bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Efficacy of Recombinant FVIII Low-Dose Prophylaxis in Chinese Pediatric Patients With Severe Hemophilia A: A Retrospective Analysis From the ReCARE Study
Authors: Yao Wanru , Xiao Juan , Cheng Xiaoling , Feng Guoshuang , Li Changgang , Zhang Xinsheng , Hu Qun , Xu Weiqun , Sun Jing , Yang Renchi , Li Xiaojing , Zhou Rongfu , Lian Shimei , Gu Jian , Wu Junde , Zhao Yongqiang , Wu Runhui 
Published: December 2016

Objective: This study explores the efficacy of recombinant factor VIII (rFVIII) low-dose prophylaxis in Chinese pediatric patients with severe hemophilia A from the Retrospective Study in Chinese Pediatric Hemophilia A Patients With rFVIII Contained Regular Prophylaxis (ReCARE) population. Methods: This is additional analysis of the multicenter, retrospective ReCARE study, in which the annual bleeding rate (ABR), annual joint bleeding rate (AJBR), and safety of >12-week, low dose (10-30 IU/kg/wk) rFVIII prophylaxis divided into primary, secondary, and tertiary groups based on the joint status and joint bleeding history were analyzed. Results: A total of 57 patients (median age: 8.2 [0.4-17.3] years) from the ReCARE study receiving primary (n = 3), secondary (n = 21), and tertiary (n = 33) prophylaxes were included. Low-dose prophylaxis had significant bleeding reduction in all 3 groups compared to the baseline ( S = 408.5, P < .001), with median ABR rates of −4.0 (−8.0 to −3.1), −4.0 (−24.0 to 8.0), and −13.9 (−110.6 to 20.6) in the primary, secondary, and tertiary groups, respectively, with a significant difference between the secondary and tertiary groups ( P = .008). Median AJBR reduction rates were −2.3 (−6.3 to 8.4) and −14.9 (−61.5 to 19.1) in the secondary and tertiary groups, respectively. But there was no significant difference in AJBRs between the secondary and tertiary groups ( P = .061), which was related to damaged joint status. Hence, longer prophylaxis was associated with better prevention of joint bleeding ( P = .024). Conclusion: Despite significant ABR/AJBR reduction in all 3 groups, the efficacy of the primary prophylaxis was better than the secondary and tertiary prophylaxes.
Injectable Diphenhydramine Used as Anaesthetic for Wide Local Excision With Flap Closure
Authors: Pickett Laura E. , Macdonald Jillian 
Published: December 2016

Background: Lidocaine-allergic patients pose a challenge for cutaneous surgical procedures. We present a case of a successful wide local excision with flap closures on the face using topical tetracaine followed by infiltration with 1% diphenhydramine. Case Summary: An 84-year-old woman, with a lidocaine intolerance, presented to the Mohs surgery clinic for wide local excision of a nodular basal cell carcinoma on her right medial cheek. Successful anaesthesia was achieved using topical 4% tetracaine followed by intradermal and subcutaneous injection of 1% diphenhydramine. Haemostasis was achieved with both electrocautery and suture ligation. Sliding rotation flaps were closed using both deep and superficial sutures. Other than some mild discomfort and temporary hyperemia on injection, the patient tolerated the procedure well with no long-term complications. Conclusion: Diphenhydramine is a very useful alternative in lidocaine-intolerant patients for cutaneous procedures including punch and shave biopsies, cyst removals, small elliptical excisions, and wide local excisions.
Dorsal intercalated segmental instability associated with malunion of a reconstructed scaphoid
Authors: Kim J.-H. , Lee K.-H. , Lee B. G. , Lee C.-H. , Kim S.-J. , Choi W.-S. 
Published: December 2016

We analysed scaphoid deformity as a result of surgical treatment of scaphoid fracture nonunion and assessed the deformity associated with a dorsal intercalated segmental instability pattern of carpal malalignment. A total of 45 patients who were treated for scaphoid fracture nonunion were included in the study. The height-to-length ratio of the scaphoid was measured on computed tomographic images and used to assess scaphoid deformity. Carpal malalignment was quantified based on the radio-lunate angle. A correlation analysis between the height-to-length ratio and the radio-lunate angle was performed. Dorsal intercalated segmental instability was defined as a radio-lunate angle >15°, and a receiver operating curve analysis was used to calculate the cutoff height-to-length ratio that can be accompanied with dorsal intercalated segmental instability. Extension of the lunate increases in proportion to the flexion deformity of the scaphoid; dorsal intercalated segmental instability can occur if the height-to-length ratio of the scaphoid is >0.73. Level of evidence: IV
Genetic modification of ER-Hoxb8 osteoclast precursors using CRISPR/Cas9 as a novel way to allow studies on osteoclast biology
Authors: Di Ceglie Irene , van den Akker Guus G H , Ascone Giuliana , ten Harkel Bas , Häcker Hans , van de Loo Fons A J , Koenders Marije I , van der Kraan Peter M , de Vries Teun J , Vogl Thomas , Roth Johannes , van Lent Peter L E M 
Published: December 2016

Abstract
Osteoclasts are cells specialized in bone resorption. Currently, studies on murine osteoclasts are primarily performed on bone marrow–derived cells with the use of many animals and limited cells available. ER-Hoxb8 cells are conditionally immortalized monocyte/macrophage murine progenitor cells, recently described to be able to differentiate toward functional osteoclasts. Here, we produced an ER-Hoxb8 clonal cell line from C57BL/6 bone marrow cells that strongly resembles phenotype and function of the conventional bone marrow–derived osteoclasts. We then used CRISPR/Cas9 technology to specifically inactivate genes by biallelic mutation. The CRISPR/Cas9 system is an adaptive immune system in Bacteria and Archaea and uses small RNAs and Cas nucleases to degrade foreign nucleic acids. Through specific-guide RNAs, the nuclease Cas9 can be redirected toward any genomic location to genetically modify eukaryotic cells. We genetically modified ER-Hoxb8 cells with success, generating NFATc1−/− and DC-STAMP−/− ER-Hoxb8 cells that lack the ability to differentiate into osteoclasts or to fuse into multinucleated osteoclasts, respectively. In conclusion, this method represents a markedly easy highly specific and efficient system for generating potentially unlimited numbers of genetically modified osteoclast precursors.
Approach to the Patient With HyperCKemia
Authors: Venance Shannon L. 
Published: December 2016

ABSTRACT

Purpose of Review: Neurologists commonly receive consultation requests regarding the evaluation of patients with an elevated serum creatine kinase (CK), a condition known as hyperCKemia. This article outlines an approach to the history and examination of patients with hyperCKemia in order to narrow the localization and differential of an elevated CK and guide possible next steps. This article aims to help clinicians identify treatable or reversible etiologies as well as those that will change management.

Recent Findings: An unrevealing patient history (assessing for acquired and hereditary etiologies) in an otherwise neurologically intact individual who has a normal nerve conduction study and EMG predicts that the likelihood of diagnosing the patient after further investigations will be quite low. After a comprehensive workup, a positive diagnosis is made in approximately 25% of cases of hyperCKemia.

Summary: The best predictors for added diagnostic yield with further testing in hyperCKemia are a higher level of CK and a younger age; the presence of weakness increases the likelihood of a specific cause other than idiopathic or familial hyperCKemia. Many etiologies do not yet have treatments that alter clinical outcomes, and, even in the absence of a specific diagnosis, good communication with patients and primary care providers remains essential to ensure longitudinal surveillance with expectant management for potential consequences. Many patients with hyperCKemia of uncertain etiology, however, will not develop significant muscle disease on longitudinal follow-up.
Facioscapulohumeral Muscular Dystrophy
Authors: Statland Jeffrey M. , Tawil Rabi 
Published: December 2016

ABSTRACT

Purpose of Review: This article describes the clinical characteristics, diagnosis, molecular pathogenesis, and treatment of facioscapulohumeral muscular dystrophy (FSHD).

Recent Findings: FSHD comprises two genetically distinct types that converge on a common downstream pathway of the expression of the toxic protein DUX4. Approximately 95% of patients have FSHD type 1 (FSHD1), in which loss of DNA repetitive elements (D4Z4 repeats) in the subtelomeric region of chromosome 4q causes decreased methylation and epigenetic derepression of DUX4, a gene contained within each D4Z4 repeat. FSHD type 2 (FSHD2) occurs through a deletion-independent mechanism but, similar to FSHD1, leads to decreased methylation and epigenetic derepression in the same region of chromosome 4q. Whereas FSHD1 is dominantly inherited, FSHD2 shows digenic inheritance, and about 80% of patients will have a mutation in the SMCHD1 gene. DUX4 lacks a polyadenylation signal, so both FSHD1 and FSHD2 only occur in the presence of permissive 4q polymorphisms, which provide a stabilizing polyadenylation sequence. FSHD is an epigenetic disease, and penetrance and severity are related to both the number of residual D4Z4 units and D4Z4 methylation.

Summary: Recent consensus guidelines outline standards for care for FSHD, and identification of potential therapeutic targets have shifted emphasis in the research community toward drug development and clinical trial planning.
Book Reviews
Authors: Shen Wenjing 
Published: December 2016

In Book Reviews, we review an extensive and diverse range of books. They cover theory and applications in operations research, statistics, management science, econometrics, mathematics, computers, and information systems. In addition, we include books in other fields that emphasize technical applications. The editor will be pleased to receive an email from those willing to review a book, with an indication of specific areas of interest. If you are aware of a specific book that you would like to review, or that you think should be reviewed, please contact the editor. The following books are reviewed in this issue of Interfaces, 46(6), November–December 2016: Only Humans Need Apply, Thomas H. Davenport and Julia Kirby; Success and Luck: Good Fortune and the Myth of Meritocracy, Robert H. Frank; and Multiobjective Linear Programming, An Introduction, Dinh The Luc.
Controlled drugs and the principle of double effect: the role of the district nurse
Authors: Griffith Richard 
Published: December 2016

The role of district nurses in the effective management of pain in palliative care has been strengthened by the Misuse of Drugs (Amendment No.2) (England, Wales and Scotland) Regulations 2012 that allow district nurses who are independent or supplementary prescribers to prescribe and administer controlled drugs. However, prescribing controlled drugs brings increased responsibility and accountability for the safe management of these medicines. In this article Richard Griffith considers the principle of double effect that seeks to ensure that patients in intractable pain receive the analgesia they require to manage that pain while district nurses avoid liability and prosecution under the law of murder.
„Im Deutschen kan das nicht“ – Text type didactics for the teaching of German modal verb constructions
Authors: Jentges Sabine 
Published: December 2016

Abstract
In the Netherlands, the similar origin Dutch shares with German lends itself to a contrastive approach to teaching and learning German and, for this reason, is promoted on all levels. This leads to Dutch textbooks and grammars being almost exclusively contrastive in nature, irrespective of the targeted language level, which is also reflected in the unanimous scientific debate. This paper discusses to which degree construction grammar approaches and/or contrastive approaches can assist the Dutch learner of German in their language acquisition. Advanced Dutch learners of German’s use - or lack thereof - of sollen ‘are supposed to’ vs. sollten ‘should’ in written texts is analyzed and compared to the presentation of this topic in Dutch teaching materials for German as a foreign language. Furthermore, the questions are raised whether a contrastive approach mainly promotes the strategy of avoiding errors stemming from L1 inferences as well as whether a construction grammar approach leads to a more adequate acquisition of the target language. Based on these discussions, possible implementations for teaching soll(t)en to Dutch learners of German are proposed.
Frame-based instruction: Teaching polysemous nouns in the L2
Authors: Kemsies Ronald 
Published: December 2016

Abstract
Across the various L2 teaching methodologies, polysemy represents a mostly neglected phenomenon. Due to the widespread belief that multiple meaning extensions are largely arbitrary as well as due to the lack of a systematic teaching method, rote learning of polysemous word meanings frequently appears to be a common practice in L2 classrooms. A cognitive semantic view of polysemy claims that polysemous meaning extensions are motivated and form a systematic network (Lakoff 1987). Against this backdrop, this article introduces a CL-based learning/teaching technique in order to systematically approximate the intended meanings of polysemous L2 nouns in context. ‘Frame-based instruction’ capitalizes on the notion of frame-inherent ‘slots’ and ‘fillers’ (cf., e.g., Barsalou 1992; Martin 1997; Minsky 1975). According to this view, polysemy arises through context- dependent slot-filler configurations. The method grants L2 learners access to these structures through a systematic array of questions scanning generic slots within the contextual frame and the polyseme-frame. In doing so, it guides them to unveil the intended polysemous word meaning in context in a step-by-step fashion. After an illustration of the method on the basis of a practical example, an empirical study is presented which tested the functionality of frame-based instruction in a classroom setting with 13-year-old EFL learners in Austria. Although the experiment produced a statistical null result, several future avenues of research crystallized with regard to an improved implementation of the method.
Gesture as a window onto conceptualization in multiple tasks: Implications for second language teaching
Authors: Stam Gale 
Published: December 2016

Abstract
This paper demonstrates that it is necessary to look at learners’ gestures and speech to have a complete picture of their conceptualizations in their L2. It argues that context and task affect learners’ speech and gesture and need to be considered in SLA. A comparison of the speech and gesture of an L2 learner in two tasks (an oral proficiency interview and a narration task) over a fourteen-year period of time shows that fewer gestures per clause and primarily metaphoric gestures were produced in the oral proficiency task and more gestures per clause and primarily iconic gestures were produced in the narration. Implications for second language teaching are discussed.
Modelling of imbibition phenomena in two-phase fluid flow through fractured porous media
Authors: Patel Hardik S. , Meher Ramakanta 
Published: December 2016

AbstractIn this paper, the counter-current imbibition phenomenon in two phase fluid through fracture porous media is discussed and Adomian decomposition method is applied to find the saturation of wetting phase and the recovery rate of the reservoir. A simulation result is developed for the saturation of wetting phase in fracture matrix and in porous matrix for some interesting choices of parametric value to study the recovery rate of the oil reservoir with dimensionless time. This problem has a great importance in the oil recovery process.
MultiLing. Center for Multilingualism in Society across the Lifespan: A Center of Excellence in Norway
Authors: Lanza Elizabeth , Lane Pia , Røyneland Unn 
Published: December 2016

Abstract
The world is connecting in many complex ways, driven by the globally integrated nature of technological innovation and human mobility across continents. Never before have so many different speakers and languages existed side by side in the OECD countries as they do today. This reality has placed multilingualism in the spotlight. Increased migration and transcultural flows across borders in Europe have highlighted the urgency for research on multilingualism in the individual and society. Academic institutions have recognized the necessity for such knowledge and research centers have evolved to meet this challenge (cf. Obermayer et al. 2014). MultiLing - the Center for Multilingualism in Society across the Lifespan is a research center aimed at academic excellence and financed by the Research Council of Norway through its Center of Excellence scheme. MultiLing is hosted by the Department of Linguistics and Scandinavian Studies at the Faculty of Humanities of the University of Oslo in Norway. It opened in June of 2013 and is currently in its first five-year period of operation.
The main goal of the Center is to generate state-of-the-art scientific knowledge on individual and societal multilingualism across the lifespan that will address the challenges and potentials multilingualism poses for the individual in the family, school, other institutions, and society in general. Moreover, the Center aims at providing research-based knowledge on multilingualism to central policymakers and stakeholders. Language planning and the standardization of languages are some of the Center’s key research foci and are indeed vital issues today at the top of the agenda for policymakers.
The Center’s vision is to contribute to how society can deal with the challenges of multilingualism through increased knowledge, promoting agency for individuals in society, and a better quality of life, no matter what linguistic and social background.
Three Small-Receptive-Field Ganglion Cells in the Mouse Retina Are Distinctly Tuned to Size, Speed, and Object Motion
Authors: Jacoby Jason , Schwartz Gregory W. 
Published: December 2016

Retinal ganglion cells (RGCs) are frequently divided into functional types by their ability to extract and relay specific features from a visual scene, such as the capacity to discern local or global motion, direction of motion, stimulus orientation, contrast or uniformity, or the presence of large or small objects. Here we introduce three previously uncharacterized, nondirection-selective ON–OFF RGC types that represent a distinct set of feature detectors in the mouse retina. The three high-definition (HD) RGCs possess small receptive-field centers and strong surround suppression. They respond selectively to objects of specific sizes, speeds, and types of motion. We present comprehensive morphological characterization of the HD RGCs and physiological recordings of their light responses, receptive-field size and structure, and synaptic mechanisms of surround suppression. We also explore the similarities and differences between the HD RGCs and a well characterized RGC with a comparably small receptive field, the local edge detector, in response to moving objects and textures. We model populations of each RGC type to study how they differ in their performance tracking a moving object. These results, besides introducing three new RGC types that together constitute a substantial fraction of mouse RGCs, provide insights into the role of different circuits in shaping RGC receptive fields and establish a foundation for continued study of the mechanisms of surround suppression and the neural basis of motion detection.SIGNIFICANCE STATEMENTThe output cells of the retina, retinal ganglion cells (RGCs), are a diverse group of ∼40 distinct neuron types that are often assigned “feature detection” profiles based on the specific aspects of the visual scene to which they respond. Here we describe, for the first time, morphological and physiological characterization of three new RGC types in the mouse retina, substantially augmenting our understanding of feature selectivity. Experiments and modeling show that while these three “high-definition” RGCs share certain receptive-field properties, they also have distinct tuning to the size, speed, and type of motion on the retina, enabling them to occupy different niches in stimulus space.
Binomial sampling of Paraleyrodes Quaintance pos. bondari (Hemiptera: Aleyrodidae) in Persea americana Mill.
Authors: Caicedo R. Luis Sigifredo , Varón D. Edgar Herney , Brochero Helena Luisa 
Published: December 2016

Fresno (Tolima), in Colombia, is a notable avocado producer, with 36% of the national production. In this paper, two sampling methods are presented to assess natural populations of Paraleyrodes Quaintance pos. bondari attacking avocado trees of Hass and Lorena cultivars under field conditions. The presence/absence of whitefly nymph colonies on 30 leaves located at the high, medium and low strata per host plant from both cultivars was evaluated. Visual estimations were performed to count the number ofwhitefly nymphs on 1.25 cm2 of five leaves/ bud in low and medium strata per tree to evaluate the spatial distribution of whitefly population in accordance to Poisson distribution, Negative Binomial distribution and b parameter of Law of Taylor. Significant differences in percentages of infestation (P≤0.03) from leaves that belonged to the low avocado tree strata were found between the Lorena (31.88±1.2%) and Hass (15.64±1.8%) cultivars. Natural populations of P. pos. bondari were located on the abaxial leaf side, showing an aggregate distribution in avocado tree from orchards located at different altitudes. Our findings recommend entomological surveillance for Paraleyrodes sp. pos. bondari in Fresno (Tolima), sampling four branches from the medium and low avocado tree strata through inspection of five buds/branches/tree throughout each branch with the presence/absence method to count whitefly nymph colonies on the abaxial side of pre-basal leaves. In total, the sampling involved five leaves/branch (20 leaves/strata or 40 leaves/tree) on 13 avocado trees per hectare.
Morphological evaluation of an in situ collection of species from the Annonaceae family in Colombia
Authors: Castañeda-Garzón Sandra Liliana , Ligarreto M. Gustavo Adolfo , Miranda-Lasprilla Diego 
Published: December 2016

An in situ collection composed of 167 accessions of the species Annona cherimola, A. glabra, A. reticulata, and A. squamosa, Rollinia sp., and the interspecific hybrid A. squamosa x A. cherimola was established through bioprospecting of genetics resources of the Annonaceae family in the Colombian Andean and Caribbean regions. These accessions were distributed in 13 departments and 48 municipalities. The most representative species of this collection are A. squamosa and A. reticulata, which are derived from traditional cultivars of populated centers with a variable altitude range of up to 1,021 m a.s.l. The morpho-agronomic evaluation was carried out on 98 accessions using 25 qualitative and quantitative descriptors, which identified the phenotypic traits in A. squamosa and A. reticulata that are related to fruit quality, such as size, weight, symmetry, degrees Brix, pH, acidity, seed weight and seed number, which are the more discriminating descriptors, differentiating six clusters in A. squamosa and four clusters in A. reticulata.