bluuebunny/crossref_metadata_2025_split huggingface dataset:
Antioxidants restore protoporphyrinogen oxidase in variegate porphyria patients
Authors: Ferrer Miguel D. , Tauler Pedro , Sureda Antoni , Palacín Clara , Tur Josep A. , Pons Antoni 
Published: April 2013

AbstractBackgroundVariegate porphyria (VP) is the result of decreased protoporphyrinogen oxidase (PPOX) activity and results in the accumulation of porphyrins and porphyrin precursors. Our aims were to analyse the basal antioxidant defences and oxidative damage markers and the effects of a diet supplementation with vitamins E and C on the oxidant/antioxidant status and PPOX gene expression in lymphocytes of variegate porphyria (VP) patients.Materials and methodsTwelve women affected by VP and 12 control women participated in a randomized and double‐blind crossover study. Each participant took either 50 mg/day vitamin E and 150 mg/day vitamin C or a placebo for 6 months.ResultsLymphocyte PPOX gene expression, together with catalase and glutathione peroxidase activities, was reduced in VP women. No differences were observed in the levels of malondialdehyde and protein carbonyl derivatives. Stimulated lymphocyte H2O2 production was higher in porphyric women. Supplementation with antioxidant vitamins increased PPOX expression in VP patients. Glutathione reductase (GRd) and superoxide dismutase (SOD) activities were higher in the treatment groups.ConclusionsLymphocytes from VP patients show reduced PPOX expression and present a greater susceptibility to producing H2O2 and impaired H2O2 detoxifying mechanisms. Supplementation with vitamins E and C restores PPOX expression in VP patients and enhances GRd and SOD activity, suggesting the potential benefits of a diet rich in vitamins E and C in these patients.
Preserved thrombin‐inducible platelet activation in thienopyridine‐treated patients
Authors: Gremmel Thomas , Kopp Christoph W. , Seidinger Daniela , Koppensteiner Renate , Steiner Sabine , Panzer Simon 
Published: April 2013

AbstractBackgroundAbundant thrombin generation may be a major reason for subsequent thromboembolic events in patients with cardiovascular disease receiving dual antiplatelet therapy. We therefore investigated the susceptibility of thienopyridine responders and nonresponders to thrombin receptor–activating peptide (TRAP)‐6‐ and adenosine diphosphate (ADP)‐inducible platelet activation.Materials and methodsResponse to clopidogrel or prasugrel was determined by the vasodilator‐stimulated phosphoprotein (VASP) phosphorylation assay and multiple electrode aggregometry (MEA) in 317 patients undergoing angioplasty and stenting for cardiovascular disease. Baseline, TRAP‐6‐, and ADP‐inducible P‐selectin expression, activated glycoprotein IIb/IIIa (GPIIb/IIIa) and monocyte–platelet aggregate (MPA) formation were measured as sensitive parameters of platelet activation.ResultsIn patients with high on‐treatment residual ADP‐inducible platelet reactivity (HRPR), baseline P‐selectin expression, GPIIb/IIIa and MPA formation were similar to those in patients without HRPR (all P > 0·05). After platelet activation with TRAP‐6 or ADP, patients with HRPR by both assays exhibited significantly higher levels of P‐selectin expression, GPIIb/IIIa and MPA formation than patients with an adequate thienopyridine‐mediated platelet inhibition (all P ≤ 0·02). However, high levels of TRAP‐6‐inducible P‐selectin, GPIIb/IIIa and MPA formation also occurred in 20·4%, 19·1% and 20·1% of the good responders by the VASP assay, and in 19·6%, 16·6% and 20·6% of the good responders by MEA, respectively.ConclusionsThienopyridine nonresponders are more susceptible to thrombin‐ and ADP‐inducible platelet activation than patients with good platelet inhibition. However, even patients with adequate thienopyridine‐mediated platelet inhibition often show a preserved responsiveness to thrombin. These patients may benefit from additional thrombin receptor blockage or inhibition of thrombin generation.
The LPS‐induced increase in circulating microparticles is not affected by vitamin C in humans
Authors: Weisshaar Stefan , Gouya Ghazaleh , Nguyen Daniel , Kapiotis Stylianos , Wolzt Michael 
Published: April 2013

AbstractObjectiveMicroparticles (MP) are considered to promote coagulation. This study aimed to characterize the time course of MP levels and the effect of high‐dose vitamin C on MP formation during inflammation in an in vivo Escherichia coli endotoxin (LPS) model.MethodsMicroparticle formation was studied in 14 male subjects in a cross‐over trial who received either intravenous vitamin C at 320 mg/kg body weight (BW) or 480 mg/kg BW or saline solution in a random order on alternate trial days 3 h after intravenous exposure to LPS (2 ng/kg BW). Venous blood samples were taken before, 3 and 6 h after LPS. D‐dimer, leucocyte count, C‐reactive protein, plasma vitamin C and body temperature were assessed as inflammatory parameters. MP were detected using flow cytometric analysis and expressed in 10³ MP/mL plasma.ResultsMicroparticles levels were decreased from baseline 848 units [range 431–1705] by 21% to 671 units [253–1586] at 3 h and increased by 32% to 1119 units [288–4443] at 6 h after LPS. This pattern was not influenced by administration of vitamin C, with a change from 730 units [399–1396] at baseline by an increase to 832 units [215–2168] at 3 h to 1055 units [350–4858] at 6 h. MP subpopulations followed similar dynamics. Alterations in inflammatory parameters were independent from vitamin C administration during endotoxemia.ConclusionMicroparticles are increased in acute systemic inflammation with inconsistent changes in MP subgroups in healthy subjects. Systemic vitamin C administration does not mitigate MP formation and D‐dimer levels during acute systemic inflammation, suggesting that MP‐induced coagulation activity is not affected by vitamin C.
Prognostic impact of pretransplant iron overload measured with magnetic resonance imaging on severe infections in allogeneic stem cell transplantation
Authors: Virtanen Johanna M. , Itälä‐Remes Maija A. , Remes Kari J. , Vahlberg Tero , Saunavaara Jani P. , Sinisalo Marjatta , Parkkola Riitta K. 
Published: April 2013

AbstractObjectiveInfections and graft‐versus‐host disease (GVHD) are the main causes of transplant‐related mortality (TRM) of patients undergoing allo‐SCT. The role of iron overload (IO) has been debated in this context. Studies, performed with non‐specific surrogate markers of iron, suggest that IO predicts poor outcome after allo‐SCT.MethodsIn this prospective study, we quantified pretransplant IO with MRI‐based hepatic iron concentration (HIC) measurement; the degree of IO was used to predict infections, GVHD, and mortality after allo‐SCT. Logistic univariate, multivariate, and Cox's regression analyses were performed.ResultsIron overload was present in 78% of the patients (HIC>36 μmol/g). The median HIC was 98 μmol/g (range 5–348). There were no cases of cardiac iron excess. IO was significantly associated with severe infections during the early post‐transplant period (for every 10 μmol/g increase OR: 1.15, 95% CI 1.05–1.26, P = 0.003). The odds for severe infections increased 6.5‐ (>125 μmol/g OR: 6.5, P = 0.013) to 14‐fold (>269 μmol/g OR: 14.1, P = 0.040) with increasing HIC. IO was found to be associated with reduced risk of acute and chronic GVHD. Although TRM was due to infection‐related deaths, IO was not associated with TRM or OS.ConclusionPretransplant IO, measured with a direct MRI‐based measurement, predicts severe infections in the early post‐transplant period.
Decreased expression of GLT‐1 in the R6/2 model of Huntington's disease does not worsen disease progression
Authors: Petr Geraldine T. , Schultheis Laurel A. , Hussey Kayla C. , Sun Yan , Dubinsky Janet M. , Aoki Chiye , Rosenberg Paul A. 
Published: April 2013

AbstractExcitotoxicity is thought to be important in the pathogenesis of Huntington's disease (HD). Glutamate is the predominant excitatory neurotransmitter in the brain, and excess activation of glutamate receptors can cause neuronal dysfunction and death. Glutamate transporters regulate the extracellular concentration of glutamate. GLT‐1 is the most abundant glutamate transporter, and accounts for most of the glutamate transport in the brain. Administration of ceftriaxone, an antibiotic that increases the functional expression of GLT‐1, can improve the behavioral phenotype of the R6/2 mouse model of HD. To test the hypothesis that GLT‐1 expression critically affects the HD disease process, we generated a novel mouse model that is heterozygous for the null allele of GLT‐1 and carries the R6/2 transgene (double mutation). We demonstrated that the protein expression of total GLT‐1, as well as two of its isoforms, is decreased within the cortex and striatum of 12‐week‐old R6/2 mice, and that the expression of EAAC1 was decreased in the striatum. Protein expression of GLT‐1 was further decreased in the cortex and striatum of the double mutation mice compared with the R6/2 mice at 11 weeks. However, the effects of the R6/2 transgene on weight loss, accelerating rotarod, climbing and paw‐clasping were not exacerbated in these double mutants. Na+‐dependent glutamate uptake into synapatosomes isolated from the striatum and cortex of 11‐week‐old R6/2 mice was unchanged compared with controls. These results suggest that changes in GLT‐1 expression or function per se are unlikely to potentiate or ameliorate the progression of HD.
The Evolving and Multilayered EU–India Investment Relations—Regulatory Issues and Policy Conjectures
Authors: Chaisse Julien , Chakraborty Debashis 
Published: April 2013

AbstractIndia and several EU member countries share a rich history of investment collaborations. The collaboration has been cemented with several formal agreements with individual EU members, and the recent negotiations with the trade bloc since June 2007 on a broad‐based Bilateral Trade and Investment Agreement (BTIA) can be considered as a culmination of this process while ongoing WTO negotiations on Mode 3 commitments remain essential in terms of market opening. The present article analyzes the multi‐layered regulation of foreign investment against the backdrop of the evolving EU‐India economic relations. The 2009 Treaty of Lisbon gave a new competence to the EU which will impact ongoing negotiations with India whose global standing has been significantly changing in recent years. The economic vibrancy, coupled with large market size, has earned India greater relevance in several international forums, thereby making the future EU—India investment treaty one of the most promising investment agreements.
Longitudinal study of regional cerebral blood flow in elderly patients with idiopathic rapid eye movement sleep behavior disorder
Authors: Sakurai Hirofumi , Hanyu Haruo , Inoue Yuichi , Kanetaka Hidekazu , Nakamura Masaki , Miyamoto Tomoyuki , Sasai Taeko , Iwamoto Toshihiko 
Published: April 2013

AimSingle photon emission computed tomography (SPECT) studies showed that regional cerebral blood flow (rCBF) abnormalities in idiopathic rapid eye movement (REM) sleep behavior disorder (iRBD) are similar to those seen in Parkinson's disease (PD) and dementia with Lewy bodies (DLB). The aim of the present study was to assess the longitudinal rCBF changes in patients with iRBD using repeated SPECT.MethodsNine patients with iRBD (7 men and 2 women; mean age 71.1 ± 3.2 years) underwent baseline and follow‐up SPECT studies (a mean interval of 22.8 ± 9.2 months).ResultsA decrease in rCBF was found in bilateral parietotemporal and occipital areas at the first and second SPECT. Compared with the first SPECT, the second SPECT showed a decreased rCBF in the medial portions of the parietooccipital lobe with a significant decrease in rCBF of the right posterior cingulate. None of the patients showed any neurological deficits, including extrapyramidal and cerebellar signs, visual hallucinations, and neuropsychological impairments during the study.ConclusionThese findings suggest that longitudinal measurements of rCBF can show the presence of progressing neurodegenerative process in iRBD. Longitudinal SPECT study can be used to monitor the progression of degenerative process in patients with iRBD, even though there were no evolving neurological and neuropsychiatric impairments. Geriatr Gerontol Int 2014; 14: 115–120.
Nodal reactive and neoplastic proliferation of monocytoid and marginal zone B cells: an immunoarchitectural and molecular study highlighting the relevance of IRTA1 and T‐bet as positive markers
Authors: Bob Roshanak , Falini Brunangelo , Marafioti Teresa , Paterson Jennifer C , Pileri Stefano , Stein Harald 
Published: April 2013

AimsMarginal zone B cells (MZCs) and monocytoid B cells (MBCs) appear to be related lymphoid cells that take part in reactive and neoplastic marginal zone proliferations. These lesions are not yet well characterized, and the aim of this study was to find better diagnostic criteria for them.Methods and resultsWe analysed 60 nodal lesions with MBC and/or MZC proliferation for their morphological, immunophenotypic, molecular genetic and IG gene rearrangement features. On the basis of the results of the rearrangement assay and immunoglobulin light chain restriction, the lesions were divided into reactive and neoplastic groups. Among the neoplastic lesions, polymorphic and monomorphic subgroups emerged. All reactive lesions had morphological features of the polymorphic subgroup. By immunohistochemistry, IRTA1 and/or T‐bet expression was found in all reactive lesions and in 90% of neoplastic lesions.ConclusionsIRTA1 and T‐bet are positive markers for the identification of MZC/MBC proliferations, and thus for the diagnosis of nodal marginal zone lymphoma (NMZL). Polymorphic and monomorphic subgroups of NMZL could be distinguished. Most morphological and immunophenotypic patterns in reactive and neoplastic nodal expansions of MZCs and MBCs overlapped. Therefore, PCR clonality assay of the immunoglobulin heavy and light chain gene loci is the most reliable method for their differentiation.
Study of the Glass Transition Temperature of As‐S Glasses for the Fabrication of Chalcogenide Optical Fibers
Authors: Skripachev Igor V. , El‐Amraoui Mohamed , Messaddeq Younes , Santagneli Silvia H. 
Published: April 2013

Values of glass transition temperature (Tg) and of linear expansion coefficient (α) for Asx S100−x glasses were measured in the range of concentrations 35 × 42. Because of the importance of the glass formation region 35 × 42 for the optical fibers elaboration, special attention was made on high‐pure Asx S100−x glasses. For the glass in the range of 35 × 38, we measure Tg with the interval of x equal to 1 at.% of arsenic. We also measured the Tg values with the interval of x equal to 0.5 at.% of As. We obtained nonlinear behavior of Tg, reflecting the change in molecular composition of As‐S glass in the glass composition range studied. The control of such parameters is important to produce optical fibers with specific numerical aperture.
Transcription factors and CD4 T cells seeking identity: masters, minions, setters and spikers
Authors: Vahedi Golnaz , Kanno Yuka , Sartorelli Vittorio , O'Shea John J. 
Published: April 2013

SummaryNaive T cells differentiate and become distinct subsets in response to changes in the cytokine milieu. Such specialization arises through a complex and dynamic utilization of cis‐regulatory enhancer elements. In this brief essay, we review recent findings on the relative contributions of sensors of the cytokine milieu, especially the signal transducer and activator of transcription family transcription factors, ‘master regulators’, and other transcription factors in the enhancer architecture of T cells. These findings provide new insights into how signal transduction impinges upon the genome.
Endoscopic papillary balloon dilation for a 7‐year‐old girl with choledocholithiasis
Authors: Sogo Tsuyoshi , Kondo Takeo , Tsunoda Tomoyuki , Murayama Akitoshi , Komatsu Haruki , Inui Ayano , Fujisawa Tomoo 
Published: April 2013

AbstractA 7‐year‐old girl was referred to our hospital for severe abdominal pain and elevated serum levels of amylase and aminotransferase. Radiological examinations revealed choledocholithiasis. EPBD was performed under intravenous anesthesia using midazolam and ketamine chloride. After fluoroscopic confirmation of a bile duct stone, a dilation balloon (30 mm‐long, 8 mm‐diameter) was passed over the guidewire and placed across the papilla. The balloon was gradually inflated until the notch created by the papillary sphincter disappeared. At 4 atm, the notch disappeared and the pressure was maintained for 15 s. Stone extraction was performed by using a retrieval balloon catheter. Abdominal pain disappeared immediately after EPBD, and she resumed oral intake 2 days after EPBD. In conclusion, EPBD may be a safe and effective technique for the treatment of choledocholithiasis in pediatric patients. EPDB should be selected as a treatment for pediatric choledocholithiasis in view of its advantages of preserving papillary function.
Congenital chylothorax and pulmonary hypertension complicated with heart failure and hepatopathy
Authors: Oh Jin Hee , Lee Guisera , Lee Jung Hyun 
Published: April 2013

AbstractRespiratory difficulty resulting from congenital chylothorax is usually relieved by postnatal thoracentesis, closed chest drainage, and oxygen therapy. However, early occurrence of congenital chylothorax or accumulation of a large amount of chylous fluid sometimes leads to pulmonary hypoplasia or persistent pulmonary hypertension of the newborn, which requires further customized mechanical ventilatory support. In these cases, conventional mechanical ventilation is primarily used during initial treatment and is later replaced by high‐frequency ventilation, but the advantages of inhaled nitric oxide treatment have rarely been described. This case suggests the benefits of inhaled nitric oxide in patients with congenital chylothorax, even when mechanical ventilation cannot improve respiratory distress because of severe pulmonary hypertension of the newborn leading to right cardiac dysfunction and possibly cholestasis.
Rare case of an abdominal mass presenting as acute abdomen: Torsion of the vermiform appendix
Authors: Kilincaslan Huseyin , Gedik Ahmet Hakan , Bilici Mustafa , Cakir Secil 
Published: April 2013

AbstractTorsion of the vermiform appendix is a rarely observed acute abdominal emergency. The clinical manifestations are similar to acute appendicitis. It should be included in the differential diagnosis if a patient presents with intermittent abdominal pain and an acute abdominal emergency. Here, we describe a 4‐year‐old male patient with a mass located in the right lower abdominal quadrant, whose clinical findings were consistent with an acute abdomen. The gangrenous appendix was found in the pelvic cavity and its stump was twisted clockwise. A 1.5‐cm fecalith was detected in the appendix lumen and appendectomy was performed.
Nano‐Hydroxyapatite Improves the Properties of β‐tricalcium Phosphate Bone Scaffolds
Authors: Shuai Cijun , Feng Pei , Nie Yi , Hu Huanglong , Liu Jinglin , Peng Shuping 
Published: April 2013

β‐Tricalcium phosphates have been widely used as biomaterials for bone substitutes; however, the poor mechanical properties limit the application in bearing loading bones. In this study, nano‐hydroxyapatite has been introduced to improve the mechanical properties for porous bioceramic scaffolds. Nanocomposites containing 0–10 wt% needle‐like nano‐hydroxyapatite were prepared for investigation. It has been found that needle‐like nano‐hydroxyapatite improves the toughness, hardness, and compressive strength of the porous β‐tricalcium phosphates scaffolds, as well as the microstructure properties. The study provides a reference for the development of safe, excellent bone scaffolds for bone tissue engineering.
The Muslim Settlement of Late Ottoman and Mandatory Palestine: Comparison with Jewish Settlement Patterns
Authors: Frantzman Seth J. , Kark Ruth 
Published: April 2013

AbstractIn the late Ottoman and Mandatory periods, Palestine's rural landscape underwent a great transformation. This study examines how the Muslim population expanded beyond its traditional inhabitation in the highlands and settled the fluid inventory of marginal lands in the coastal plains and unpopulated valleys of Palestine. In settling these marginal landscapes their settlement dovetailed with Jewish settlement patterns. While most studies have emphasized the competitive aspect of this process, examining Zionist and Arab national claims, this research points to a different aspect of this new settlement—mainly how much the Jewish and Muslim settlement patterns mirrored one another and how they were part of similar physical processes and complemented one another. Relying on censuses, aerial photographs, and period maps, as well as other archival sources, this is the first systematic research to examine the full extent of new Muslim settlements in Palestine in the late Ottoman and Mandatory periods, and to draw parallels between this phenomenon and the settlement endeavors of the Zionists.
Efficacy of the HealthMatters Program Train‐the‐Trainer Model
Authors: Marks Beth , Sisirak Jasmina , Chang Yen‐Ching 
Published: April 2013

BackgroundThis study examines the efficacy of a staff‐led, health promotion intervention entitled HealthMatters Program: Train‐the‐Trainer Model to improve health among adults with intellectual disabilities. While data support the benefits of health promotion for adults with intellectual disabilities in controlled settings, little research documents strategies to improve health and health behaviours in settings in which people work and live.MethodsThirty‐four staff in community‐based organizations (CBOs) participated in an 8‐h training to start a 12‐week health promotion programme for clients with intellectual disabilities. Participants with intellectual disabilities (n = 67; aged 30 and older) were randomized into an intervention (12‐week exercise and health education programme) or control group.ResultsIntervention group showed significant improvements in health status, knowledge, self‐efficacy, and fitness.ConclusionResults support the efficacy of a HealthMatters Program in CBOs to improve health among adults with intellectual disabilities. A need exists for CBOs to include health promotion in their mission and vision statement and job descriptions.