor Xla elicited an immediate activation of factors IX, X, and prothrombin, as measured by their respective activation fragments. However, whereas the activation of factors IX and X was immediate and shortlasting, (peak increments of 6- and 1.4-fold of baseline at 5 minutes after injection), the conversion of prothrombin gradually increased, reaching a summit of 6-fold baseline values after 60 min, and remaining elevated during the course of the experiments. Thrombin-antithrombin complexes also remained elevated during the study period. In the presence of hirudin, the initial activation of factors IX, X, and prothrombin was unchanged, however the further increment in prothrombin fragment FI+2 was markedly inhibited. These results demonstrate that factor Xla is a potential agonist of the intrinsic cascade in vivo, which activity is enhanced in the presence of thrombin.
Morphological Alterations in the External Gills of Some Tadpoles in Response to pH
Authors: Thabah Caressa , Devi Longjam , Hooroo Rupa , Dey Sudip 
Published: August 2018

Introduction Water pH affects the breeding, hatching, development, locomotion, mortality and habitat distributions of species in nature. The external gills of anuran tadpoles were studied by several authors in relation to abiotic factors. Exposure to low and high pH has been found to adversely affect the different tissues of various organisms. On that consideration, the present investigation was performed with tadpoles of the species Hyla annectans and Euphlyctis cyanophlyctis.
Material and Methods The maximum and the minimum pH thresholds were determined prior to the detailed experiments on the effects of pH. The pH that demonstrated 50% mortality was taken as the minimum and maximum pH thresholds. The hatchlings of both the species were then subjected to different pH (based on the minimum and maximum pH thresholds). After 48 hours of exposure, the external gills of the hatchlings were anesthetized and observed under a scanning electron microscope.
Results After 48 hours, clumping, overlapping and curling of the secondary filaments of the external gills and epithelial lesions in response to both acidic and alkaline pH were observed. The lengths of the secondary filaments were also affected by pH in both the species studied when compared with the control groups.
Conclusion Scanning electron microscopic approaches are relevant in assessing the adverse effects of pH on the morphology of the external gills of H. annectans and E. cyanophlyctis tadpoles, which included problems with osmoregulation, acid-base balance and respiratory function.
Diameter, Vessel Thickness and Angle of Bifurcation of the Radial Artery in Ethiopian Cadavers
Authors: Getachew Dereje , Astatkie Ayalew , Lemma Kinfe 
Published: August 2018

Introduction The basic knowledge about the morphology, variation, diameter, and wall thickness of the radial artery is very important for clinicians to make precise decisions in matters such as performing percutaneous coronary intervention (PCI) using catheterization.
Materials and Methods This was a descriptive study involving the dissection of 78 upper limbs (39 right, 39 left) of the cadavers of 18 adult females and 21 adult males in the age range between 25 and 77 years old whose radial artery and its branches were exposed. The measurement of the radial artery was taken at its origin from the brachial artery at the cubital fossa and at the wrist joint.
Results The mean ± standard deviation (SD) of the internal diameter of the right radial artery at the wrist joint was 2.79 ± 1.10 mm, and the mean ± (SD) of the internal diameter of the left radial artery at the wrist joint was 2.66 ± 0.94 mm. The paired samples t-test showed that this difference was not statistically significant (t-test [t] [degree of freedom (df) = 38] = 1.19; p-value = 0.24). The mean ± SD of the left internal diameter of the radial artery at the wrist joint was 2.56 ± 1.06 mm in males, and 2.77 ± 0.80 mm in females. This difference was also not statistically significant (t [df = 37] = -0.68; p = 0.12). There was also no significant difference in vessel thickness and in the bifurcation angle either between the left and right limbs or between genders.
Conclusion The diameter of the radial artery in the Ethiopian population is comparable to that of other populations, and its size is adequate for the most commonly used radial catheters.
A Guide to Utilization of the Microbiology Laboratory for Diagnosis of Infectious Diseases: 2018 Update by the Infectious Diseases Society of America and the American Society for Microbiologya
Authors: Miller J Michael , Binnicker Matthew J , Campbell Sheldon , Carroll Karen C , Chapin Kimberle C , Gilligan Peter H , Gonzalez Mark D , Jerris Robert C , Kehl Sue C , Patel Robin , Pritt Bobbi S , Richter Sandra S , Robinson-Dunn Barbara , Schwartzman Joseph D , Snyder James W , Telford Sam , Theel Elitza S , Thomson Richard B , Weinstein Melvin P , Yao Joseph D 
Published: August 2018

Abstract
The critical nature of the microbiology laboratory in infectious disease diagnosis calls for a close, positive working relationship between the physician/advanced practice provider and the microbiologists who provide enormous value to the healthcare team. This document, developed by experts in laboratory and adult and pediatric clinical medicine, provides information on which tests are valuable and in which contexts, and on tests that add little or no value for diagnostic decisions. This document presents a system-based approach rather than specimen-based approach, and includes bloodstream and cardiovascular system infections, central nervous system infections, ocular infections, soft tissue infections of the head and neck, upper and lower respiratory infections, infections of the gastrointestinal tract, intra-abdominal infections, bone and joint infections, urinary tract infections, genital infections, and other skin and soft tissue infections; or into etiologic agent groups, including arthropod-borne infections, viral syndromes, and blood and tissue parasite infections. Each section contains introductory concepts, a summary of key points, and detailed tables that list suspected agents; the most reliable tests to order; the samples (and volumes) to collect in order of preference; specimen transport devices, procedures, times, and temperatures; and detailed notes on specific issues regarding the test methods, such as when tests are likely to require a specialized laboratory or have prolonged turnaround times. In addition, the pediatric needs of specimen management are also emphasized. There is intentional redundancy among the tables and sections, as many agents and assay choices overlap. The document is intended to serve as a guidance for physicians in choosing tests that will aid them to quickly and accurately diagnose infectious diseases in their patients.
Article 101(1)
Authors: Whish Richard , Bailey David 
Published: August 2018

Abstract
This chapter discusses Article 101(1) of the Treaty on the Functioning of the European Union (TFEU), which prohibits agreements, decisions by associations of undertakings and concerted practices that restrict competition. It begins by explaining the terms ‘undertakings’ and ‘associations of undertakings’. It then considers what is meant by the terms ‘agreements’, ‘decisions’ and ‘concerted practices’, as well as what is meant by the phrase ‘prevention, restriction and distortion of competition’ that serves as the purpose of the provision. The chapter then deals with the de minimis doctrine, before explaining the requirement of an effect on trade between Member States. The chapter concludes with a checklist of agreements that, for a variety of reasons, normally fall outside Article 101(1).
Competition Act 1998 and the cartel offence: public enforcement and procedure
Authors: Whish Richard , Bailey David 
Published: August 2018

Abstract
This chapter describes the system of public enforcement under the Competition Act 1998. Reinforced by the Enterprise Act 2002 and Enterprise Regulatory Reform Act 2013, the Competition Act provides a set of procedural rules of investigation for the enforcement of the Chapter I and II prohibitions. After a section on complaints, it considers the extent to which it may be possible to receive guidance from the Competition and Markets Authority (‘the CMA’) on the application of the Act. It then deals in turn with enforcement of the Competition Act 1998, the criminal cartel offence and company director disqualification. This is followed by an overview of the concurrency provisions, stating that the CMA works hand-in-hand with the sectoral regulators. The final two sections discuss the appeal mechanism under the Competition Act and the possibility of Article 267 references to the Court of Justice.
Searching for the lost Unicorn: a prominent feature in the radial velocity distribution of stars in Vela from Gaia DR2 data
Authors: de la Fuente Marcos R , de la Fuente Marcos C 
Published: August 2018

ABSTRACT
Stellar streams are ubiquitous in the Galactic halo and they can be used to improve our understanding of the formation and evolution of the Milky Way as a whole. The so-called Monoceros Ring might have been the result of satellite accretion. Guglielmo et al. have used N-body simulations to search for the progenitor of this structure. Their analysis shows that, if the Ring has a dwarf galaxy progenitor, it might be found in the background of one out of eight specific areas in the sky. Here, we use Gaia DR2 data to perform a systematic exploration aimed at confirming or rejecting this remarkable prediction. Focusing on the values of the radial velocity to uncover possible multimodal spreads, we identify a bimodal Gaussian distribution towards Galactic coordinates (l, b) = (271°, +2°) in Vela, which is one of the locations of the progenitor proposed by Guglielmo et al. This prominent feature with central values 60 ± 7  and 97 ± 10 km s−1, may signal the presence of the long sought progenitor of the Monoceros Ring, but the data might also be compatible with the existence of an unrelated, previously unknown, kinematically coherent structure.
Sternocleidomastoid Encephalomyosynangiosis for Treatment-Resistant Moyamoya Disease
Authors: Chiarelli Peter A , Patel Anoop P , Lee Amy , Chandra Srinivasa R , Sekhar Laligam N 
Published: August 2018

Abstract

BACKGROUND AND IMPORTANCE
Refractory ischemic symptoms in moyamoya disease are a challenging problem, particularly in situations in which multiple direct and indirect revascularization techniques have already been employed. In addition, revascularization of the parietal lobes is difficult, as this area is a watershed between the middle cerebral artery and posterior cerebral artery distributions.

CLINICAL PRESENTATION
This is the case of a 50-yr-old woman with hemibody sensorimotor deficits, who had previously undergone bilateral arterial bypass and temporalis myosynangiosis. A method for indirect surgical cerebral revascularization is described, utilizing a rotated and tunneled sternocleidomastoid flap. The perfused muscle is approximated to the cortical surface, with adjacent sulci dissected to expose the underlying vasculature. After sternocleidomastoid encephalomyosynangiosis, the patient experienced symptomatic improvement, along with the appearance of new pial collateral vasculature on diagnostic cerebral angiography. Pre- and postoperative dynamic perfusion computed tomography with acetazolamide challenge demonstrate an increase in cerebral blood flow and decrease in mean transit time, as well as improved cerebrovascular reserve.

CONCLUSION
Sternocleidomastoid encephalomyosynangiosis using a tunneled muscle flap is a useful method for revascularization of the parietal and occipital lobes, particularly for refractory moyamoya in cases where a variety of other options have been exhausted.
Resection of a Lumbar Intradural Extramedullary Schwannoma: 3-Dimensional Operative Video
Authors: Dekker Simone E , Glenn Chad A , Ostergard Thomas A , Smith Mickey L , Rothstein Brian D , Ray Abhishek , Bambakidis Nicholas C 
Published: August 2018

Abstract
This 3-dimensional operative video illustrates resection of a lumbar schwannoma in a 57-yr-old female who presented with right lower extremity numbness, paresthesias, as well as a long history of lower back pain with rest. On magnetic resonance imaging (MRI), there was evidence of an intradural extramedullary enhancing lesion at L5, nearly completely encompassing the spinal canal. This video demonstrates the natural history, treatment options, surgical procedure, risks, and complications of treatment of these types of tumors. The patient underwent a posterior lumbar laminectomy with a midline dural opening for tumor resection. The tumor was encountered intradurally and electromyography recording confirmed that the tumor arose from a lumbar sensory nerve root. The sensory root was then divided and the tumor was then removed. The mass was removed en bloc and histopathologic analysis was consistent with a schwannoma. Postoperative MRI demonstrated gross total resection of the patient's neoplasm with excellent decompression of the spinal cord. The patient had an uneventful postoperative course with full recovery and complete resolution of her back pain and leg paresthesias.
Right Pretemporal-Transsylvian Approach for Resection of a Midbrain Cavernous Malformation: 3-Dimensional Operative Video
Authors: Mascitelli Justin R , Gandhi Sirin , Cavallo Claudio , Nanaszko Michael J , Wright Ernest J , Lawton Michael T 
Published: August 2018

Abstract
Brainstem cavernous malformations (CMs) account for 15% to 18% of all intracranial CMs
1
and 13% of all cerebrovascular pathology in the posterior fossa.
1,2
This video demonstrates the resection of a pontomesencephalic CM through a pretemporal approach through the oculomotor-tentorial triangle (OTT).
3
A 49-yr-old woman presented with an acute onset of left hemiparesis, diplopia, vertigo, partial oculomotor, and facial palsy. Neuroimaging revealed a 25-mm diameter right pontomesencephalic CM with evidence of prior hemorrhage. Institutional Review Board approval and patient consent were obtained for surgery. A right orbitozygomatic craniotomy was performed, and the lesion was exposed through a pretemporal-transsylvian approach. After a wide Sylvian fissure split, the oculomotor nerve (CN III) was dissected away from the temporal lobe, and the temporal lobe was mobilized posteriorly to access the OTT. The posterior cerebral and superior cerebellar arteries were visualized in this triangle, and the cerebral peduncle and the CM were accessed deep to these arteries. After hematoma evacuation, the CM was resected in a piece-meal fashion using an intracapsular technique. Postoperative imaging confirmed the gross total resection of the lesion. The patient had persistent right CN III palsy and a slight worsening of left hemiparesis, which had resolved completely at the 6-mo follow-up. The OTT provides access to the upper ventrolateral pontomesencephalic area.
3
This triangular surgical workspace is entered through a pretemporal-transsylvian corridor and widened with posterior temporal lobe retraction. The OTT is an important working space for accessing midbrain and upper pontine CMs posterolateral to CN III.