bluuebunny/crossref_metadata_2025 huggingface dataset:
Inflation, Employment, and Monetary Policy: Objectives and Outcomes in the UK and U.S. Compared
Authors: MILES DAVID
Published: September 2014

This paper explores how sensitive is monetary policy to the precise preferences of the central bank over inflation and economic activity. It does so in order to address a puzzle—which is that the U.S. Fed and the Bank of England appear to have quite different objectives and yet have adopted strikingly similar policies in recent years. I use a calibrated model to assess how policy might be sensitive to attaching different weights to inflation, output, and the output gap in central bank objectives. I find that a wide range of weights can give rise to rather similar monetary policies.
Multiple Time‐Intensity Analysis and Temporal Dominance of Sensations of Chocolate Dairy Dessert Using Prebiotic and Different High‐Intensity Sweeteners
Authors: Morais E.C. , Pinheiro A.C.M. , Nunes C.A. , Bolini H.M.A.
Published: September 2014

This paper presents a novel concept for producing chocolate dairy desserts using prebiotic and sucrose substitutes. The quality of chocolate dairy desserts was analyzed by the multiple time‐intensity analysis and temporal dominance of sensations (TDS). Time‐intensity analysis showed that the sample developed with neotame had a higher intensity of sweetness; the samples with neotame and stevia had a higher intensity of bitterness; and the samples with sucrose, sucralose, aspartame and the integral one had a higher intensity of chocolate flavor. Sucralose and aspartame provided a temporal profile with parameter curves closer to the one sweetened with sucrose, as well as the addition of prebiotics. TheTDSanalysis also showed a similar profile between the integral sample and the prebiotic light samples with sucralose and aspartame, in relation to the attributes of sweetness, bitterness, milk chocolate flavor, bittersweet chocolate flavor, milk powder, cream and off‐flavor. In this context, the addition of prebiotic and replacement of sucrose by sweeteners opens up new opportunities in product development, especially in chocolate formulation for dietetic and functional purposes.

Practical Applications

Temporal dominance of sensations (TDS) has led to improve a better understanding of temporality behavior of dairy desserts' taste and flavor. In this study, the time‐intensity analysis allowed the verification of changes in the perception of a product's attribute over time whileTDSprovided how the flavor behavior is for consumers during the dairy dessert ingestion and obtained the temporal profile of all attributes related to flavor. These methodologies are complementary and they are very useful for dairy dessert processors and people who work in the functional and sweetener industry.
Melatonin enhances the human mesenchymal stem cells motility via melatonin receptor 2 coupling with Gαq in skin wound healing
Authors: Lee Sei‐Jung , Jung Young Hyun , Oh Sang Yub , Yun Seung Pil , Han Ho Jae
Published: September 2014

Melatonin, a circadian rhythm–promoting molecule, has a variety of biological functions, but the functional role of melatonin in the motility of mesenchymal stem cells (MSCs) has yet to be studied. In a mouse skin excisional wound model, we found that transplantation of umbilical cord blood (UCB)‐MSCs pretreated with melatonin enhanced wound closure, granulation, and re‐epithelialization at mouse skin wound sites, where relatively more UCB‐MSCs which were engrafted onto the wound site were detected. Thus, we identified the signaling pathway of melatonin, which affects the motility of UCB‐MSCs. Melatonin (1 μm) significantly increased the motility of UCB‐MSCs, which had been inhibited by the knockdown of melatonin receptor 2 (MT2). We found that Gαq coupled with MT2 and that the binding of Gαq to MT2 uniquely stimulated an atypical PKC isoform, PKCζ. Melatonin induced the phosphorylation of FAK and paxillin, which were concurrently downregulated by blocking of the PKC activity. Melatonin increased the levels of active Cdc42 and Arp2/3, and it has the ability to stimulate cytoskeletal reorganization‐related proteins such as profilin‐1, cofilin‐1, and F‐actin in UCB‐MSCs. Finally, a lack of MT2 expression in UCB‐MSCs during a mouse skin transplantation experiment resulted in impaired wound healing and less engraftment of stem cells at the wound site. These results demonstrate that melatonin signaling via MT2 triggers FAK/paxillin phosphorylation to stimulate reorganization of the actin cytoskeleton, which is responsible for Cdc42/Arp2/3 activation to promote UCB‐MSCs motility.
Overexpression of MzASMT improves melatonin production and enhances drought tolerance in transgenic Arabidopsis thaliana plants
Authors: Zuo Bixiao , Zheng Xiaodong , He Pingli , Wang Lin , Lei Qiong , Feng Chao , Zhou Jingzhe , Li Qingtian , Han Zhenhai , Kong Jin
Published: September 2014

Melatonin is a potent naturally occurring reactive oxygen species (ROS) and reactive nitrogen species (RNS) scavenger in plants. Melatonin protects plants from oxidative stress and, therefore, it improves their tolerance against a variety of environmental abiotic stressors. N‐acetylserotonin‐O‐methyltransferase (ASMT) is a specific enzyme required for melatonin synthesis. In this report, an ASMT gene was cloned from apple rootstock (Malus zumi Mats) and designated as MzASMT1 (KJ123721). The MzASMT1 expression was induced by drought stress in apple leaves. The upregulation of MzASMT1 in the apple leaf positively relates to melatonin production over a 24‐hr dark/light cycle. Purified MzASMT1 protein expressed in E. coli converted its substrates to melatonin with an activity of approximately 5.5 pmol/min/mg protein. The transient transformation in tobacco identified that MzASMT1 is located in cytoplasm of the cell. When MzASMT1 gene driven by 35S promoter was transferred to Arabidopsis, melatonin levels in transgenic Arabidopsis plants were 2–4 times higher than those in the wild type. The transgenic Arabidopsis plants had significantly lower intrinsic ROS than the wild type and therefore these plants exhibited greater tolerance to drought stress than that of wild type. This is, at least partially, attributed to the elevated melatonin levels resulting from the overexpression of MzASMT1. The results elucidated the important role that membrane‐located melatonin synthase plays in drought tolerance. These findings have significant implications in agriculture.
Melatonin attenuates hypoxic pulmonary hypertension by inhibiting the inflammation and the proliferation of pulmonary arterial smooth muscle cells
Authors: Jin Haifeng , Wang Yueyue , Zhou Lei , Liu Lu , Zhang Peng , Deng Wuguo , Yuan Yuhui
Published: September 2014

Hypoxia‐induced inflammation and excessive proliferation of pulmonary artery smooth muscle cells (PASMCs) play important roles in the pathological process of hypoxic pulmonary hypertension (HPH). Melatonin possesses anti‐inflammatory and antiproliferative properties. However, the effect of melatonin on HPH remains unclear. In this study, adult Sprague–Dawley rats were exposed to intermittent chronic hypoxia for 4 wk to mimic a severe HPH condition. Hemodynamic and pulmonary pathomorphology data showed that chronic hypoxia significantly increased right ventricular systolic pressures (RVSP), weight of the right ventricle/left ventricle plus septum (RV/LV+S) ratio, and median width of pulmonary arterioles. Melatonin attenuated the elevation of RVSP, RV/LV+S, and mitigated the pulmonary vascular structure remodeling. Melatonin also suppressed the hypoxia‐induced high expression of proliferating cell nuclear antigen (PCNA), hypoxia‐inducible factor‐1α (HIF‐1α), and nuclear factor‐κB (NF‐κB). In vitro, melatonin concentration‐dependently inhibited the proliferation of PASMCs and the levels of phosphorylation of Akt and extracellular signal‐regulated kinases1/2 (ERK1/2) caused by hypoxia. These results suggested that melatonin might potentially prevent HPH via anti‐inflammatory and antiproliferative mechanisms.
Protective effect of melatonin on cigarette smoke‐induced restenosis in rat carotid arteries after balloon injury
Authors: Yang Gen‐Huan , Li Yan‐Chuan , Wang Zhan‐Qi , Liu Bao , Ye Wei , Ni Leng , Zeng Rong , Miao Shi‐Ying , Wang Lin‐Fang , Liu Chang‐Wei
Published: September 2014

Vascular restenosis after the interventional angioplasty remains the main obstacle to a favorable long‐term patency. Many researches suggest cigarette smoking is one of the most important causes of restenosis. This study was designed to investigate whether melatonin could protect against the cigarette smoke‐induced restenosis in rat carotid arteries after balloon injury. Three groups of male rats (normal condition, cigarette smoke exposed, cigarette smoke exposed, and melatonin injected) were used in this study. An established balloon‐induced carotid artery injury was performed, and the carotid arteries were harvested from these three groups 14 days later. The ratio of intima to media, the infiltration of inflammatory cells, the expression of inflammatory cytokines (NF‐κB, IL‐1β, IL‐6, TNF‐α, MCP‐1), adhesion molecules (ICAM‐1, VCAM‐1), and eNOS were measured. The results showed that cigarette smoke exposure aggravated the stenosis of the lumen, promoted the infiltration of inflammatory cells and induced the expression of the inflammatory cytokines and adhesion molecules after the balloon‐induced carotid artery injury. Moreover, cigarette smoke exposure can inhibit the expression of eNOS. Particularly, we surprised that melatonin could minimize this effect caused by cigarette smoke. These results suggested that melatonin could prevent the cigarette smoke‐induced restenosis in rat carotid arteries after balloon injury and the mechanism of its protective effect may be the inhibition of the inflammatory reaction. This also implies melatonin has the potential therapeutic applicability in prevention of restenosis after the vascular angioplasty in smokers.
Selective pressure on synonymous codon usage in mammalian protein‐encoding genes
Authors: ZHANG Xin‐Bo , MA Xiang‐Ming , WANG Bai‐Yun , MA Xiang‐Hui , WANG Zhi‐Wen
Published: September 2014

The evolutionary suppression of synonymous codon sites is a controversial topic. Although some studies have indicated that synonymous substitution is under positive selection, most of these studies relied on comparison of homologous genes and/or a limited number of sequences. In the present work, we compared the selection strength at synonymous sites for two types of protein‐encoding genes: genes encoding enzymes and protein genes encoding non‐enzymes. Our method does not require assumptions concerning, for example, evolutionary equilibrium or population size. We compared ∼70 000 genes from the fully sequenced mammalian Homo sapiens, Pan troglodytes, Mus musculus, and Rattus norvegicus genomes and found that the percentage of C and G in the third position of a codon positively correlates with the percentage of the G/C content within ± 20 000 nucleotides of the gene. More interestingly, we found that synonymous sites in mammalian genes encoding enzymes have undergone stronger selection than did such sites in genes encoding proteins that are not enzymes.
Clinical, Pathologic, and Immunohistochemical Prognostic Factors in Dogs with Thyroid Carcinoma
Authors: Campos M. , Ducatelle R. , Rutteman G. , Kooistra H.S. , Duchateau L. , de Rooster H. , Peremans K. , Daminet S.
Published: September 2014

Background

Prognostic markers for dogs with thyroid tumors are limited.

Hypothesis/Objectives

To identify clinical, pathologic, and immunohistochemical prognostic factors for dogs with thyroid tumors.

Animals

Seventy dogs with thyroid neoplasia.

Methods

Retrospective study. Dogs with thyroid neoplasia were included when follow‐up information and formalin‐fixed paraffin‐embedded tumor samples were available. Immunohistochemistry (IHC) was performed for thyroglobulin, calcitonin, Ki‐67, and E‐cadherin. Correlation of tumor variables (diameter, volume, localization, scintigraphic uptake, thyroid function,IHC) with local invasiveness and metastatic disease was performed on all tumor samples. Forty‐four dogs treated by thyroidectomy were included in a survival analysis.

Results

Fifty dogs (71%) had differentiated follicular cell thyroid carcinoma (dFTC) and 20 (29%) had medullary thyroid carcinoma (MTC). At diagnosis, tumor diameter (P = .007;P = .038), tumor volume (P = .020), tumor fixation (P = .002), ectopic location (P = .002), follicular cell origin (P = .044), and Ki‐67 (P = .038) were positively associated with local invasiveness; tumor diameter (P = .002), tumor volume (P = .023), and bilateral location (P = .012) were positively associated with presence of distant metastases. Forty‐four dogs (28dFTC, 16 MTC; stage I–III) underwent thyroidectomy. Outcome was comparable between dogs withdFTCandMTC. Macroscopic (P = .007) and histologic (P = .046) vascular invasion were independent negative predictors for disease‐free survival. Although time to presentation, histologic vascular invasion and Ki‐67 were negatively associated with time to metastases, and time to presentation was negatively associated with time to recurrence, no independent predictors were found. E‐cadherin expression was not associated with outcome.

Conclusions and Clinical Importance

Prognostic factors have been identified that provide relevant information for owners and clinicians.
Risk Factors for Survival in a University Hospital Population of Dogs with Epilepsy
Authors: Fredsø N. , Koch B.C. , Toft N. , Berendt M.
Published: September 2014

Background

Although a common neurological disorder in dogs, long‐term outcome of epilepsy is sparsely documented.

Objectives

To investigate risk factors for survival and duration of survival in a population of dogs with idiopathic epilepsy or epilepsy associated with a known intracranial cause.

Animals

One hundred and two client owned dogs; 78 dogs with idiopathic epilepsy and 24 dogs with epilepsy associated with a known intracranial cause.

Methods

A retrospective hospital based study with follow‐up. Dogs diagnosed with epilepsy between 2002 and 2008 were enrolled in the study. Owners were interviewed by telephone using a structured questionnaire addressing epilepsy status, treatment, death/alive, and cause of death.

Results

Median life span was 7.6 years, 9.2 years, and 5.8 years for all dogs, and dogs with idiopathic epilepsy or dogs with epilepsy associated with a known intracranial cause (P < .001), respectively. Survival time for dogs with idiopathic epilepsy was significantly (P = .0030) decreased for dogs euthanized because of epilepsy (median: 35 months) compared to dogs euthanized for other reasons (median: 67.5 months). Neutered male dogs with idiopathic epilepsy had a significant (P = .031) shorter survival (median: 38.5 months) after index seizure compared to intact male dogs (median: 71 months). Treatment with two antiepileptic drugs (AED′s) did not negatively influence survival (P = .056).

Conclusion and Clinical Importance

Dogs with idiopathic epilepsy can in many cases expect a life span close to what is reported for dogs in general. In dogs where mono‐therapy is not sufficient, the need for treatment with two AED′s is not linked to a poor prognosis.
Game Theory and the Evolution of Meaning
Authors: Franke Michael , Wagner Elliott O.
Published: September 2014

Evolutionary game theory is a general, but mathematically precise framework for modeling the competition between and fitness‐based selection of different types of behavior. We review recent applications of this framework to account for the evolution of behavior that lends meaning to ostensible acts and signs.
Hierarchical population structure and habitat differences in a highly mobile marine species: the Atlantic spotted dolphin
Authors: Viricel Amélia , Rosel Patricia E.
Published: September 2014

Recent molecular studies have shown that highly mobile species with continuous distributions can exhibit fine‐scale population structure. In this context, we assessed genetic structure within a marine species with high dispersal potential, the Atlantic spotted dolphin (Stenella frontalis). Using 19 microsatellite loci and mitochondrial control region sequences, population structure was investigated in the western North Atlantic, the Gulf of Mexico and the Azores Islands. Analyses of the microsatellite data identified four distinct genetic clusters, which were supported by the control region sequences. The highest level of divergence was seen between two clusters corresponding to previously described morphotypes that inhabit oceanic and shelf waters. The combined morphological and genetic evidence suggests these two lineages are on distinct evolutionary trajectories and could be considered distinct subspecies despite their parapatry. Further analysis of the continental shelf cluster resulted in three groups: animals inhabiting shelf waters in the western North Atlantic, the eastern Gulf of Mexico and the western Gulf of Mexico. Analyses of environmental data indicate the four genetic clusters inhabit distinct habitats in terms of depth and sea surface temperature. Contemporary dispersal rate estimates suggest all of these populations should be considered as distinct management units. Conversely, no significant genetic differentiation was observed between S. frontalis from offshore waters of the western North Atlantic and the Azores, which are separated by approximately 4500 km. Overall, the hierarchical structure observed within the Atlantic spotted dolphin shows that the biogeography of the species is complex because it is not shaped solely by geographic distance.
The transcriptional repressor EUO regulates both subsets of Chlamydia late genes
Authors: Rosario Christopher J. , Hanson Brett R. , Tan Ming
Published: September 2014

The pathogenic bacterium Chlamydia replicates in a eukaryotic host cell via a developmental cycle marked by temporal waves of gene expression. We have previously shown that late genes transcribed by the major chlamydial RNA polymerase, σ66 RNA polymerase, are regulated by a transcriptional repressor EUO. We now report that EUO also represses promoters for a second subset of late genes that are transcribed by an alternative polymerase called σ28 RNA polymerase. EUO bound in the vicinity of six σ28‐dependent promoters and inhibited transcription of each promoter. We used a mutational analysis to demonstrate that the EUO binding site functions as an operator that is necessary and sufficient for EUO‐mediated repression of σ28‐dependent transcription. We also verified specific binding of EUO to σ66‐dependent and σ28‐dependent promoters with a DNA immunoprecipitation assay. These findings support a model in which EUO represses expression of both σ66‐dependent and σ28‐dependent late genes. We thus propose that EUO is the master regulator of late gene expression in the chlamydial developmental cycle.
Bifidobacteria exert strain‐specific effects on stress‐related behavior and physiology in BALB/c mice
Authors: Savignac H. M. , Kiely B. , Dinan T. G. , Cryan J. F.
Published: September 2014

Background

Accumulating evidence suggests that commensal bacteria consumption has the potential to have a positive impact on stress‐related psychiatric disorders. However, the specific bacteria influencing behaviors related to anxiety and depression remain unclear. To this end, we compared the effects of two different Bifidobacteria on anxiety and depression‐like behavior; an antidepressant was also used as a comparator.

Methods

Innately anxious BALB/c mice received daily Bifidobacterium longum (B.) 1714, B. breve 1205, the antidepressant escitalopram or vehicle treatment for 6 weeks. Behavior was assessed in stress‐induced hyperthermia test, marble burying, elevated plus maze, open field, tail suspension test, and forced swim test. Physiological responses to acute stress were also assessed.

Key Results

Both Bifidobacteria and escitalopram reduced anxiety in the marble burying test; however, only B. longum 1714 decreased stress‐induced hyperthermia. B. breve 1205 induced lower anxiety in the elevated plus maze whereas B. longum 1714 induced antidepressant‐like behavior in the tail suspension test. However, there was no difference in corticosterone levels between groups.

Conclusions & Inferences

These data show that these two Bifidobacteria strains reduced anxiety in an anxious mouse strain. These results also suggest that each bacterial strain has intrinsic effects and may be beneficially specific for a given disorder. These findings strengthen the role of gut microbiota supplementation as psychobiotic‐based strategies for stress‐related brain‐gut axis disorders, opening new avenues in the field of neurogastroenterology.
The Eucalyptus grandis R2R3‐MYB transcription factor family: evidence for woody growth‐related evolution and function
Authors: Soler Marçal , Camargo Eduardo Leal Oliveira , Carocha Victor , Cassan‐Wang Hua , San Clemente Hélène , Savelli Bruno , Hefer Charles A. , Paiva Jorge A. Pinto , Myburg Alexander A. , Grima‐Pettenati Jacqueline
Published: September 2014

The R2R3‐MYB family, one of the largest transcription factor families in higher plants, controls a wide variety of plant‐specific processes including, notably, phenylpropanoid metabolism and secondary cell wall formation. We performed a genome‐wide analysis of this superfamily in Eucalyptus, one of the most planted hardwood trees world‐wide.

A total of 141 predicted R2R3‐MYB sequences identified in the Eucalyptus grandis genome sequence were subjected to comparative phylogenetic analyses with Arabidopsis thaliana, Oryza sativa, Populus trichocarpa and Vitis vinifera. We analysed features such as gene structure, conserved motifs and genome location. Transcript abundance patterns were assessed by RNAseq and validated by high‐throughput quantitative PCR.

We found some R2R3‐MYB subgroups with expanded membership in E. grandis, V. vinifera and P. trichocarpa, and others preferentially found in woody species, suggesting diversification of specific functions in woody plants. By contrast, subgroups containing key genes regulating lignin biosynthesis and secondary cell wall formation are more conserved across all of the species analysed.

In Eucalyptus, R2R3‐MYB tandem gene duplications seem to disproportionately affect woody‐preferential and woody‐expanded subgroups. Interestingly, some of the genes belonging to woody‐preferential subgroups show higher expression in the cambial region, suggesting a putative role in the regulation of secondary growth.
Genome engineering via TALENs and CRISPR/Cas9 systems: challenges and perspectives
Authors: Mahfouz Magdy M. , Piatek Agnieszka , Stewart Charles Neal
Published: September 2014

The ability to precisely modify genome sequence and regulate gene expression patterns in a site‐specific manner holds much promise in plant biotechnology. Genome‐engineering technologies that enable such highly specific and efficient modification are advancing with unprecedented pace. Transcription activator‐like effectors (TALEs) provide customizable DNA‐binding modules designed to bind to any sequence of interest. Thus, TALEs have been used as a DNA targeting module fused to functional domains for a variety of targeted genomic and epigenomic modifications. TALE nucleases (TALENs) have been used with much success across eukaryotic species to edit genomes. Recently, clustered regularly interspaced palindromic repeats (CRISPRs) that are used as guide RNAs for Cas9 nuclease‐specific digestion has been introduced as a highly efficient DNA‐targeting platform for genome editing and regulation. Here, we review the discovery, development and limitations of TALENs and CRIPSR/Cas9 systems as genome‐engineering platforms in plants. We discuss the current questions, potential improvements and the development of the next‐generation genome‐editing platforms with an emphasis on producing designer plants to address the needs of agriculture and basic plant biology.
Munc13‐4 deficiency with CD5 downregulation on activated CD8+ T cells
Authors: Wada Taizo , Yasumi Takahiro , Toma Tomoko , Hori Masayuki , Maeda Sayaka , Umeda Katsutsugu , Heike Toshio , Adachi Souichi , Usami Ikuya , Yachie Akihiro
Published: September 2014

Familial hemophagocytic lymphohistiocytosis (FHL) is characterized by uncontrolled activation of T cells and macrophages and hypercytokinemia. We have recently described a significant increase in a subpopulation of CD8+ T cells with downregulation of CD5 during the acute phase of FHL type2 (FHL2; perforin deficiency), which declines after successful treatment, with a concomitant reduction in serum cytokine level. This unusual subset of CD8+ T cells, however, has not been characterized in patients with other subtypes of FHL. Herein, we describe a patient with FHL3 (Munc13‐4 deficiency) carrying compound heterozygous mutations in the UNC13D gene. He had high serum levels of pro‐inflammatory cytokines and significantly increased activated CD8+ T cells with downregulation of CD5 during the acute phase, similar to that found in FHL2. This immunophenotypic feature may serve as a useful marker of immune dysregulation in FHL3 in addition to FHL2.
Ex utero intrapartum treatment for an infant with cerebro‐costo‐mandibular syndrome
Authors: Ogasawara Kei , Honda Yoshinobu , Hosoya Mitsuaki
Published: September 2014

Cerebro‐costo‐mandibular syndrome (CCMS) is a rare disorder characterized by multiple rib abnormalities, micrognathia described as Pierre–Robin sequence, and cerebral involvement. Appropriate management of respiratory distress immediately after birth is crucial to rescue these patients. A boy, having a mother with Pierre–Robin sequence and a sister with CCMS, was diagnosed prenatally with CCMS and successfully treated with ex utero intrapartum treatment (EXIT) at 36 weeks 6 days of gestation. EXIT would be an effective option for rescuing patients with prenatally diagnosed CCMS and preventing neonatal hypoxia.
Streptococcus pulmonary empyema after varicella infection in a serologically immunocompetent boy
Authors: Yamaguchi Hiroshi , Nagumo Kiyoshi , Sasaki Daisuke , Aoyagi Hayato , Kato Hiroaki , Narita Yoshiaki , Tamura Takuya , Kosugiyama Kiyotaka , Nakashima Taiji , Kinugawa Yoshikazu
Published: September 2014

Varicella zoster virus (VZV) is the etiologic agent of varicella, and it remains common among children in Japan due to low vaccination rates. It can cause a variety of serious and life‐threatening complications. Generally, the most frequent complication of varicella in healthy children is bacterial superinfection, but empyema after VZV infection is a rare condition. This case report describes a previously healthy 21‐month‐old boy who attended nursery school with a recent varicella and group A β‐hemolytic streptococcus (GABHS) pharyngitis outbreak and who presented with a 7 day history of vesicular rash along with progressive fever. Due to continued mild cough and prolonged fever, however, chest radiography was done, which showed a right pleural effusion. Further computed tomography showed a right pulmonary empyema, and purulent material was drained and eventually grew GABHS. This report hereby describes the development of pleural empyema caused by GABHS after VZV infection in a serologically immunocompetent patient.
Acute lymphoblastic leukemia in a girl with Wilson's disease
Authors: Maeda Sayaka , Matsubara Hiroshi , Hiejima Eitaro , Tanaka Atsushi , Okada Masayuki , Kato Itaru , Umeda Katsutsugu , Hiramatsu Hidefumi , Watanabe Ken‐ichiro , Heike Toshio , Adachi Souichi
Published: September 2014

Wilson's disease (WD) is an autosomal recessive defect in cellular copper transportation. Although acute lymphoblastic leukemia (ALL) is the most common form of childhood malignancy, only two cases of ALL associated with WD have been reported to date. One patient died of relapse and infection, and the other died of neutropenic sepsis during the treatment. We here describe the case of a 10‐year‐old girl with WD and ALL. Adverse events of chemotherapy, including liver toxicity and severe myelosuppression, necessitated adjustments in the chemotherapy doses. After completion of the treatment, the patient has remained in remission from ALL without progression of liver damage for 2 years. Severe treatment‐related toxicity should be considered in chemotherapy for patients with WD.
Carbamazepine–imatinib interaction in a child with chronic myeloid leukemia
Authors: Taguchi Kazuaki , Kouroki Masahiko , Ohmura Takafumi , Jono Hirofumi , Endo Fumio , Saito Hideyuki
Published: September 2014

Imatinib mesylate, a selective tyrosine kinase inhibitor, is the frontline therapeutic agent used for the treatment of chronic myeloid leukemia (CML), and its therapeutic efficacy is associated with trough concentrations. Therefore, monitoring imatinib trough concentrations is strongly recommended for successful treatment of CML patients. It has been recently shown that some drugs altered imatinib plasma levels in adult patients. However, drug interactions with imatinib in children are still unknown. Here, we report a case of a 12‐year‐old child with epilepsy who was also diagnosed with CML and given imatinib in addition to an enzyme‐inducing antiepileptic drug, carbamazepine. Compared to population kinetics data, the data obtained for the patient showed a significant decrease of imatinib plasma concentrations. Our findings suggest that monitoring imatinib plasma concentrations in children receiving enzyme‐inducing antiepileptic drugs is needed to optimize the therapeutic efficacy of imatinib.
Case of Desbuquois dysplasia type 1: Potentially lethal skeletal dysplasia
Authors: Inoue Shinkai , Ishii Atsushi , Shirotani Goro , Tsutsumi Makoto , Ohta Eiji , Nakamura Masatoshi , Mori Toshiko , Inoue Takahito , Nishimura Gen , Ogawa Atsushi , Hirose Shinichi
Published: September 2014

We report a boy with Desbuquois dysplasia type 1. He had the typical skeletal changes: a “Swedish key” appearance of the proximal femora; advanced carpal ossification and other distinctive features of the hand, including an extra‐ossification center at the base of the proximal phalanx of the index and middle fingers; dislocation of the metacarpophalangeal joint of the index finger; and bifid distal phalanx of the thumb. In addition, he presented with very severe prenatal growth failure, respiratory distress as a neonate, subsequent failure to thrive and susceptibility to airway infection, and sudden death in early childhood. Molecular analysis identified homozygous 1 bp deletion in the Calcium‐Activated Nucleotidase 1 gene (CANT1). To our knowledge, this is the first report of Desbuquois dysplasia type 1 in Japan. Our experience suggests potential lethality in the disorder.
Facial palsy as an unusual presenting symptom associated with acute myeloid leukemia
Authors: Kubota Kazuo , Ozeki Michio , Hori Tomohiro , Kanda Kaori , Funato Michinori , Asano Takahiko , Fukao Toshiyuki , Kondo Naomi
Published: September 2014

Extramedullary infiltration is common in acute myeloid leukemia (AML) patients. Although AML can cause neurological symptoms, especially when associated with extramedullary infiltration, a presenting manifestation of facial palsy is rare. We report on a 1‐year‐old boy who developed right facial palsy. Detailed examination led to a diagnosis of AML (French–American–British classification M1). Magnetic resonance imaging enhanced with gadolinium‐diethylenetriamine penta‐acetic acid showed abnormal enhancement of the right facial nerve, which disappeared after chemotherapy. AML should be considered as a differential diagnosis of facial palsy. Enhanced magnetic resonance imaging may be useful for diagnosing facial palsy associated with AML and for evaluating treatment outcome.
Current human T‐cell lymphotropic virus type 1 mother‐to‐child transmission prevention status in Kagoshima
Authors: Nerome Yasuhito , Kojyo Kanami , Ninomiya Yumiko , Ishikawa Tamayo , Ogiso Ayano , Takei Syuji , Kawano Yoshifumi , Douchi Tsutomu , Takezaki Toshiro , Owaki Tetsuhiro
Published: September 2014

The aim of this study was to assess the current human T‐cell lymphotropic virus type 1 (HTLV‐I) mother‐to‐child transmission (MTCT) prevention system in Kagoshima Prefecture. We investigated the rate of carrier pregnant women from obstetrics facilities in Kagoshima by mail in 2012 and compared our results with previous study results. We interviewed carrier pregnant women about their choices for infant nutrition, and we interviewed midwives about the follow‐up system. In 2012, 8719 screening tests were performed, covering 58.1% of all pregnant women in Kagoshima; the rate of carrier pregnant women was 1.3%. Of 59 carriers, 39 chose short‐term breast‐feeding. The HTLV‐I carrier rate among pregnant women in Kagoshima has declined. The current HTLV‐I MTCT prevention system in Kagoshima is effective, but not sufficient. To bring the nutrition methods to completion, various types of support are needed. Further studies will elucidate many unsolved problems concerning MTCT.
The “Europeanization” of the Basel process: Financial harmonization between globalization and parliamentarization
Authors: Greenwood Justin , Roederer‐Rynning Christilla
Published: September 2014

Public policy initiatives aimed at the prevention of future financial crises originate with global harmonization in the form of executive standards issued by theBaselCommittee onBankingSupervision. This article explores the role of theEuropeanParliament (EP) in the process of adapting the standards in European Union legislation passed in 2013 as theCapitalRequirementsRegulation/CapitalRequirementsDirectiveIV. Unlike accounts casting theEPas increasingly dependent upon outside sources in order to meet its enhanced legislative role, we find it increasingly dexterous in developing and using in‐house policy ideas, expertise, and not least a common sense of institutional purpose. NotableEPsuccesses in final legislation include (but are not restricted to) a headline cap on bankers' bonuses in the face of entrenched business and national interests. The argument is developed by drawing upon a broad range of interviews together with other primary and secondary sources, tracing the contribution of theEPfrom the early stages of agenda‐setting through to the development of an“esprit de corps”among the committee lead team which survived intact throughout the “black box” of trilogue negotiations. Besides illuminating the notoriously opaque trilogue process, the analysis also contributes to contemporary debates about whether theEP's increased legislative powers are resulting in a shift away from its traditional allegiances with diffuse interests toward a greater engagement with producer sources in order to fulfill requirements for policy expertise.
How Accurate Are We in Sending Patients to Angiography?
Authors: Neves Marta , Ponce Pedro , Maia Pedro , Neves Fernando ,   the Lisbon and Coimbra Vascular Access Center Interventional Nephrology Teams
Published: September 2014

Physical examination (PE), and surveillance and monitoring techniques should be a core part of standard care provided to hemodialysis patients. This study aimed to define which referral criteria within a vascular access (VA) program were associated with indication for therapeutic endovascular intervention (EI). Retrospective study at two vascular centers, considering 1265 procedures between 1 November 2012 and 30 November 2013. All 617 arteriovenous fistulas (AVFs) and 648 arteriovenous grafts (AVGs) referred for angiography were enrolled. Each population was divided into two groups regarding absence (group 1) or presence (group 2) of a significant lesion on the angiogram, contemplating formal indication for EI. On multivariate analysis, factors independently associated with presence of a significant lesion in AVFs were referral due to increased dynamic venous pressure (p = 0.01), suspected stenosis by abnormal PE (p = 0.019), confirmed reduction in access flow rate (p = 0.003), and mean Kt/V < 1.4 (p = 0.004). In AVGs, although multivariate analysis did not find independent association of studied factors with presence of significant stenoses, 97.2% of the exams revealed relevant lesions previously suspected clinically. Aside from surveillance strategies, monitoring strategies including routine PE of the VA are imperative as an integral part of evaluation, especially in AVFs.
Identification of risk factors for suffering fear of hypoglycemia in type 1 Diabetes Mellitus patients
Authors: Anarte María Teresa , Carreira Mónica , Machado Alberto , Domínguez Marta , Tapia María José , Valdés Sergio , Ruiz de Adana María Soledad , Soriguer Federico
Published: September 2014

Hypoglycemia is one of the main burdens for type I Diabetes Mellitus (DM I) patients. The consequences of hypoglycemia can be quite unpleasant due to the variety of disagreeable physical and psychological symptoms it triggers. The patient's previous experience with hypoglycemia episodes will condition his psychological reaction to future episodes, promoting behavioral modifications that associate with poor glycemic control and worse prognosis, and even with developing psychological disorders, leading to fear of hypoglycemia (FH). To be able to provide tailored prevention and treatment of patients with FH it is necessary to identify the risk factors in DM I patients. We developed and validated the FH‐15 scale, a novel instrument to assess FH, which showed good concurrent and predictive validity in DM I patients. In this work we aim to identify the risk factors for suffering FH by detecting DM I patients with FH using the FH‐15 scale and then analyzing the association of clinical and sociodemographic variables. We found that age, needing help to resolve an episode of hypoglycemia, and a perceived lack of social support are risk factors for suffering FH.
When Prejudice Is Popular: Implications for Discriminatory Behavior
Authors: Poteat V. Paul
Published: September 2014

Prejudice and popularity represent two major areas of research. Yet studies have not considered whether prejudiced adolescents actually can be popular. Among 572 high school students (Mage = 15.80 years), the current study tested the association between popularity (based on sociometric peer nominations) and sexual prejudice against gay and lesbian individuals, moderated by gender and perspective taking. As hypothesized, the association was significant for males but not females, and it was significant for adolescents lower on perspective taking but not those higher on perspective taking. Moreover, adolescents who were popular and expressed strong sexual prejudice were more likely to engage in homophobic behavior than prejudiced adolescents who were less popular. Popular adolescents with strong sexual prejudice beliefs may be more prone to use homophobic behavior as a way to maintain their dominant position. Similarly, prejudiced adolescents who are popular may face less pushback for their engagement in homophobic behavior. Continued attention to the connection between sexual prejudice and popularity is important because of the high status, influence, and visibility of popular adolescents.
Immunohematologic tolerance of chronic transfusion exchanges with erythrocytapheresis in sickle cell disease
Authors: Michot Jean‐Marie , Driss Françoise , Guitton Corinne , Moh Klaren Julia , Lefebvre François , Chamillard Xavier , Gallon Philippe , Fourn Erwan , Pela Alain M. , Tertian Gérard , Le Bras Philippe , Chantalat‐Auger Christelle , Delfraissy Jean‐François , Goujard Cécile , Lambotte Olivier
Published: September 2014

Background

Sickle cell disease (SCD) has become a major public health issue. Hydroxyurea and red blood cell (RBC) transfusion are the cornerstone treatments. Erythrocytapheresis (ECP) is an automated method for transfusion exchange. Given that ECP requires more blood than conventional transfusion, there is concern about alloimmunization and hemolytic transfusion reactions. We evaluate the incidence of hemolytic transfusion reactions and alloimmunization rates in patients receiving conventional blood transfusions and in patients participating in long‐term blood exchange programs with ECP.

Study Design and Methods

All hemolytic transfusion reactions and alloimmunizations in SCD patients were recorded over the period 2006 to 2011. Conventional transfusions and ECP were compared.

Results

The cohort consisted of 188 SCD patients. The median (±SD) age was 23 (±14) years. The ECP and conventional transfusion groups comprised 49 and 139 patients, respectively. The prevalence of alloimmunization was 33% in the ECP group and 22% in the conventional transfusion group (p = 0.1797). The alloimmunization/RBC unit (RBCU) ratio was lower in the ECP group than in the conventional transfusion group (1.6 and 11.6 per 1000, respectively; p < 0.0001). Although patients in the ECP group received more than 10 times more RBCUs than patients in the conventional transfusion group (206 vs. 19 RBCUs per patient, respectively; p < 0.0001), none of the four recorded hemolytic transfusion reactions (n = 4) occurred.

Conclusion

Regarding alloimmunization, ECP exhibits a good immunohematologic safety profile relative to conventional transfusion in a large SCD mainly adult cohort.
Blood transfusion transmission of the tick‐borne relapsing fever spirochete Borrelia miyamotoi in mice
Authors: Krause Peter J. , Hendrickson Jeanne E. , Steeves Tanner K. , Fish Durland
Published: September 2014

Background

Borrelia miyamotoi, a recently discovered relapsing fever spirochete, occurs in hard‐bodied ticks wherever Lyme disease is endemic. Human infection is associated with relapsing fever and can cause meningoencephalitis in immunocompromised patients. A few cases of transfusion transmission of other relapsing fever spirochete species have been reported but none for B. miyamotoi. Our objective was to determine whether B. miyamotoi transfusion transmission could occur in a murine transfusion model. Herein, we report transfusion transmission of B. miyamotoi through fresh or stored red blood cells (RBCs) in a mouse model.

Study Design and Methods

Inbred mice were transfused with B. miyamotoi–infected murine blood that was either freshly collected or stored for 7 days before transfusion. Recipient blood was then longitudinally examined after transfusion by smear and wet mount for evidence of spirochetemia.

Results

Motile spirochetes were observed in immunocompromised (SCID) mouse recipients for 28 days after transfusion of both fresh and stored murine B. miyamotoi–infected RBCs. Transient spirochetemia was observed in immunocompetent DBA/2 and C57BL/6 mice, with spirochete clearance occurring within 5 days after transfusion.

Conclusion

These data demonstrate that transfusion transmission of B. miyamotoi can occur in mice and suggest that it also may occur in humans.
Differentiating between analytical and diagnostic performance evaluation with a focus on the method comparison study and identification of bias
Authors: Flatland Bente , Friedrichs Kristen R. , Klenner Stefanie
Published: September 2014

Prior to introduction of a new method to the diagnostic laboratory, analytical performance must be validated to ensure operation within the manufacturer's specifications and/or within predetermined quality requirements. In addition, the new method may require diagnostic performance assessment to ensure it differentiates between diseased and nondiseased individuals as intended. These 2 phases of assessment, while complementary, are not equivalent and require a different set of experiments, statistical analyses, and interpretation. Studies of analytical performance typically include a method comparison experiment, the purpose of which is to identify bias (inaccuracy) of the “test” (or “index”) method (new method) relative to a “comparative method” (established method). Analysis of method comparison data is facilitated by commercial software programs that present the statistical significance of identified bias; however, the clinical relevance of any bias also should be considered. Studies of diagnostic performance should not be pursued until analytical performance is fully characterized and may not be required for well‐established tests or for those for which results are nonspecific (ie, not referable to a specific disease or condition). Diagnostic performance assessment may include assessment of sensitivity, specificity, predictive values, odds ratios, and/or likelihood ratios. The purpose of this review is to clarify differences between the assessment of analytical and diagnostic performance, and to explore the method comparison study and bias assessment from a perspective not addressed in prior veterinary articles.
Using the Nonlinear Kinematic Hardening Material Model of Chaboche for Elastic–Plastic Ratcheting Analysis
Authors: Kalnins Arturs , Rudolph Jürgen , Willuweit Adrian
Published: September 2014

Commonly used design codes for power plant components and pressure vessels include rules for ratcheting analysis that specify limits on accumulated strain. No guidance is provided on the use of the material model. The objective of the paper is to provide guidance that may be helpful to analysts. The Chaboche nonlinear kinematic (NLK) hardening material model is chosen as an appropriate model. Two methods are selected for its calibration that can determine the parameters for stainless steels. One is manual that requires no outside software and the other uses finite element software. Both are based on the monotonic stress–strain curve obtained from a tension specimen. The use of the Chaboche parameters for cases when ratcheting is caused by cyclic temperature fields is selected as the example of an application. The conclusion is that the number of allowable design cycles is far higher when using the parameters with temperature dependency than those at the constant maximum temperature that is being cycled.
An All-Metal Compliant Seal Versus a Labyrinth Seal: A Comparison of Gas Leakage at High Temperatures
Authors: San Andrés Luis , Anderson Alain
Published: September 2014

Parasitic secondary flows (seals' leakage) in centrifugal compressors and gas and steam turbines represent a substantial loss in efficiency and power delivery with an increase in specific fuel consumption. Labyrinth seals (LS) are the most common and inexpensive means of reducing secondary leakage, albeit wearing out with operation and thereby penalizing performance and even affecting rotordynamic stability. The novel hydrostatic advanced low leakage (HALO) seal is an all-metal seal with flexibly supported shoes that enable clearance self-control to effectively reduce leakage, in particular for operation with high pressure ratios and at high surface rotor speeds. This paper presents leakage tests with hot air (max. 300 °C) conducted in a test rig holding a LS and a HALO seal, both of similar diameter, axial length, and clearance. The novel seal leaks much less than the LS as the supply/discharge pressure ratio (Ps/Pa) increases. The leakage reduction is ∼50% for (Ps/Pa) < 2 and continuously dropping to 70% for (Ps/Pa) > 3.0. Thus, the savings in leakage are maximized for operation with a high pressure differential. Leakage measurements with a rotor spinning to a maximum speed of 2700 rpm (surface speed ∼24 m/s) produce a slight decrease in leakage for both seals. Characterization of seal leakage in terms of a flow factor removes the effect of temperature and supply pressure; the LS showing a constant flow factor for (Ps/Pa) > 2. Application of the novel seal technology will aid to increase system efficiency by reducing leakage and will extend maintenance intervals since it eliminates wear of components.