bluuebunny/crossref_metadata_2025_split huggingface dataset:
An Australian tuberous sclerosis cohort: Are surveillance guidelines being met?
Authors: Chopra Maya , Lawson John A , Wilson Meredith , Kennedy Sean E , Taylor Peter , Buckley Michael F , Wargon Orli , Parasivam Gayathri , Camphausen Christoph , Yates Deborah , Mowat David 
Published: March 2011

Aim:  This study aims to describe the phenotypic and genotypic characteristics of 45 Australian patients with tuberous sclerosis complex (TSC), to assess risk factors for intellectual disability, to compare patients with TSC1 and TSC2 mutations and to assess adherence to surveillance recommendations.Methods:  Phenotypic features were recorded in 45 patients who fulfilled established criteria for a diagnosis of definite TSC. All patients underwent TSC1 and TSC2 sequencing and multiplex ligand probe amplification. Features were compared in patients with TSC1 mutations versus TSC2 mutations. Recent surveillance was recorded at the point of first contact. Surveillance adherence was compared in the adult and paediatric cohorts.Results:  This cohort consisted of 31 children and 14 adults with definite TSC. The rates of TSC manifestations and TSC1 and TSC2 mutation detection rates were consistent with previous studies. There was a trend towards greater severity for patients with TSC2 mutations compared with their TSC1 counterparts, particularly for autistic spectrum disorder, but this did not reach statistical significance. The presence of seizures was shown to be a risk factor for intellectual disability (P < 0.001). Overall, 12/45 patients (27%) were not undergoing recommended surveillance at the point of first contact. Surveillance guidelines were being followed in 3/31 (11%) children compared with 9/14 (64%) adult patients (P < 0.05).Conclusions:  The genotypic and phenotypic characteristics of this TSC cohort were consistent with previous studies. Surveillance rates in adult patients were significantly lower than in paediatric patients. This highlights the need for patients with TSC to undergo a focussed transition into adult services.
Fetal and post‐natal diagnosis of major congenital heart disease: Implications for medical and psychological care in the current era
Authors: Sholler Gary F , Kasparian Nadine A , Pye Victoria E , Cole Andrew D , Winlaw David S 
Published: March 2011

Aim:  The fetal or post‐natal diagnosis of major congenital cardiac abnormality has important medical and psychological consequences.Methods:  We reviewed infants who underwent cardiac surgery in the first year of life at the Heart Centre for Children, The Children's Hospital at Westmead during 2009. The aims of this study were to: (i) examine the key features of cardiac diagnosis and clinical outcome, and (ii) consider how these data can inform priorities for the delivery of clinical services.Results:  Over the 12‐month study period, a first cardiac surgical procedure was performed on 195 infants, with 85 infants (44%) diagnosed in the antenatal period. Of the total sample, a subset of 90 babies (46%) underwent their first procedure in the neonatal period, with 62% having had a fetal diagnosis. Major intracardiac lesions including truncus arteriosus (100%), single ventricular lesions (83%), pulmonary atresia with ventricular septal defect (78%) and transposition of the great arteries (53%) were diagnosed prior to birth. Improved haemodynamic stability at initial presentation was found in those with a fetal diagnosis. The overall mortality rate for all patients was 6.1% at 12 months, with a higher mortality in infants with single ventricle.Conclusions:  The contemporary paradigm of care for infants with major congenital heart disease requires a multidisciplinary approach to care, with improvements in clinician–clinician and clinician–family communication, and psychological support and education for families. Changes in the allocation of resources are required to meet this model of best practice.
Interpreting positive cultures of endotracheal aspirates: Factors associated with treatment decisions in ventilated neonates
Authors: Murila Florence , Francis Johny V , Bland Amy , Kumbla Surekha , Doherty Richard , Sehgal Arvind 
Published: March 2011

Aims:  Diagnosis of ventilator‐associated pneumonia in newborns is challenging because of ease of colonisation, non‐specific chest radiograph changes and lack of a consensus definition. The aims of this study were to review treatment decisions in neonates with culture‐positive endotracheal aspirate and to assess impact on respiratory outcomes using blinded review of radiological studies.Methods:  Charts from all very low birthweight neonates ventilated for >48 h and with positive culture were assessed. Chest radiographs were reviewed by a radiologist masked to the grouping of the episode (treated/not treated). Clinical, investigational and radiological features used in practice were assessed on impact on treatment decisions. Association between treatment and outcomes was assessed.Results:  Seventy‐four episodes of culture‐positive endotracheal aspirate were analysed in 38 babies. Fifty‐eight episodes were treated with antibiotics. Gestational age at birth and birthweight in both groups (treated vs. non‐treated) were statistically comparable (25.5 ± 3.1 vs. 27.2 ± 2.3 weeks and 809 ± 302 vs. 870 ± 262 g). Comparative chest radiographs were available in 51 of 58 treated episodes; deterioration was noted in 42 (82.3%). Ventilatory parameters were significantly higher in the treatment group and showed a significant improvement after antibiotics. Twenty‐three babies developed chronic lung disease. Odds ratio (of having chronic lung disease when treatment is initiated) was 4.5 (95% confidence interval = 0.97–20.8, P= 0.06).Conclusions:  Treated culture‐positive aspirate episodes were accompanied by higher ventilatory requirements, increased symptoms and elevated septic markers. Need for treatment was associated with greater likelihood of developing chronic lung disease.
Overexpression of Eg5 predicts unfavorable prognosis in non‐muscle invasive bladder urothelial carcinoma
Authors: Ding Sentai , Xing Naidong , Lu Jiaju , Zhang Hui , Nishizawa Koji , Liu Shuai , Yuan Xiaodong , Qin Yejun , Liu Ying , Ogawa Osuma , Nishiyama Hiroyuki 
Published: March 2011

Objective:  To investigate the relationship between Eg5 expression and prognosis of patients with non‐muscle invasive bladder urothelial carcinoma.Methods:  Eg5 expression was examined by immunohistochemistry in non‐muscle invasive urothelial carcinoma specimens (grade: G1, 32 cases; G2, 92 cases; and G3, 39 cases. Stage: pTa, 49 cases and pT1, 114 cases). The correlation between clinicopathological characteristics and Eg5 expression was evaluated. The prognostic significance of Eg5 immunoreactivity was analyzed through survival analysis in 163 non‐muscle invasive cases that were treated with transurethral resection and adjuvant intravesical instillations.Results:  The expression of Eg5 was significantly associated with tumor grade (P = 0.006), with a trend towards significant association with stage (P = 0.057). The 163 patients with non‐muscle invasive tumors were regularly followed with the mean of 32.52 (from 6 to 72) months. Univariate analysis showed Eg5 overexpression exhibited a significant unfavorable influence on intravesical recurrence (P = 0.012) while having only a marginal correlation with disease progression (P = 0.070). Subsequent Cox hazard multivariate analysis showed that both grade (P = 0.045) and Eg5 expression (P = 0.029) were independent predictors for early intravesical recurrence.Conclusions:  Overexpression of Eg5 correlates with poor differentiation of bladder cancer, and it represents an independent prognostic factor in predicting early intravesical recurrence in non‐muscle invasive bladder carcinoma patients.
The Big Five Personality Factors as Predictors of Changes Across Time in Burnout and Its Facets
Authors: Armon Galit , Shirom Arie , Melamed Samuel 
Published: March 2011

AbstractWe tested the effects of Neuroticism and Conscientiousness on burnout across time, controlling for age, gender, work hours, and depressive symptoms. Our theoretical model included both global burnout and its physical, emotional, and cognitive facets, consistent with the bifactor approach to modeling second‐order constructs in structural equation modeling. Data were gathered from 1,105 respondents (63% men) who completed questionnaires at Time 1 (T1) and approximately 24 months later at Time 2 (T2). Neuroticism positively predicted T1 global burnout and negatively predicted T1 and T2 emotional exhaustion. Conscientiousness negatively predicted T1 global burnout and T1 and T2 cognitive weariness, and positively predicted T1 and T2 emotional exhaustion. Our gender‐specific exploratory analysis revealed that for each gender, Neuroticism and Conscientiousness predicted different facets of burnout at T1 and T2. We recommend that future research test the possibility that the associations of Neuroticism and Conscientiousness with global burnout and its facets may be gender specific.
Plastid expression of a double‐pentameric vaccine candidate containing human papillomavirus‐16 L1 antigen fused with LTB as adjuvant: transplastomic plants show pleiotropic phenotypes
Authors: Waheed Mohammad T. , Thönes Nadja , Müller Martin , Hassan Syed W. , Gottschamel Johanna , Lössl Elke , Kaul Hans‐Peter , Lössl Andreas G. 
Published: March 2011

SummaryHuman papillomavirus (HPV) causes cervical cancer in women worldwide, which is currently prevented by vaccines based on virus‐like particles (VLPs). However, these vaccines have certain limitations in their availability to developing countries, largely due to elevated costs. Concerning the highest burden of disease in resource‐poor countries, development of an improved mucosal and cost‐effective vaccine is a necessity. As an alternative to VLPs, capsomeres have been shown to be highly immunogenic and can be used as vaccine candidate. Furthermore, coupling of an adjuvant like Escherichia coli heat‐labile enterotoxin subunit B (LTB) to an antigen can increase its immunogenicity and reduce the costs related to separate co‐administration of adjuvants. Our study demonstrates the expression of two pentameric proteins: the modified HPV‐16 L1 (L1_2xCysM) and LTB as a fusion protein in tobacco chloroplasts. Homoplasmy of the transplastomic plants was confirmed by Southern blotting. Western blot analysis showed that the LTB‐L1 fusion protein was properly expressed in the plastids and the recombinant protein was estimated to accumulate up to 2% of total soluble protein. Proper folding and display of conformational epitopes for both LTB and L1 in the fusion protein was confirmed by GM1‐ganglioside binding assay and antigen capture ELISA, respectively. However, all transplastomic lines showed chlorosis, male sterility and growth retardation, which persisted in the ensuing four generations studied. Nevertheless, plants reached maturity and produced seeds by pollination with wild‐type plants. Taken together, these results pave the way for the possible development of a low‐cost adjuvant‐coupled vaccine with potentially improved immunogenicity against cervical cancer.
Chloroplast‐derived vaccines against human diseases: achievements, challenges and scopes
Authors: Lössl Andreas G. , Waheed Mohammad T. 
Published: March 2011

SummaryInfectious diseases represent a continuously growing menace that has severe impact on health of the people worldwide, particularly in the developing countries. Therefore, novel prevention and treatment strategies are urgently needed to reduce the rate of these diseases in humans. For this reason, different options can be considered for the production of affordable vaccines. Plants have been proved as an alternative expression system for various compounds of biological importance. Particularly, plastid genetic engineering can be potentially used as a tool for cost‐effective vaccine production. Antigenic proteins from different viruses and bacteria have been expressed in plastids. Initial immunological studies of chloroplast‐derived vaccines have yielded promising results in animal models. However, because of certain limitations, these vaccines face many challenges on production and application level. Adaptations to the novel approaches are needed, which comprise codon usage and choice of proven expression cassettes for the optimal yield of expressed proteins, use of inducible systems, marker gene removal, selection of specific antigens with high immunogenicity and development of tissue culture systems for edible crops to prove the concept of low‐cost edible vaccines. As various aspects of plant‐based vaccines have been discussed in recent reviews, here we will focus on certain aspects of chloroplast transformation related to vaccine production against human diseases.
Happy to See Me, Aren’t You, Sally? Signal Detection Analysis of Emotion Detection in Briefly Presented Male and Female Faces
Authors: PIXTON TONYA S. 
Published: March 2011

Pixton, T. S. (2011). Happy to see me, aren’t you, Sally? Signal detection analysis of emotion detection in briefly presented male and female faces. Scandinavian Journal of Psychology 52, 361–368.Using signal detection methods, possible effects of emotion type (happy, angry), gender of the stimulus face, and gender of the participant on the detection and response bias of emotion in briefly presented faces were investigated. Fifty‐seven participants (28 men, 29 women) viewed 90 briefly presented faces (30 happy, 30 angry, and 30 neutral, each with 15 male and 15 female faces) answering yes if the face was perceived as emotional and no if it was not perceived as emotional. Sensitivity [d’, z(hit rate) minus z(false alarm rate)] and response bias (β, likelihood ratio of “signal plus noise” vs. “noise”) were measured for each face combination for each presentation time (6.25, 12.50, 18.75, 25.00, 31.25 ms). The d’ values were higher for happy than for angry faces and higher for angry‐male than for angry‐female faces, and there were no effects of gender‐of‐participant. Results also suggest a greater tendency for participants to judge happy‐female faces as emotional, as shown by lower β values for these faces as compared to the other emotion‐gender combinations. This happy‐female response bias suggests, at least, a partial explanation to happy‐superiority effects in studies where performance is only measured as percent correct responses, and, in general, that women are expected to be happy.
Preliminary examination of the psychometric properties of the Psychiatric Scepticism Scale
Authors: SWAMI VIREN , FURNHAM ADRIAN 
Published: March 2011

Swami, V. & Furnham, A. (2011). Preliminary examination of the psychometric properties of the Psychiatric Scepticism Scale. Scandinavian Journal of Psychology 52, 399–403.Despite much commentary on “psychiatric skepticism”, there remains a dearth of appropriate psychometric scales for the measurement of this concept. To overcome this limitation, the present study examined the psychometric properties and correlates of the recently developed Psychiatric Scepticism Scale (PSS). A total of 564 individuals from the community in London, England, completed the PSS along with measures of anti‐scientific attitudes, attitudes to authority, knowledge of mental health disorders, and demographics. Results showed that the PSS has a one‐dimensional factor structure with very good internal consistency. In addition, it showed adequate convergent (anti‐scientific attitudes, knowledge of mental health disorders) and construct validity (attitudes to authority, religiosity). Results also showed that there were small but significant differences in psychiatric skepticism by ethnicity and education, but not sex or previous diagnosis of mental health disorder. These results confirm that the PSS has adequate psychometric properties for the measurement of psychiatric skepticism.
Selective suppression of self‐initiated sounds in an auditory stream: An ERP study
Authors: Baess Pamela , Horváth János , Jacobsen Thomas , Schröger Erich 
Published: March 2011

AbstractNumerous studies have shown that the N1 event‐related potential (ERP) response is attenuated when it is elicited by self‐initiated sounds. This N1 suppression effect is generally interpreted to reflect an internal prediction mechanism, which enables the discrimination of the sensory consequences of our own actions and those of others. The blocked design used in the forerunner studies (i.e., self‐ and externally initiated sounds presented in different blocks) seriously limits the relevance of these findings, because the N1 effect can simply be explained by contextual task differences. In the present study, self‐ and externally initiated sounds were mixed within blocks. N1 suppression was found, and its magnitude was even larger than that observed in a traditional blocked condition. This result supports the involvement of an internal prediction mechanism in the discrimination of the sensory consequences of one's own actions and those of others.
Expecting the unexpected: An N400 study of risky sentence processing in adolescents
Authors: Grose‐Fifer Jillian , Hoover Steven , Zottoli Tina , Rodrigues Andrea 
Published: March 2011

AbstractTeens often engage in risk taking. Avoiding risk may be aided by rapid access to cognitive models for danger. This study investigated whether these schemata are immature in adolescence. An N400 sentential priming paradigm compared risky, predictable, and incongruent sentence processing in adolescents and adults. Adults and teens processed predictable sentences similarly, as evidenced by equivalent N400 priming. However, in adults, more activation was required to access final words in a risky sentence than when the situation was predictable and benign. Conversely, teens showed little difference in N400s generated by risky or expected sentences. This suggests that risky scenario final words were unexpected for adults but not for adolescents because of age‐related differences in world knowledge and risk‐related schemata. This study may help to explain why teenagers engage in risky activities when there is little time for deliberative thought.
The cannabinoid receptor inverse agonist AM251 regulates the expression of the EGF receptor and its ligands via destabilization of oestrogen‐related receptor α protein
Authors: Fiori JL , Sanghvi M , O'Connell MP , Krzysik‐Walker SM , Moaddel R , Bernier M 
Published: March 2011

BACKGROUND AND PURPOSE AM251 is an inverse agonist of the cannabinoid 1 receptor (CB1R) that can exert ‘off‐target’ effects in vitro and in CB1R knock‐out mice. AM251 is also potent at modulating tumour cell growth, suggesting that growth factor‐mediated oncogenic signalling could be regulated by AM251. Since dysregulation of the EGF receptor has been associated with carcinogenesis, we examined AM251 regulation of EGF receptor (EGFR) expression and function.EXPERIMENTAL APPROACH The various biological functions of AM251 were measured in CB1R‐negative human cancer cells. Pharmacological and genetic approaches were used to validate the data.KEY RESULTS The mRNA levels for EGFR and its associated ligands, including HB‐EGF, were induced several fold in PANC‐1 and HCT116 cells in response to AM251. This event was associated with enhanced expression of EGFR on the cell surface with concomitant increase in EGF‐induced cellular responses in AM251‐treated cells. Exposure to XCT790, a synthetic inverse agonist of the orphan nuclear oestrogen‐related receptor α (ERRα), also induced EGFR and HB‐EGF expression to the same extent as AM251, whereas pretreatment with the ERRα‐selective agonist, biochanin A, blunted AM251 actions. AM251 promoted the degradation of ERRα protein without loss of the corresponding mRNA. Knock‐down of ERRα by siRNA‐based approach led to constitutive induction of EGFR and HB‐EGF levels, and eliminated the biological responses of AM251 and XCT790. Finally, AM251 displaced diethylstilbestrol prebound to the ligand‐binding domain of ERRα.CONCLUSIONS AND IMPLICATIONS AM251 up‐regulates EGFR expression and signalling via a novel non‐CB1R‐mediated pathway involving destabilization of ERRα protein in selected cancer cell lines.
Influence of ABCB1 genetic polymorphisms on the pharmacokinetics of risperidone in healthy subjects with CYP2D6*10/*10
Authors: Yoo Hee‐Doo , Lee Sang‐No , Kang Hyun‐Ah , Cho Hea‐Young , Lee Il‐Kwon , Lee Yong‐Bok 
Published: March 2011

BACKGROUND AND PURPOSE The objective of this study was to investigate the combined influence of genetic polymorphisms in ABCB1 and CYP2D6 genes on risperidone pharmacokinetics.EXPERIMENTAL APPROACH Seventy‐two healthy Korean volunteers receiving a single oral dose of 2 mg risperidone were included in this study.KEY RESULTS Significant differences were observed between the ABCB1 3435C>T genotypes for the pharmacokinetic parameters (peak serum concentration) of risperidone and the active moiety (risperidone and its main metabolite, 9‐hydroxyrisperidone). There were no significant differences in the area under the serum concentration‐time curves of risperidone and the active moiety among the ABCB1 2677G>T/A and 3435C>T genotypes. However, the peak serum concentration and area under the serum concentration‐time curves were significantly different among the ABCB1 3435C>T genotypes in CYP2D6*10/*10.CONCLUSIONS AND IMPLICATIONS These findings indicate that polymorphisms of ABCB1 3435C>T in individuals with CYP2D6*10/*10, which has low metabolic activity, could play an important role in the potential adverse effects or toxicity of risperidone.
Kaempferol acts through mitogen‐activated protein kinases and protein kinase B/AKT to elicit protection in a model of neuroinflammation in BV2 microglial cells
Authors: Park SE , Sapkota K , Kim S , Kim H , Kim SJ 
Published: March 2011

BACKGROUND AND PURPOSE Kaempferol, a dietary flavonoid and phyto‐oestrogen, is known to have anti‐inflammatory properties. Microglial activation has been implicated in various neurodegenerative diseases. Anti‐inflammatory effects of kaempferol and the underlying mechanisms were investigated by using LPS‐stimulated microglial BV2 cells.EXPERIMENTAL APPROACH Cell viability was measured using MTT and neutral red assays. elisa, Western blot, immunocytochemistry and electrophoretic mobility‐shift assay were used to analyse NO, PGE2, TNF‐α and IL‐1β production, inducible NOS (iNOS), COX‐2 expression and the involvement of signalling pathways such as toll‐like receptor‐4 (TLR4), MAPK cascades, PKB (AKT) and NF‐κB. Accumulation of reaction oxygen species (ROS) was measured by nitroblue tetrazolium and 2′7′‐dichlorofluorescein diacetate assay. Matrix metalloproteinase activity was investigated by zymography and immunoblot assay. Phagocytotic activity was assessed by use of latex beads.KEY RESULTS Kaempferol significantly attenuated LPS‐induced NO, PGE2, TNF‐α, IL‐1β and ROS production and phagocytosis in a concentration‐dependent manner. Kaempferol suppressed the expression of iNOS, COX‐2, MMP‐3 and blocked the TLR4 activation. Moreover, kaempferol inhibited LPS‐induced NF‐κB activation and p38 MAPK, JNK and AKT phosphorylation.CONCLUSION AND IMPLICATIONS Kaempferol was able to reduce LPS‐induced inflammatory mediators through the down‐regulation of TLR4, NF‐κB, p38 MAPK, JNK and AKT suggesting that kaempferol has therapeutic potential for the treatment of neuroinflammatory diseases.