bluuebunny/crossref_metadata_2025_split huggingface dataset:
Slow Elevation in Protein C Activity without a PROC Mutation in a Neonate with Intracranial Hemorrhage
Authors: Uehara Erika , Nakao Hiro , Tsumura Yusuke , Nakadate Hisaya , Amari Shoichiro , Fujinaga Hideshi , Tsutsumi Yoshiyuki , Kang Dongchon , Ohga Shouichi , Ishiguro Akira 
Published: April 2018

AbstractSevere protein C (PC) deficiency leads to purpura fulminans and stroke in newborns. However, the clinical impact of plasma PC activity on the development of neonatal cerebral disease remains elusive. We report a case of hemorrhagic stroke associated with neonatal asphyxia and severe PC deficiency. Plasma PC and protein S activity 7 days after birth was 12% and 43%, respectively. No PROC mutation was found. PC levels did not exceed 20% until 2 months of age, even in the absence of consumption coagulopathy or vitamin K deficiency. Neither thromboembolic nor hemorrhagic events occurred during the infusion of activated PC concentrate (twice weekly, up to 68 days after birth). The PC activity levels gradually increased to the standard value for age by 9 months of age. The present case showed that neonatal PC deficiency without a PROC mutation caused an intracranial hemorrhage before a slow increase in PC activity.
Spezialisierte Ambulante Palliativ-Versorgung
Authors: Nauck Friedemann , Jansky Maximiliane 
Published: April 2018

AbstractSince 2007, patients with severe advanced life-limiting illnesses and high, complex symptom burdens have a right to receive specialized outpatient palliative care (SAPV). Multi-professional teams with heterogeneous organizational structures provide care in cooperation with primary care givers, not limited to cancer patients. The aim of SAPV is to foster patient’s autonomy and quality of life. SAPV can be provided as counseling of patient and care givers, coordination of care, additional supportive and full care provision. While the basis of SAPV provision is regulated by a SAPV directive, different contracts between care providers and health care insurances regulate organization, cooperation, definition of care levels, service provision and compensation. Some regions have model contracts that are binding for all SAPV teams in the area; in other regions teams negotiate e. g. compensation, individually with insurances. The article gives an overview of the regulations regarding SAPV.
COPD – Stellenwert der Lungenfunktionsanalyse in Diagnostik und Therapie
Authors: Windisch Wolfram , Criée Carl 
Published: April 2018

AbstractPulmonary function testing is essential for diagnosis and treatment-guidance of chronic obstructive pulmonary disease (COPD). Airway obstruction as assessed by spirometry should follow the reference-values provided by the Global Lung Initiative (GLI) of the European Respiratory Society (ERS). In addition, lung function testing should also include the assessment of lung hyperinflation and pulmonary emphysema by full-body plethysmography and determination of diffusion capacity. This is important since both, lung hyperinflation and pulmonary emphysema, can present without existing airway obstruction. Even though this formally excludes the diagnosis of COPD, these entities still belong to this disease complex. However, strictly speaking, pharmaceutical treatment is valid only for those patients with co-existing airway obstruction according to Global Lung Initiative for Chronic Obstructive Lung Disease (GOLD) criteria – since the absence of airway obstruction serves as exclusion criterion in nearly all randomized controlled trials. Nevertheless, progressive symptoms still require detailed pulmonary function testing for the guidance of non-pharmaceutical treatment – such as endoscopic or surgical lung volume reduction, long-term oxygen therapy, long-term non-invasive ventilation, and lung transplantation.
Fluchtwege von schwerkranken Kindern und Jugendlichen aus den ehemaligen Ostblockländern nach Deutschland zur Behandlung des Nierenversagens
Authors: Mammadova Dilbar , Marek Ines , Galiano Matthias , Rascher Wolfgang 
Published: April 2018

Zusammenfassung
Hintergrund Erhöhte Patientenmobilität und eingeschränkte Behandlungsmöglichkeiten von Kindern mit terminaler Niereninsuffizienz veranlassten Familien aus den ehemaligen Ostblockländern mit ihren Kindern zur Behandlung nach Deutschland zu fliehen.
Methode In einer Fallstudie wurden Krankenakten retrospektiv analysiert. In strukturierten Interviews wurden Eltern und Patienten nach dem Fluchtweg, der Behandlung und Integration befragt.
Ergebnisse Von 2003 bis 2013 kamen 8 Kinder und Jugendliche zur Behandlung eines terminalen Nierenversagens nach Erlangen. Die meisten Patienten kamen mithilfe eines Schleusers mit einem Touristenvisum. Auffallend oft gaben sie an, in der Aufregung ihre Papiere verloren zu haben. Eine Familie erhielt vom Schleuser neue Pässe mit gefälschten Namen und Geburtsdaten. Die Familien waren gezwungen, für die Flucht hohe Kosten auf sich zu nehmen, um durch die Behandlung in Deutschland das Leben ihres Kindes zu retten. Auch wenn sich die Dialysetherapie wegen eines anderen Krankheitsverständnisses oft schwierig gestaltete, war der Gesamtverlauf zufriedenstellend. Vier Patienten konnten mittlerweile erfolgreich transplantiert werden.
Schlussfolgerung Diese Fallstudie zeigt neue Facetten der Patientenmobilität, die durch die bessere medizinische Versorgung in Deutschland motiviert ist und das Ziel hat, das Überleben des Kindes zu sichern. Ein ethisches Problem ergab sich dadurch, dass eine Dialysebehandlung bei Kindern mit terminaler Niereninsuffizienz die Nierentransplantation als therapeutisches Ziel impliziert.
Experiences of care labour, gender and work for men who teach young children
Authors: O’Keeffe Suzanne 
Published: April 2018

AbstractThis paper explores five Irish male primary teachers’ daily experiences of care labour and gender in contemporary Irish schools. Taking a feminist poststructural approach, the study employs three data-collection phases using the interview as the primary method of enquiry. It employs a voice-centred relational method of data analysis, which involves four readings of data with each reading troubling the data in different ways. This paper places specific focus on three everyday phenomena: care, emotions and the body. The evolving dynamic between gender and work is discussed in terms of a socio-cultural tension that informs the experiences of men who work with young children. Overall, two major challenges are identified. First, emotions are considered as individual, internal and private responses to situations. Yet, we absorb the norms and values of our society in the form of social and cultural practices that preserve society, which bring emotions into line with the rules proposed by society. Second, teaching is considered a soft option career for men and an essentially feminised occupation rather than a masculine one. As softness is very often associated with weakness, primary teaching does not align with traditional views of masculinities that are built on rationality, individualisation and heroism. This is a further challenge for male teachers to care in schools. Overall, male teachers are required to reproduce accounts of themselves in terms of valued masculine attributes due to the historical association between women, emotionality and care.
Geographies of emotional and care labour
Authors: Parish Jessica , Montsion Jean Michel 
Published: April 2018

AbstractRecent years have witnessed shifts in the social organisation of emotional and care labour, especially as they intersect with new global trends in migratory patterns and international mobility, the restructuring of social reproduction and public—private divides, as well as the flexibilization of labour markets and a resurgence of unpaid labour such as volunteer work. With a focus on emotions and affect as a central epistemological and methodological orientation, this essay aims to draw connections between three distinct but related bodies of feminist scholarship: social reproduction theory, studies of emotional labour, and emotional geographies. The paper frames these approaches relative to the project of understanding the spatial dimensions of forms of emotional and care labour in neoliberal times.
« Articles contractés » ou « preposizioni articolate » ?
Authors: Sőrés Anna 
Published: April 2018

Abstract
The morphemes such as the French au or the Italien nel are designed by different terms in grammars of
Romance languages, which shows that it is difficult to identify the lexical category of this type of fusional morpheme. The aim of this
paper is to propose a detailed analysis of these contracted forms. I suggest that the fusion occurs during a secondary grammaticalization.
This process involves, on the one hand, the definite article, i.e. a grammatical element, and, on the other hand, some prepositions which,
semantically, can be functional or lexical but which function syntactically as grammatical elements. The analysis shows that case inflection
of nominals has been transferred to the preposition while the other categories appear in the article which merged with prepositions.
Therefore, the fusional forms can be considered as prepositions marked by the grammatical categories of gender, number and definiteness.
Trade costs, global value chains and economic development
Authors: Zi Yuan 
Published: April 2018

Abstract
This paper develops a model to study the impact of trade costs on developing countries’ industrialization when sequential production is networked in global value chains (GVCs). In a two-country setting, a decrease in trade costs of intermediates is associated with South joining and moving up the value chain and both North and South experiencing a welfare improvement. The wage gap between North and South first increases and then decreases. Extending the model to a multi-country setting, I show that reduced trade frictions lead to South countries joining GVCs due to wage differentials and low trade costs. This increases the wage in North but may decrease the wages of South countries that are already part of the network. Moreover, South countries that join tend to be regionally clustered. The model provides a first look at GVCs from the development angle, and raises policy questions regarding the governance of GVCs.
Community-Acquired Respiratory Paramyxovirus Infection After Allogeneic Hematopoietic Cell Transplantation: A Single-Center Experience
Authors: Spahr Yasmin , Tschudin-Sutter Sarah , Baettig Veronika , Compagno Francesca , Tamm Michael , Halter Jörg , Gerull Sabine , Passweg Jakob , Hirsch Hans H , Khanna Nina 
Published: April 2018

Abstract

Background
Paramyxoviruses include respiratory syncytial virus (RSV), parainfluenza virus (PIV), and human metapneumovirus (MPV), which may cause significant respiratory tract infectious disease (RTID) and mortality after allogeneic hematopoietic cell transplantation (HCT). However, clinical data regarding frequency and outcome are scarce.

Methods
We identified all paramyxovirus RTIDs in allogeneic HCT recipients diagnosed by multiplex polymerase chain reaction between 2010 and 2014. Baseline characteristics of patients, treatment, and outcome of each episode were analyzed; ie, moderate, severe, and very severe immunodeficiency (verySID) according to HCT ≤6 months, T- or B-cell depletion ≤3 months, graft-versus-host disease, neutropenia, lymphopenia, or hypo-gammaglobulinemia.

Results
One hundred three RTID episodes in 66 patients were identified (PIV 47% [48 of 103], RSV 32% [33 of 103], MPV 21% [22 of 103]). Episodes occurred in 85% (87 of 103) at &gt;100 days post-HCT. Lower RTID accounted for 36% (37 of 103). Thirty-nine percent (40 of 103) of RTID episodes required hospitalization and more frequently affected patients with lower RTID. Six percent progressed from upper to lower RTID. Overall mortality was 6% and did not differ between paramyxoviruses. Sixty-one percent (63 of 103) of episodes occurred in patients with SID, and 20.2% (19 of 63) of episodes occurred in patients with verySID. Oral ribavirin plus intravenous immunoglobulin was administered in 38% (39 of 103) of RTIDs, preferably for RSV or MPV (P ≤ .001) and for SID patients (P = .001). Patients with verySID frequently progressed to lower RTID (P = .075), required intensive care unit transfer, and showed higher mortality.

Conclusion
Paramyxovirus RTID remains a major concern in allogeneic HCT patients fulfilling SID and verySID, emphasizing that efficacious and safe antiviral treatments are urgently needed.
Topical Adjuncts to Pulsed Dye Laser for Treatment of Port Wine Stains: Review of the Literature
Authors: Lipner Shari R. 
Published: April 2018

BACKGROUND
Port wine stains (PWS) pose a therapeutic challenge. Pulsed dye laser (PDL) is the treatment of choice; however, treatment is often ineffective and recurrences are common.

OBJECTIVE
This article provides a review of topical therapies that have been investigated to improve efficacy of PDL for the treatment of PWS.

MATERIALS AND METHODS
A literature search was performed through PubMed, EMBASE, Web of Science, and CINAHL, using the search terms “port wine stain,” “pulsed dye laser,” and “topical.”

RESULTS
Clinical trials have investigated the topical agents, timolol, imiquimod, and rapamycin (RPM) in combination with PDL for the treatment of PWS. Topical timolol with PDL failed to show improved efficacy compared with PDL alone. Two clinical trials using imiquimod and PDL showed enhanced blanching of PWS compared with controls. Rapamycin and PDL were more effective than controls for facial PWS, but not for nonfacial PWS.

CONCLUSION
Topical imiquimod and RPM have shown some efficacy in treating PWS with PDL, but to date there is no topical adjuvant to PDL that reliably improves results for PWS.
Recommendations and Extraction of Clinical Variables of Pediatric Multiple Sclerosis Using Common Data Elements
Authors: Newland Pamela , Newland John M. , Hendricks-Ferguson Verna L. , Smith Judith M. , Oliver Brant J. 
Published: April 2018

ABSTRACT

Purpose: The purpose of this article was to demonstrate the feasibility of using common data elements (CDEs) to search for information on the pediatric patient with multiple sclerosis (MS) and provide recommendations for future quality improvement and research in the use of CDEs for pediatric MS symptom management strategies Methods: The St. Louis Children’s Hospital (SLCH), Washington University (WU) pediatrics data network was evaluated for use of CDEs identified from a database to identify variables in pediatric MS, including the key clinical features from the disease course of MS. The algorithms used were based on International Classification of Diseases, Ninth/Tenth Revision, codes and text keywords to identify pediatric patients with MS from a de-identified database. Data from a coordinating center of SLCH/WU pediatrics data network, which houses inpatient and outpatient records consisting of patients (N = 498 000), were identified, and detailed information regarding the clinical course of MS were located from the text of the medical records, including medications, presence of oligoclonal bands, year of diagnosis, and diagnosis code. Results: There were 466 pediatric patients with MS, with a few also having the comorbid diagnosis of anxiety and depression. Conclusions: St. Louis Children’s Hospital/WU pediatrics data network is one of the largest databases in the United States of detailed data, with the ability to query and validate clinical data for research on MS. Nurses and other healthcare professionals working with pediatric MS patients will benefit from having common disease identifiers for quality improvement, research, and practice. The increased knowledge of big data from SLCH/WU pediatrics data network has the potential to provide information for intervention and decision-making that can be personalized to the pediatric MS patient.
Autologous Bone Flap Resorption Years After Subtemporal Craniotomy
Authors: Obafunwa John Oladapo , Hammerl Emily , Jaskierny David , Taylor Livia , Russell Lynette , Reinhard Karl 
Published: April 2018

AbstractIn Spring of 2012, the partially undressed and skeletonized remains of a homeless adult Hispanic male was found in a fairly open wooded area in Nebraska. The remains showed evidence of extensive pathologies, which included healed traumas and surgeries. Examination of the decedent's medical records revealed that he had a history of kidney and liver problems, alcohol abuse, several traumas including a major head injury that necessitated a craniotomy, and radiological features of neurocysticercosis. The autologous bone flap, which was replaced after the craniotomy, had resorbed significantly away from the edges of the injury. Death was variously attributed to craniocerebral injury with hypothermia sequel to blunt force trauma most probably due to a fall. The manner of death was ruled as an accident. This study makes use of the forensic evidence and medical records to examine the possibility that the unique combination of failed cranioplasty, hypothermia, and neurocysticercosis may have contributed to the victim's death. Other potential causes of death are considered.
Disruption of the transcription factorNEUROD2causes an autism syndrome via cell-autonomous defects in cortical projection neurons
Authors: Runge Karen , Mathieu Rémi , Bugeon Stéphane , Lafi Sahra , Beurrier Corinne , Sahu Surajit , Schaller Fabienne , Loubat Arthur , Herault Leonard , Gaillard Stéphane , Cahuc Mélanie , Pallesi-Pocachard Emilie , Montheil Aurélie , Bosio Andreas , Rosenfeld Jill A , Hudson Eva , Lindstrom Kristin , Mercimek-Andrews Saadet , Jeffries Lauren , van Haeringen Arie , Vanakker Olivier , Pichon Bruno , Van Hecke Audrey , Amrom Dina , Küry Sebastien , Gamble Candace , Jacq Bernard , Fasano Laurent , Santpere Gabriel , Lorente-Galdos Belen , Sestan Nenad , Gelot Antoinette , Giacuzzo Sylvie , Represa Alfonso , Cardoso Carlos , Cremer Harold , de Chevigny Antoine 
Published: April 2018

AbstractWe identified seven families associatingNEUROD2pathogenic mutations with ASD and intellectual disability. To get insight into the pathophysiological mechanisms, we analyzed cortical development inNeurod2KO mice. Cortical projection neurons (CPNs) over-migrated during embryogenesis, inducing abnormal thickness and laminar positioning of cortical layers. At juvenile ages, dendritic spine turnover and intrinsic excitability were increased in L5 CPNs. Differentially expressed genes inNeurod2KO mice were enriched for voltage-gated ion channels, and the human orthologs of these genes were strongly associated with ASD. Furthermore, adultNeurod2KO mice exhibited core ASD-like behavioral abnormalities. Finally, by generatingNeurod2conditional mutant mice we demonstrate that forebrain excitatory neuron-specificNeurod2deletion recapitulates cellular and behavioral ASD phenotypes found in full KO mice. Our findings demonstrate crucial roles forNeurod2in cortical development and function, whose alterations likely account for ASD and related symptoms in the newly definedNEUROD2mutation syndrome.
CHOP and IRE1α-XBP1/JNK signaling promote Newcastle Disease Virus induced apoptosis and benefit virus proliferation
Authors: Li Yanrong , Liao Ying , Niu Qiaona , Gu Feng , Sun Yingjie , Meng Chunchun , Tan Lei , Song Cuiping , Qiu Xusheng , Ding Chan 
Published: April 2018

ABSTRACTNewcastle disease virus (NDV) causes severe infectious disease in poultry, and selectively kills tumor cells by inducing apoptosis. In this report, we revealed the mechanisms underlying NDV-induced apoptosis via investigation of endoplasmic reticulum (ER) stress-related unfolded protein response (UPR) in HeLa cells. We found that NDV infection induced the expression of pro-apoptotic transcription factor CHOP via PKR-eIF2α pathway. Knock down and exogenous expression studies showed that CHOP promoted cell apoptosis by down-regulation of anti-apoptotic protein BCL-2 and MCL-1, promotion of pro-apoptotic JNK and p38 signaling, and suppression of pro-survival AKT signaling. Meanwhile, CHOP facilitated NDV proliferation. Furthermore, virus infection activated IRE1α, another ER stress sensor, thereby promoting the mRNA splicing of XBP1 and resulting in the translation of transcription factor XBP1s. XBP1s entered into cell nucleus, promoted the expression of ER chaperones and components of ER associated degradation (ERAD). Exogenous expression of XBP1s helped IBV proliferation, and silence of XBP1s reduced virus proliferation. Meanwhile, exogenous expression and knock down studies demonstrated that IRE1α activated pro-apoptotic JNK signaling, promoted apoptosis and inflammation. In conclusion, our current study demonstrates that the induction of CHOP and activation of IRE1α-XBP1/JNK signaling cascades promote apoptosis and benefit NDV proliferation.IMPORTANCEIt is well known that NDV kills host animal and tumor cells by inducing cell apoptosis. Although several studies investigate the apoptotic phenomena in NDV-infected tumor cells, the molecular mechanisms underlying this oncolytic virus induced apoptosis is not well understood yet. In this study, we focus on characterization of the ER stress responses in NDV-infected tumor cells, and find that virus induces apoptosis by up-regulation or activation of several unfolded protein responses (UPR) related transcription factors and signaling: such as ATF4, CHOP and XBP1s, and pro-apoptotic kinases (IRE1α, JNK, p38). Moreover, activation of these transcription factors and signaling cascades helps virus proliferation. Our study dissects the UPR induced apoptosis in NDV-infected tumor cells, and provides the evidence that UPR favors NDV proliferation.
Mass Action Kinetic Model of Apoptosis by TRAIL-Functionalized Leukocytes
Authors: Lederman Emily E. , King Michael R. 
Published: April 2018

1 AbstractBackgroundMetastasis through the bloodstream contributes to poor prognosis in many types of cancer. A unique approach to target and kill colon, prostate, and other epithelial-type cancer cells in the blood has been recently developed that causes circulating leukocytes to present the cancer-specific, liposome-bound Tumor Necrosis Factor (TNF)-related apoptosis inducing ligand (TRAIL) on their surface along with E – selectin adhesion receptors. This approach, demonstrated both in vitro with human blood and in mice, mimics the cytotoxic activity of natural killer cells. The resulting liposomal TRAIL-coated leukocytes hold promise as an effective means to neutralize circulating tumor cells that enter the bloodstream with the potential to form new metastases.ResultsThe computational biology study reported here examines the mechanism of this effective signal delivery, by considering the kinetics of the coupled reaction cascade, from TRAIL binding death receptor to eventual apoptosis. In this study, a collision of bound TRAIL with circulating tumor cells (CTCs) is considered and compared to a prolonged exposure of CTCs to soluble TRAIL. An existing computational model of soluble TRAIL treatment was modified to represent the kinetics from a diffusion-limited 3D reference frame into a 2D collision frame with advection and adhesion to mimic the E – selectin and membrane bound TRAIL treatment. Thus, the current model recreates the new approach of targeting cancer cells within the blood. The model was found to faithfully reproduce representative observations from experiments of liposomal TRAIL treatment under shear. The model predicts apoptosis of CTCs within 2 hr when treated with membrane bound TRAIL, while apoptosis in CTCs treated with soluble TRAIL proceeds much more slowly over the course of 10 hrs, consistent with previous experiments. Given the clearance rate of soluble TRAIL in vivo, this model predicts that the soluble TRAIL method would be rendered ineffective, as found in previous experiments.ConclusionThis study therefore indicates that the kinetics of the coupled reaction cascade of liposomal E – selectin and membrane bound TRAIL colliding with CTCs can explain why this new approach to target and kill cancer cells in blood is much more effective than its soluble counterpart.