bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long‐term outcome in pyridoxine‐dependent epilepsy
Authors: BOK LEVINUS A , HALBERTSMA FEICO J , HOUTERMAN SASKIA , WEVERS RON A , VREESWIJK CHARLOTTE , JAKOBS CORNELIS , STRUYS EDUARD , VAN DER HOEVEN JOHAN H , SIVAL DEBORAH A , WILLEMSEN MICHÈL A 
Published: July 2012

Aim  The long‐term outcome of the Dutch pyridoxine‐dependent epilepsy cohort and correlations between patient characteristics and follow‐up data were retrospectively studied.Method  Fourteen patients recruited from a national reference laboratory were included (four males, 10 females, from 11 families; median age at assessment 6y; range 2y 6mo–16y). The following data were retrieved: sex; age at seizure onset; age at the start of pyridoxine therapy; level of urinary alpha‐aminoadipic semialdehyde; antiquitin mutations; developmental milestones; evaluation of neurocognitive functioning and school career; magnetic resonance imaging (MRI) and electroencephalography (EEG) assessments.Results  Pyridoxine was started antenatally in two children, in the first week of life in five, in the first month of life in three, or after the first month of life (range 2.5–8mo) in four. No child was physically disabled; however, only five walked at 2 years of age. Mental development was delayed in most: median IQ or developmental index was 72 (SD 19). Pyridoxine monotherapy controlled seizures in 10 of 14 children, whereas four needed additional antiepileptic drugs. Seizure persistence, antiepileptic drugs (other than pyridoxine), EEG background, and epileptiform activity were not associated with outcome. On neonatal MRI, structural and white matter abnormalities occurred in five of eight children; on follow‐up, the number of abnormal MRIs was increased. Delayed initiation of pyridoxine medication and corpus callosum abnormalities were significantly associated with unfavourable neurodevelopmental outcome, but normal follow‐up imaging did not predict a good outcome.Interpretation  Outcome of patients with pyridoxine‐dependent epilepsy remains poor. Individual outcome cannot be predicted by the evaluated characteristics. We suggest that collaborated research in structured settings could help to improve treatment strategies and outcome for pyridoxine‐dependent epilepsy.
U.S. International Equity Investment
Authors: AMMER JOHN , HOLLAND SARA B. , SMITH DAVID C. , WARNOCK FRANCIS E. 
Published: July 2012

ABSTRACTUsing a comprehensive data set of all U.S. investment in foreign equities, we find that the single most important determinant of the amount of U.S. investment a foreign firm receives is whether the firm cross‐lists on a U.S. exchange. Correcting for selection biases, cross‐listing leads to a doubling (or more) in U.S. investment, an impact greater than all other factors combined. Much of this increased U.S. investment is purchased in the foreign market, implying that the cross‐listing effect reflects something more fundamental about a firm than easier acquisition of its securities. We also demonstrate that cross‐listing is an important determinant of U.S. international investment at the country level and describe easy‐to‐implement methods for including a cross‐listing variable as an endogenous control.
Strategy Selection, Surrogation, and Strategic Performance Measurement Systems
Authors: CHOI JONGWOON (WILLIE) , HECHT GARY W. , TAYLER WILLIAM B. 
Published: July 2012

ABSTRACTStrategic performance measurement systems operationalize firm strategy with a set of performance measures. A consequence of such alignment is the tendency for managers to lose sight of the strategic construct(s) the measures are intended to represent, and subsequently act as though the measures are the constructs of interest, a phenomenon referred to as surrogation. We investigate how involvement in strategy selection affects managers’ propensity to exhibit surrogation. We predict and find that strategy selection reduces surrogation. Surprisingly, we do not find that engaging in strategy deliberation, a key process underlying strategy selection, reduces surrogation. Thus, managers’ involvement in the actual choice of strategy appears to be both a necessary and sufficient condition to mitigate surrogation. Our paper broadens understanding of factors that influence surrogation, such as the effects of different aspects of managers’ strategic involvement and buy‐in. Further, by documenting how managers behave within (as opposed to simply with) strategic performance measurement systems, we highlight the potential for managers to endogenously influence the effectiveness of such systems.
Effects of Zoospore Aggregation and Substrate Rugosity on Kelp Recruitment Success
Authors: Muth Arley F. 
Published: July 2012

Successful kelp recruitment is important for kelp population persistence and associated kelp forest communities. The proximity of settled kelp zoospores is a known requirement for successful kelp recruitment and proximity can be increased as zoospores aggregate. Substrate rugosity can also be an important factor affecting macroalgal settlement and recruitment in wave‐swept areas, and may affect kelp recruitment by aggregating zoospores. In this study, kelp zoospores were cultured at different levels of small‐scale aggregation and kelp recruitment was quantified. Sporophyte production significantly increased as zoospores became more aggregated indicating that processes that aggregate kelp zoospores have the potential to enhance kelp recruitment. A 13‐month field experiment demonstrated differential kelp recruitment onto settlement plates that mimicked surface rugosities of two common rock types within Stillwater Cove, Carmel Bay in central California (Carmelo Formation sandstone and Santa Lucia granodiorite). Significantly more kelp recruited to molds mimicking granodiorite over the yearlong study (granodiorite = 2.7 recruits ± SE 0.50, sandstone = 1.2 recruits ± SE 0.51). There was a significant difference in recruitment between seasons and this variability was due to the fact that spring had the highest average number of kelp recruits per mold. However, the interaction between substrate and season was not significant. This study emphasizes the importance of kelp zoospore aggregation on kelp recruitment and demonstrates that small‐scale rugosity affects kelp recruitment.
DESCRIPTION OF VIRIDILOBUS MARINUS (GEN. ET SP. NOV.), A NEW RAPHIDOPHYTE FROM DELAWARE'S INLAND BAYS
Authors: Demir‐Hilton, Elif , Hutchins David A. , Czymmek Kirk J. , Coyne Kathryn J. 
Published: July 2012

Delaware's Inland Bays (DIB), USA, are subject to blooms of potentially harmful raphidophytes, including Heterosigma akashiwo. In 2004, a dense bloom was observed in a low salinity tributary of the DIB. Light microscopy initially suggested that the species was H. akashiwo; however, the cells were smaller than anticipated. 18S rDNA sequences of isolated cultures differed substantially from all raphidophyte sequences in GenBank. Phylogenetic analysis placed it approximately equidistant from Chattonella and Heterosigma with only ~96% sequence homology with either group. Here, we describe this marine raphidophyte as a novel genus and species, Viridilobus marinus (gen. et sp. nov.). We also compared this species with H. akashiwo, because both species are superficially similar with respect to morphology and their ecological niches overlap. V. marinus cells are ovoid to spherical (11.4 × 9.4 μm), and the average number of chloroplasts (4 per cell) is lower than in H. akashiwo (15 per cell). Pigment analysis of V. marinus revealed the presence of fucoxanthin, violaxanthin, and zeaxanthin, which are characteristic of marine raphidophytes within the family Chattonellaceae of the Raphidophyceae. TEM and confocal microscopy, however, revealed diagnostic microscopic and ultrastructural characteristics that distinguish it from other raphidophytes. Chloroplasts were in close association with the nucleus and thylakoids were arranged either parallel or perpendicular to the cell surface. Putative mucocysts were identified, but trichocysts were not observed. These features, along with DNA sequence data, distinguish this species from all other raphidophyte genera within the family Chattonellaceae of the Raphidophyceae.
Physical Activity and Physical Self‐Concept in Adolescence: A Comparison of Girls at the Extremes of the Biological Maturation Continuum
Authors: Cumming Sean P. , Sherar Lauren B. , Gammon Catherine , Standage Martyn , Malina Robert M. 
Published: July 2012

This study examined differences in physical activity and physical self‐concept between the least and most biologically mature female adolescents within chronological age and academic year groups. A total of 252 British female adolescents (M age = 12.9 years; SD = .7) in Years 7–9 completed self‐report measures to assess physical activity and physical self‐concept. Percentage of predicted adult (mature) stature served as the index of biological maturation. Univariate analyses of covariance, controlling for decimal age, revealed that the least mature girls generally held higher perceptions of physical self‐concept but were no more, or less, active than the most mature girls. A subsequent meta‐analysis suggested, however, that across age and year groups the least mature girls were more active than the most mature girls.
Riboflavin and ultraviolet light reduce the infectivity of Babesia microti in whole blood
Authors: Tonnetti Laura , Thorp Aaron M. , Reddy Heather L. , Keil Shawn D. , Goodrich Raymond P. , Leiby David A. 
Published: July 2012

BACKGROUND: Babesia microti is the parasite most frequently transmitted by blood transfusion in the United States. Previous work demonstrated the efficacy of riboflavin (RB) and ultraviolet (UV) light to inactivate B. microti in apheresis plasma and platelet units. In this study we investigated the effectiveness of RB and UV light to reduce the levels of B. microti in whole blood (WB).STUDY DESIGN AND METHODS: WB units were spiked with B. microti‐infected hamster blood. Spearman‐Karber methods were used to calculate infectivity of each sample in terms of hamster infectious dose 50% (HID50) value. After RB addition, the units were illuminated with 80 J/mLRBC UV light. Two samples were collected: one before illumination and one after illumination. The samples were serially diluted and dilutions injected into a group of five naive hamsters. Four weeks postinoculation (PI), blood was collected from the animals and evaluated by microscopic observation.RESULTS: One pilot study showed a good dose response in the animals and demonstrated that sample infectivity could be calculated in terms of an HID50. Three additional replicates were performed in the same manner as the pilot study, but with fewer dilutions. Infectivity values were consistent between the experiments and were used to calculate log reduction. The posttreatment reduction of B. microti for all the experiments was more than 5 log.CONCLUSIONS: The data collected indicate that use of RB and UV is able to decrease the parasite load in WB units thus reducing the risk of transfusion‐transmitted B. microti from blood components containing B. microti‐infected RBCs.
Comparison of a gel microcolumn assay with the conventional tube test for red blood cell alloantibody titration
Authors: Finck Rachel , Lui‐Deguzman Carrie , Teng Shih‐Mao , Davis Rebecca , Yuan Shan 
Published: July 2012

BACKGROUND: Titration is a semiquantitative method used to estimate red blood cell (RBC) alloantibody reactivity. The conventional tube test (CTT) technique is the traditional method for performing titration studies. The gel microcolumn assay (GMA) is also a sensitive method to detect RBC alloantibodies. The aim of this study was to compare a GMA with the CTT technique in the performance of Rh and K alloantibody titration.STUDY DESIGN AND METHODS: Patient serum samples that contained an RBC alloantibody with a singular specificity were identified by routine blood bank workflow. Parallel titration studies were performed on these samples by both the CTT method and a GMA (ID‐Micro Typing System anti‐IgG gel card, Micro Typing Systems, Inc., an Ortho‐Clinical Diagnostics Company).RESULTS: Forty‐eight samples were included, including 11 anti‐D, five anti‐c, 13 anti‐E, one anti‐C, three anti‐e, and 15 anti‐K. Overall, the two methods generated identical results in 21 of 48 samples. For 42 samples (87.5%) the two methods generated results that were within one serial dilution, and for the remaining six samples, results were within two dilutions.CONCLUSION: GMA systems may perform comparably to the CTT in titrating alloantibodies to Rh and Kell antigens.
Red blood cell alloimmunization in sickle cell disease: prevalence in 2010
Authors: Miller Scott T. , Kim Hae‐Young , Weiner Debra L. , Wager Carrie G. , Gallagher Dianne , Styles Lori A. , Dampier Carlton D. , Roseff Susan D. ,   Investigators of the Sickle Cell Disease Clinical Research Network (SCDCRN)
Published: July 2012

BACKGROUND: Transfusion of red blood cells (RBCs) is frequently required for care of individuals with sickle cell disease (SCD). Alloimmunization rates are high and may be reduced by matching for RBC antigens that can cause alloimmunization.STUDY DESIGN AND METHODS: During the PROACTIVE Feasibility Study, patients with SCD age 2 years or older admitted for pain without acute chest syndrome were enrolled for possible randomization to preventive blood transfusion or standard care. Transfusion and antibody histories were obtained at each site, and antibody screening was done, to assess transfusion burden and alloimmunization prevalence. Participating sites were surveyed regarding antigen matching practice.RESULTS: A total of 237 patients (169 SS, 42 SC, 15 Sβ0‐thalassemia, 11 Sβ+‐thalassemia), 118 males and 119 females, were enrolled. Mean age was 19.3 years (range, 2.0‐68.0); there were 122 children and 115 adults. A total of 75.8% had received at least a single transfusion of RBCs before the study. Thirty‐four patients (14.4%) had a history of at least one alloantibody and 17 of these had more than one. When surveyed, 19 sites (83% of responders) reported antigen matching to at least include C, E, and K for transfusion of all patients with SCD.CONCLUSION: Though antigen typing before transfusion of people with SCD and providing antigen‐negative units is now widely employed by sickle cell centers, the alloimmunization rate remains quite high in contemporary sickle cell populations and may be due in large part to transfusions received at institutions not providing extended matching.
RHCE*ceTI encodes partial c and partial e and is often in cis to RHD*DIVa
Authors: Westhoff Connie M. , Vege Sunitha , Halter Hipsky Christine , Hue‐Roye Kim , Copeland Tamara , Velliquette Randall W. , Horn Trina , Lomas‐Francis Christine , Reid Marion E. 
Published: July 2012

BACKGROUND: In the Rh blood group system, variant RhD and RhCE express several partial antigens. We investigated RH in samples with partial DIVa that demonstrated weak and variable reactivity with anti‐C.STUDY DESIGN AND METHODS: Standard hemagglutination techniques, polymerase chain reaction–based assays, and RH sequencing were used.RESULTS: DNA analysis showed that six red blood cell (RBC) samples with weak and inconsistent reactivity with anti‐C lacked RHCE*C, but all had RHD*DIVa, which encodes partial D and Goa. We then tested RBCs from 19 Go(a+) cryopreserved samples (confirmed to have RHD*DIVa) with four anti‐C and observed weak variable reactions. RHCE genotyping found all but one of the samples with RHD*DIVa also had RHCE nt 48G>C and 1025C>T, named RHCE*ceTI. Lookback of samples referred for workup and found to have either allele revealed 47 of 55 had both RHD*DIVa and RHCE*ceTI, four had RHD*DIVa without RHCE*ceTI, and four had RHCE*ceTI without RHD*DIVa. Alloanti‐c was found in a patient with c+ RBCs and RHCE*ceTI in trans to RHCE*Ce, and alloanti‐e was found in a patient with e+ RBC and RHCE*ceTI in trans to RHCE*cE. RHD*DIVa in trans to RHD erroneously tested as RHD hemizygous.CONCLUSIONS: RHD*DIVa and RHCE*ceTI almost always, but not invariably, travel together. This haplotype is found in people of African ancestry and the RBCs can demonstrate aberrant reactivity with anti‐C. RHCE*ceTI encodes partial c and e antigens. We confirm that RHD zygosity assays are unreliable in samples with RHD*DIVa.
Synthesis and Electrical Properties of Sputtered (Na0.5Bi0.5)TiO3 Thin Films on Silicon Substrate
Authors: Quignon Sébastien , Soyer Caroline , Remiens Denis 
Published: July 2012

The growth and characterizations of polycrystalline (Na0.5Bi0.5)TiO3 (NBT) thin films have been performed. (Na0.5Bi0.5)TiO3 films have been deposited by rf‐magnetron sputtering on the unheated Pt/TiOx/SiO2/Si structure and postannealed to crystallize the film in the perovskite phase. The optimal sputtering conditions and postannealing treatment to obtain a high degree of NBT crystallinity are reported. The dielectric, ferroelectric, and piezoelectric properties of NBT films were investigated. At 10 kHz, the dielectric constant and losses are 520 and 0.032, respectively. The ferroelectric hysteresis loops are well defined with a remnant polarization of 12 μC/cm2 and a coercive field of 125 kV/cm, but piezoelectric measurements at the macroscopic level were also performed: a high piezo coefficient (d33effmax) of 80 pm/V was obtained, similar to PZT films. This result demonstrates that NBT thin film is really a promising candidate for lead‐free MEMS applications. The local piezoresponse was evaluated by piezoelectric force microscopy.
Bepridil decreases Aβ and calcium levels in the thalamus after middle cerebral artery occlusion in rats
Authors: Sarajärvi Timo , Lipsanen Anu , Mäkinen Petra , Peräniemi Sirpa , Soininen Hilkka , Haapasalo Annakaisa , Jolkkonen Jukka , Hiltunen Mikko 
Published: July 2012

AbstractAlzheimer's disease (AD) and cerebral ischaemia share similar features in terms of altered amyloid precursor protein (APP) processing and β‐amyloid (Aβ) accumulation. We have previously shown that Aβ and calcium deposition, and β‐secretase activity, are robustly increased in the ipsilateral thalamus after transient middle cerebral artery occlusion (MCAO) in rats. Here, we investigated whether the non‐selective calcium channel blocker bepridil, which also inhibits β‐secretase cleavage of APP, affects thalamic accumulation of Aβ and calcium and in turn influences functional recovery in rats subjected to MCAO. A 27‐day bepridil treatment (50 mg/kg, p.o.) initiated 2 days after MCAO significantly decreased the levels of soluble Aβ40, Aβ42 and calcium in the ipsilateral thalamus, as compared with vehicle‐treated MCAO rats. Expression of seladin‐1/DHCR24 protein, which is a potential protective factor against neuronal damage, was decreased at both mRNA and protein levels in the ipsilateral thalamus of MCAO rats. Conversely, bepridil treatment restored seladin‐1/DHCR24 expression in the ipsilateral thalamus. Bepridil treatment did not significantly affect heme oxygenase‐1‐ or NAD(P)H quinone oxidoreductase‐1‐mediated oxidative stress or inflammatory responses in the ipsilateral thalamus of MCAO rats. Finally, bepridil treatment mitigated MCAO‐induced alterations in APP processing in the ipsilateral thalamus and improved contralateral forelimb use in MCAO rats. These findings suggest that bepridil is a plausible therapeutic candidate in AD or stroke owing to its multifunctional role in key cellular events that are relevant for the pathogenesis of these diseases.
Regenerative medicine for the treatment of spinal cord injury: more than just promises?
Authors: Pêgo Ana Paula , Kubinova Sarka , Cizkova Dasa , Vanicky Ivo , Mar Fernando Milhazes , Sousa Mónica Mendes , Sykova Eva 
Published: July 2012

AbstractSpinal cord injury triggers a complex set of events that lead to tissue healing without the restoration of normal function due to the poor regenerative capacity of the spinal cord. Nevertheless, current knowledge about the intrinsic regenerative ability of central nervous system axons, when in a supportive environment, has made the prospect of treating spinal cord injury a reality. Among the range of strategies under investigation, cell‐based therapies offer the most promising results, due to the multifactorial roles that these cells can fulfil. However, the best cell source is still a matter of debate, as are clinical issues that include the optimal cell dose as well as the timing and route of administration. In this context, the role of biomaterials is gaining importance. These can not only act as vehicles for the administered cells but also, in the case of chronic lesions, can be used to fill the permanent cyst, thus creating a more favourable and conducive environment for axonal regeneration in addition to serving as local delivery systems of therapeutic agents to improve the regenerative milieu. Some of the candidate molecules for the future are discussed in view of the knowledge derived from studying the mechanisms that facilitate the intrinsic regenerative capacity of central nervous system neurons. The future challenge for the multidisciplinary teams working in the field is to translate the knowledge acquired in basic research into effective combinatorial therapies to be applied in the clinic.
Immunoregulation of skin sensitization and regulatory T cells
Authors: Kimber Ian , Travis Mark A. , Martin Stefan F. , Dearman Rebecca J. 
Published: July 2012

It is well established that, in common with other adaptive immune responses, the acquisition of skin sensitization is carefully orchestrated and finely controlled. This is achieved in a number of ways. However, in recent years, there has been an increasing interest in the roles that regulatory T cells (Tregs) play in allergic contact dermatitis.Here, we review briefly the phenotype and function of Tregs, and consider how they may impact on various aspects of skin sensitization, including: inter‐individual differences in susceptibility; variations in the potency of chemical allergens; and the prevention of excessive, and potentially damaging, levels of sensitization.
Increased infection risk postliver transplant without pretransplant dental treatment
Authors: Helenius‐Hietala J , Åberg F , Meurman JH , Isoniemi H 
Published: July 2012

ObjectiveInfections cause considerable morbidity after liver transplantation (LT). Acute liver failure is a rapidly progressing life‐threatening condition where pretransplant dental evaluation is not always possible. We investigated how missing pretransplant dental treatment in acute or subacute liver failure correlates with post‐transplant infectious complications.Subjects and methodsMedical and dental data came from hospital records and infection data from the Finnish LT registry. The follow‐up was until February 2011. Of 51 patients (LT during 2000–2006), 16 had and 35 did not have dental treatment pretransplant.ResultsUnivariate Cox regression analysis demonstrated a 2.46‐fold (95% CI 1.06–5.69) infection risk among the patients omitted from dental treatment. After adjustment for either pretransplant factors alone or both pre‐ and post‐transplant factors, the corresponding infection risk increased, respectively, to 8.17‐fold (95% CI 2.19–30.6) and 8.54‐fold (95% CI 1.82–40.1). This increased risk involved a variety of bacterial, viral, and fungal infections of various sources both < 6 and > 6 months after transplantation.ConclusionHigh risk of infections was noticed in acute liver failure patients without pretransplant dental treatment, but a more severe medical condition might have influenced the results. We encourage eradication of dental infection foci whenever clinical condition allows.