bluuebunny/crossref_metadata_2025_split huggingface dataset:
Frontotemporal dementia and progressive supranuclear palsy‐like syndrome with a novel TARDBP mutation
Authors: Yabe Ichiro , Nakano Fumihito , Shirai Shinichi , Matsushima Masaaki , Takahashi Ikuko , Sasaki Hidenao 
Published: January 2016

AbstractTARDBP mutation has been shown to cause frontotemporal dementia and progressive supranuclear palsy‐like disease. In this case study, we present the first reported Japanese case of frontotemporal dementia and progressive supranuclear palsy‐like syndrome caused by a novel TARDBP mutation. A 63‐year‐old man was diagnosed with frontotemporal dementia and progressive supranuclear palsy‐like disease. An older brother of the patient's deceased father showed similar symptoms. Genetic analysis showed a novel TARDBP heterozygous mutation (c.715 A > G, p.I239V). This mutation is not found in databases of the 1000 genome project and Human Genomic Variation, but is identified as a pathogenic mutation by Mutation Taster. Although the hot cross bun sign as a result of frontotemporal dementia and progressive supranuclear palsy has been reported only rarely, brain magnetic resonance imaging of this patient showed the hot cross bun sign and T2‐hyperintensity of the middle cerebellar peduncles along with frontotemporal and midbrain tegmentum atrophy.
Medication‐related osteonecrosis of the jaw associated with bisphosphonates and denosumab in osteoporosis
Authors: Bagan J , Peydró A , Calvo J , Leopoldo M , Jiménez Y , Bagan L 
Published: January 2016

ObjectiveTo describe the clinical characteristics and evolution of our series of medication‐related osteonecrosis of the jaws (MRONJ) associated with denosumab in osteoporotic patients.Material and MethodsWe present 10 new cases of MRONJ in patients receiving denosumab for osteoporosis. We describe the mean doses of denosumab, previous bisphosphonate intake, and the clinical characteristics associated with the osteonecrosis, such as local contributing factors, symptoms, and evolution after treatment.ResultsThe mean number of denosumab doses was 3.4 ± 2.2. In 90% of patients, there was a prior history of oral bisphosphonate intake, with a mean duration of 46.78 ± 25.11 months. The most common local factor was dental extraction (6 cases; 60%), and most cases had necrotic bone exposure (9/10, 90%). Sclerosis of the bone was the most common radiographic finding. Stage 1 was the most common ONM stage, found in 80%. ‘Cure’ after conservative treatments was obtained in 71.4%.ConclusionsMost of our cases were in the early stages of MRONJ, and the success rate after conservative treatment was high.
A new family ofCambrian rhynchonelliformean brachiopods (OrderNaukatida) with an aberrant coral‐like morphology
Authors: Streng Michael , Butler Aodhán D. , Peel John S. , Garwood Russell J. , Caron Jean‐Bernard 
Published: January 2016

AbstractTomteluva perturbatagen. et sp. nov. andNasakia thulensisgen. et sp. nov., two new rhynchonelliformean brachiopod taxa, are described from carbonate beds from the lower middleCambrian (Series 3,Stage 5) basinalStephenFormation,Canada, and the upper lowerCambrian (Series 2,Stage 4)HensonGletscherFormation,NorthGreenland, respectively. The two taxa are characterized by an unusual coral‐like morphology typified by a high conical ventral valve with an anteriorly curved umbo and a tube‐like structure inside the ventral valve, interpreted as pedicle tube. Both resemble the problematic late middleCambrian (Drumian) speciesAnomalocalyx cawoodiBrock fromAustralia, whose systematic affiliation is controversial. Together, the three genera are interpreted as representatives of a new family of rhynchonelliformean brachiopods, theTomteluvidae fam. nov., which is interpreted as an aberrant or derived taxon within theOrderNaukatida. Convergence between theTomteluvidae and the coralla of small solitaryCambrian coralimorphs, as well as the latePalaeozoic reef‐building richthofenioid brachiopods, might indicate adaptation to a similar life habits and environments. However, their small size (length 4 mm), well‐developed pedicle and perfect morphological symmetry make it more likely that tomteluvids lived attached to frondose algae or sponges, above the seafloor, in a similar fashion to the acrotretoid brachiopods with which they show a high degree of morphological convergence. Morphological features of the pedicle tube ofN. thulensissuggest that the tomteluvid pedicle is homologous to that in modern rhynchonelliformean brachiopods. This is the first evidence of the pedicle type within the Naukatida and represents the oldest confirmation of a rhynchonellate pedicle.
Ellis–van Creveld syndrome associated with chronic intestinal pseudo‐obstruction
Authors: Iwakura Hideo , Fujii Katsunori , Furutani Yoshiyuki , Takatani Tomozumi , Ebata Ryota , Nakanishi Toshio , Mitsunaga Tetsuya , Saito Takeshi , Kishimoto Takashi , Yoshida Hideo , Shimojo Naoki 
Published: January 2016

AbstractEllis–van Creveld (EVC) syndrome is a rare autosomal recessive disorder characterized by hypoplastic nails, polydactyly, and achondroplasia. Patients usually exhibit normal cognitive function and no remarkable developmental delay. We herein present an unusual case of EVC syndrome. A Japanese 2‐year‐old boy was born at term, but immediately developed severe respiratory failure due to thorax deformity, postaxial polydactyly and nail hypoplasia. We identified a novel pattern of germinal compound heterozygous nonsense EVC2 mutations of c.1814C > A (p. S605X) and c.2653C > T (p. R885X), leading to the diagnosis of EVC syndrome. Interestingly, he also had severe developmental delay, and suddenly developed excessive abdominal distension at the age of 2. On surgery, extensive necrotic bowel with chronic intestinal pseudo‐obstruction was noted. This is, to our knowledge, a most severe phenotype of EVC syndrome, illustrating that the specific pattern of EVC2 compound heterozygous mutations may cause severe developmental delay and intestinal malfunction.
Specificity of Chroomonas (Cryptophyceae) as a source of kleptochloroplast for Nusuttodinium aeruginosum (Dinophyceae)
Authors: Onuma Ryo , Horiguchi Takeo 
Published: January 2016

SummaryThe unarmoured dinoflagellate Nusuttodinium aeruginosum retains a kleptochloroplast, which is a transient chloroplast stolen from members of the cryptomonad genus, Chroomonas. Both N. aeruginosum and the closely related N. acidotum have been shown to restrict their diet to a limited number of species of this blue‐green genus of cryptophyte. However, it is still unclear how flexible the predators are with regard to the ingestion and utilization of Chroomonas spp. as a source of kleptochloroplast. To address specificity of cryptomonad in N. aeruginosum, we collected the cells of N. aeruginosum from several ponds in Japan, and analysed the phylogeny of the kleptochloroplasts based on their plastidial 16S rDNA sequences. All sequences obtained in this study were restricted to only one (the subclade 4) of four subclades known to comprise the Chroomonas/Hemiselmis clade. Therefore, N. aeruginosum is specific in its dietary requirements, selecting their prey within the subclade level.
Effectiveness of low‐dose pregabalin in three patients with Lewy body disease and central neuropathic pain
Authors: Ukai Katsuyuki , Fujishiro Hiroshige , Ozaki Norio 
Published: January 2016

AbstractMany patients with Lewy body disease complain of pain, and their pain may be associated with this disease. Recently, pain has become a focus of attention in Parkinson's disease, but there is little information regarding pain in patients who have dementia with Lewy bodies. We used pregabalin to treat three Lewy body disease patients with chronic pain that may have been related to degeneration of central neurons. All three patients responded well to pregabalin at 25–50 mg/day. To our knowledge, there have been no previous reports of pregabalin showing efficacy for central neuropathic pain in Parkinson's disease or Lewy body disease.
Extensive and Intensive Margins of Exports and Labor Heterogeneity
Authors: Luong Tuan Anh , Chen Wei‐Chih 
Published: January 2016

AbstractThis paper investigates how changes in skilled and unskilled labor supply affect different margins of exports. Using bilateral trade data in manufacturing sectors of 34 countries from 1995 to 2010, we find that most of the impact of skilled labor on exports goes through the intensive margin, whereas most of the effect of unskilled labor works through the extensive margin. These outcomes result from the impact of labor skill composition on the productivity cut‐off of exporters. We also find that the impact of skilled and unskilled labor on trade margins depends on the income level of countries and on the type of products. The results indicate that the effect of skilled labor is greater for low‐income countries and differentiated products, while that of unskilled labor is greater for high‐income countries and homogeneous products.
Economic Integration, Monopoly Power and Productivity Growth without Scale Effects
Authors: Davis Colin , Hashimoto Ken‐ichi 
Published: January 2016

AbstractThis paper considers how monopoly power affects the relationship between economic integration and economic growth that is not biased by a scale effect. In a two‐country model of trade, productivity growth is generated by firm‐level investment in process innovation, and the location of economic activity is determined by relative market size, trade costs and imperfect knowledge diffusion. Equilibrium features the partial concentration of manufacturing and the full concentration of innovation in the larger country. Increased economic integration raises the concentration of manufacturing in the larger country, and when monopoly power is strong, leads to decreased product variety, accelerated productivity growth and greater national welfare. With weak monopoly power, however, it raises product variety and dampens productivity growth, but may benefit or hurt welfare.
Intellectual Property Rights and the Quality of Transferred Technology in Developing Countries
Authors: Yang Lei , Tsai Yingyi , Mukherjee Arijit 
Published: January 2016

AbstractThis paper addresses the effects of a stronger patent system in developing countries on the quality of transferred technology and welfare. We show that a stronger patent system can reduce the quality of licensed technology. The presence of technology licensing may encourage the developing country to adopt a stronger patent system compared with the situation where licensing is not an option.
Capital Structure Premium in Multinational SOEs: Evidence from China
Authors: Zhang Rui , Zhang Xun 
Published: January 2016

AbstractWe investigate the relationship among multinational operations, ownership and capital structure using data from China's A‐share listed companies. We find that, in general, multinational enterprises (MNEs) have lower leverages than domestic enterprises (DEs). More importantly, we document a capital structure premium in China's multinational state‐owned enterprises (SOEs). Since the state supports multinational SOEs that promote overseas national strategy, these multinational SOEs will have higher credit availability and therefore higher debt–equity ratios. This study sheds light on the Chinese government's impact on firm's creditability.
Regionalism as Industrial Policy: Evidence from MERCOSUR
Authors: Moncarz Pedro , Olarreaga Marcelo , Vaillant Marcel 
Published: January 2016

AbstractThis paper empirically explores whether trade preferences can be used as a substitute for industrial policy and help countries achieve their industrialization objectives at the expense of other regional members. Results show that Mercado Común del Sur (MERCOSUR) preferences obtained by Brazilian exporters have led to an increase in exports of relatively sophisticated products in which Brazil does not enjoy a global comparative advantage. On the contrary, smaller members of MERCOSUR export to the region products in which they have strong comparative advantages and with relatively low levels of sophistication. This suggests that MERCOSUR has helped Brazil achieve its industrialization objectives, but has not contributed to the industrialization of its smaller members.
The first case of acute unilateral pan‐ureteritis caused by BK polyomavirus in an allogeneic stem cell transplant patient
Authors: Nigo M. , Marin D. , Mulanovich V.E. 
Published: January 2016

AbstractSeveral cases of ureteral obstruction have been reported in stem cell transplant (SCT) patients; however, they were bilateral and concomitant with or preceded by hemorrhagic cystitis. We describe, to our knowledge, a first case of acute unilateral pan‐ureteritis caused by BK polyomavirus (BKPyV) in an SCT patient. This case may represent an early phase of BKPyV reactivation. BKPyV infection should be considered as a potential cause of acute unilateral ureteritis even among SCT recipients.
Decreasing child mortality, spatial clustering and decreasing disparity in North‐Western Burkina Faso
Authors: Becher Heiko , Müller Olaf , Dambach Peter , Gabrysch Sabine , Niamba Louis , Sankoh Osman , Simboro Seraphin , Schoeps Anja , Stieglbauer Gabriele , Yé Yazoume , Sié Ali 
Published: January 2016

SummaryWithin relatively small areas, there exist high spatial variations of mortality between villages. In rural Burkina Faso, with data from 1993 to 1998, clusters of particularly high child mortality were identified in the population of the Nouna Health and Demographic Surveillance System (HDSS), a member of the INDEPTH Network. In this paper, we report child mortality with respect to temporal trends, spatial clustering and disparity in this HDSS from 1993 to 2012. Poisson regression was used to describe village‐specific child mortality rates and time trends in mortality. The spatial scan statistic was used to identify villages or village clusters with higher child mortality. Clustering of mortality in the area is still present, but not as strong as before. The disparity of child mortality between villages has decreased. The decrease occurred in the context of an overall halving of child mortality in the rural area of Nouna HDSS between 1993 and 2012. Extrapolated to the Millennium Development Goals target period 1990–2015, this yields an estimated reduction of 54%, which is not too far off the aim of a two‐thirds reduction.
Biologics in pregnancy – for the obstetrician
Authors: Soh May Ching , MacKillop Lucy 
Published: January 2016

Key content

The authors discuss commencing biologics in pregnancy and the potential side effects. Infection is a particular risk, which may be atypical or present atypically.

While there is no evidence of teratogenicity, these drugs cross the placenta and neonatal cord drug levels may exceed those of maternal drug levels.

If possible, anti‐tumour necrosis factor (TNF) agents should be discontinued midtrimester but the gestation to which each drug can be used is slightly different.

Neonates exposed in utero to anti‐TNF agents beyond the recommended gestation should not be given live attenuated vaccines for the first 6 months of life and any infections promptly treated.

Data are lacking on the long‐term effects of biologics on children exposed in utero. However, the benefits of controlling active autoimmune disease in the mother often outweigh the small risk to the exposed infants.

Learning objectives

To know the biologics most commonly used in pregnancy.

To understand the literature regarding safety of the common biologics in pregnancy.

To be able to counsel a woman taking a biologic about the potential benefits and risks of taking it at different gestations.

To be able to counsel a woman receiving biologics in pregnancy about vaccinations for her baby after birth.

Ethical issues

Biologic agents are relatively new drugs that are being increasingly used in young women because of their perceived ‘fertility sparing’ benefits despite a lack of high quality evidence.

Most women who have been prescribed biologics are often refractory to other treatments, and therefore these agents are required for disease control. The decision to continue or discontinue these drugs in pregnancy, especially late pregnancy, is particularly contentious.

The long‐term effect of biologics on infants exposed in utero is not known and the decision to use these drugs has to be balanced against the risk of severe maternal flares.
An online x‐ray based position validation system for prostate hypofractionated radiotherapy
Authors: Arumugam Sankar , Sidhom Mark , Xing Aitang , Holloway Lois 
Published: January 2016

Purpose:Accurate positioning of the target volume during treatment is paramount for stereotactic body radiation therapy (SBRT). In this work, the authors present the development of an in‐house software tool to verify target position with an Elekta‐Synergy linear accelerator using kV planar images acquired during treatment delivery.Methods:In‐house software, SeedTracker, was developed in matlab to perform the following three functions: 1. predict intended seed positions in a planar view perpendicular to any gantry angle, simulating a portal imaging device, from the 3D seed co‐ordinates derived from the treatment planning system; 2. autosegment seed positions in kV planar images; and 3. report the position shift based on the seed positions in the projection images. The performance of SeedTracker was verified using a CIRS humanoid phantom (CIRS, VA, USA) implanted with three Civco gold seed markers (Civco, IA, USA) in the prostate. The true positive rate of autosegmentation (TPRseg) and the accuracy of the software in alerting the user when the isocenter position was outside the tolerance (TPRtrig) were studied. Two‐dimensional and 3D static position offsets introduced to the humanoid phantom and 3D dynamic offsets introduced to a gel phantom containing gold seeds were used for evaluation of the system.Results:SeedTracker showed a TPRseg of 100% in the humanoid phantom for projection images acquired at all angles except in the ranges of 80°–100° and 260°–280° where seeds are obscured by anatomy. This resulted in a TPRtrig of 88% over the entire treatment range for considered 3D static offsets introduced to the phantom. For 2D static offsets where the position offsets were only introduced in the anterior–posterior and lateral directions, the TPRtrig of SeedTracker was limited by both seed detectability and positional offset. SeedTracker showed a false positive trigger in the projection angle range between 130°–170° and 310°–350° (a maximum of 24% of treatment time) due to limited information that can be derived from monoscopic images. The system accurately determined the dynamic trajectory of the isocenter position in the superior and inferior direction for the studied dynamic offset scenarios based on the seed position in monoscopic images.Conclusions:The developed software has been shown to accurately autosegment the seed positions in kV planar images except for two 20° arcs where seeds are obscured by anatomical structures. The isocenter trajectories determined by the system, based on the monoscopic images, provide useful information for monitoring the prostate position. The developed system has potential application for monitoring prostate position during treatment delivery in linear accelerator based SBRT.
Decolonization in Prevention of Health Care-Associated Infections
Authors: Septimus Edward J. , Schweizer Marin L. 
Published: January 2016

SUMMARY

Colonization with health care-associated pathogens such as
Staphylococcus aureus
, enterococci, Gram-negative organisms, and
Clostridium difficile
is associated with increased risk of infection. Decolonization is an evidence-based intervention that can be used to prevent health care-associated infections (HAIs). This review evaluates agents used for nasal topical decolonization, topical (e.g., skin) decolonization, oral decolonization, and selective digestive or oropharyngeal decontamination. Although the majority of studies performed to date have focused on
S. aureus
decolonization, there is increasing interest in how to apply decolonization strategies to reduce infections due to Gram-negative organisms, especially those that are multidrug resistant. Nasal topical decolonization agents reviewed include mupirocin, bacitracin, retapamulin, povidone-iodine, alcohol-based nasal antiseptic, tea tree oil, photodynamic therapy, omiganan pentahydrochloride, and lysostaphin. Mupirocin is still the gold standard agent for
S. aureus
nasal decolonization, but there is concern about mupirocin resistance, and alternative agents are needed. Of the other nasal decolonization agents, large clinical trials are still needed to evaluate the effectiveness of retapamulin, povidone-iodine, alcohol-based nasal antiseptic, tea tree oil, omiganan pentahydrochloride, and lysostaphin. Given inferior outcomes and increased risk of allergic dermatitis, the use of bacitracin-containing compounds cannot be recommended as a decolonization strategy. Topical decolonization agents reviewed included chlorhexidine gluconate (CHG), hexachlorophane, povidone-iodine, triclosan, and sodium hypochlorite. Of these, CHG is the skin decolonization agent that has the strongest evidence base, and sodium hypochlorite can also be recommended. CHG is associated with prevention of infections due to Gram-positive and Gram-negative organisms as well as
Candida
. Conversely, triclosan use is discouraged, and topical decolonization with hexachlorophane and povidone-iodine cannot be recommended at this time. There is also evidence to support use of selective digestive decontamination and selective oropharyngeal decontamination, but additional studies are needed to assess resistance to these agents, especially selection for resistance among Gram-negative organisms. The strongest evidence for decolonization is for use among surgical patients as a strategy to prevent surgical site infections.
Progress toward a Respiratory Syncytial Virus Vaccine
Authors: Neuzil Kathleen M. 
Published: January 2016

ABSTRACT

In accompanying papers (P. L. Acosta, M. T. Caballero, and F. P. Polack, Clin Vaccine Immunol 23:189–195, 2016,
http://dx.doi.org/10.1128/CVI.00609-15
; M. Vissers, I. M. L. Ahout, M. I. de Jonge, and G. Ferwerda, Clin Vaccine Immunol 23:243–245, 2016,
http://dx.doi.org/10.1128/CVI.00590-15
) in this issue of
Clinical and Vaccine Immunology
, the history of and immune mechanisms underlying vaccine-enhanced respiratory syncytial virus (RSV) disease and of investigations of mucosal antibodies and their association with viral load in RSV-infected children, respectively, are described. This commentary discusses RSV vaccine candidates, target populations, and the challenges associated with achieving a safe and effective vaccine.