bluuebunny/crossref_metadata_2025 huggingface dataset:
Staphylococcus aureus Evades the Extracellular Antimicrobial Activity of Mast Cells by Promoting Its Own Uptake
Authors: Abel Jens , Goldmann Oliver , Ziegler Christina , Höltje Claudia , Smeltzer Mark S. , Cheung Ambrose L. , Bruhn Daniela , Rohde Manfred , Medina Eva
Published: June 2011

In this study, we investigated the interactions of Staphylococcus aureus with mast cells, which are multifunctional sentinels lining the surfaces of the body. We found that bone marrow-derived murine mast cells (BMMC) exerted a powerful phagocytosis-independent antimicrobial activity against S. aureus. Both the release of extracellular traps as well as discharge of antimicrobial compounds were the mechanisms used by the BMMC to kill extracellular S. aureus. This was accompanied by the secretion of mediators such as TNF-α involved in the recruitment of effector cells. Interestingly, S. aureus subverted the extracellular antimicrobial activity of the BMMC by internalizing within these cells. S. aureus was also capable to internalize within human mast cells (HMC-1) and within murine skin mast cells during in vivo infection. Bacteria internalization was, at least in part, mediated by the α5β1 integrins expressed on the surface of the mast cell. In the intracellular milieu, the bacterium survived and persisted by increasing the cell wall thickness and by gaining access into the mast cell cytosol. The expression of α-hemolysin was essential for staphylococci intracellular persistence. By hiding within the long-life mast cells, staphylococci not only avoid clearance but also establish an infection reservoir that could contribute to chronic carriage.
Effect of Nilotinib on Bleomycin-Induced Acute Lung Injury and Pulmonary Fibrosis in Mice
Authors: Rhee Chin Kook , Lee Sang Haak , Yoon Hyung Kyu , Kim Seok Chan , Lee Sook Young , Kwon Soon Suk , Kim Young Kyoon , Kim Kwan Hyoung , Kim Tae Jung , Kim Jin Woo
Published: June 2011

Background: The tyrosine kinase inhibitor imatinib mesylate was developed as an inhibitor of the kinase activity of BCR-ABL. However, imatinib also has potent inhibitory activity against the platelet-derived growth factor receptor (PDGFR). Nilotinib is approved for treating patients with chronic myeloid leukemia showing resistance or intolerance to imatinib. Like imatinib, nilotinib selectively inhibits the tyrosine kinase activity of PDGFR. Objectives: We examined the effect of imatinib and nilotinib on acute lung injury and pulmonary fibrosis in a mouse model. Methods: Mice were treated by intratracheal instillation of bleomycin. Imatinib or nilotinib were administered by oral gavage. To study the early inflammatory and late fibrotic phases of lung injury, mice were sacrificed on days 3, 7, 14 and 21 after bleomycin instillation. Results: Histopathology showed that imatinib and nilotinib attenuated the extent of lung injury and fibrosis. The numbers of inflammatory cells and levels of IL-6, IL-1β and tumor necrosis factor-α were decreased in the imatinib and nilotinib groups on days 3 and 7. Imatinib and nilotinib therapy significantly reduced the levels of hydroxyproline on days 14 and 21, which was accompanied by decreased expression levels of transforming growth factor (TGF)-β1 and PDGFR-β. Imatinib and nilotinib also significantly reduced the expression levels of the genes for TGF-β1 and platelet-derived growth factor (PDGF). Imatinib and nilotinib treatment also significantly inhibited the PDGF-induced proliferation of lung fibroblasts in vitro. When imatinib or nilotinib was given 7 days after the instillation of bleomycin, only nilotinib attenuated pulmonary fibrosis. Conclusions: Imatinib and nilotinib attenuated bleomycin-induced acute lung injury and pulmonary fibrosis in mice. In a therapeutic model, nilotinib showed more potent antifibrotic effects than imatinib.
Corpus Callosum Atrophy in Patients with Mild Alzheimer’s Disease
Authors: Frederiksen Kristian Steen , Garde Ellen , Skimminge Arnold , Ryberg Charlotte , Rostrup Egill , Baaré William F.C. , Siebner Hartwig R. , Hejl Anne-Mette , Leffers Anne-Mette , Waldemar Gunhild
Published: June 2011

Background/Objectives: Several studies have found atrophy of the corpus callosum (CC) in patients with Alzheimer’s disease (AD). However, it remains unclear whether callosal atrophy is already present in the early stages of AD, and to what extent it may be associated with other structural changes in the brain, such as age-related white matter changes (ARWMC) and progression of the disease. Meth ods: Twenty-eight patients in the early stages of AD and 50 non-demented elderly subjects with varying degrees of ARWMC were investigated using MRI. The CC was assessed semi-automatically, and ARWMC were rated according to the Fazekas scale. Results: A significant difference in posterior CC size could be detected between non-demented elderly subjects and early stage AD patients. The sizes of the total CC, rostral body and splenium at baseline were correlated with change from baseline MMSE score after a 1-year follow-up in AD patients. There was no association between CC size and ARWMC. Conclusions: The present findings indicate that posterior CC atrophy is present in mild AD independently of ARWMC. Furthermore, CC atrophy may be associated with cognitive deterioration.
Suicidal Behavior in Prodromal Huntington Disease
Authors: Fiedorowicz Jess G. , Mills James A. , Ruggle Adam , Langbehn Douglas , Paulsen Jane S. ,   PREDICT-HD Investigators of the Huntington Study Group
Published: June 2011

Background: Several studies have suggested a greater risk of suicide in Huntington disease (HD); however, unique risk factors for suicide in HD are not established. Objective: We sought to determine risk factors for suicidal behavior, defined as suicide or attempted suicide, in prodromal HD. Methods: From the prospective PREDICT-HD cohort, we identified 735 cases with HD gene expansion but no manifest symptoms of HD and 194 non-gene-expanded controls. In survival analysis, a number of potential risk factors for suicidal behavior were assessed, including symptoms of depression, hopelessness, substance abuse, marital status, gender, and psychiatric history. Results: During a mean of 3.7 years of prospective follow-up, 12 cases (1.6%) attempted suicide and 1 completed suicide (0.1%). No suicides were observed among controls. In univariate Cox proportional hazards regression models, a history of suicide attempts (HR 8.5, 95% CI 2.8–26.1, p < 0.0002) and a Beck Depression Inventory II score >13 (HR 7.2, 95% CI 2.3–22.0, p < 0.0006) were associated with suicidal behavior. These risk factors had independent effects in multivariate models. A history of incarceration in the past 2 years was also associated (HR 12.5, 95% CI 2.7–56.6, p < 0.002), though uncommon. No further risk factors were identified. Conclusion: A history of suicide attempts and the presence of depression are strongly predictive of suicidal behavior in prodromal HD. As these risk factors are among the most robust risk factors for suicide, established suicide risk factors appear applicable to those with prodromal HD.
Genetic Variation in KIFAP3 Is Associated with an Upper Motor Neuron-Predominant Phenotype in Amyotrophic Lateral Sclerosis
Authors: Orsetti V. , Pegoraro E. , Cima V. , D’Ascenzo C. , Palmieri A. , Querin G. , Volpe M. , Ermani M. , Angelini C. , Sorarù G.
Published: June 2011

Background: Some authors have recently reported that the CC genotype of single-nucleotide polymorphism (SNP) rs1541160 mapping within the kinesin-associated protein 3 (KIFAP3) gene is associated with increased survival in sporadic amyotrophic lateral sclerosis (sALS). Objective and Methods: The relationship between the rs1541160 genotype and several clinical features of 228 ALS patients was evaluated with the intent of assessing any association between the ALS phenotype and KIFAP3. The SNP rs1541160 within the KIFAP3 expression profile was investigated using real-time PCR in a group of 6 patients harboring the CC genotype and in 12 patients harboring the TT genotype. Results: Analysis of our patients’ clinical features showed that almost half of those with the CC genotype were classified as having upper motor neuron-predominant ALS (UMN-ALS). Conversely, there was an approximately 10% frequency of UMN-ALS in both the TT and the TC patient groups as well as in the entire cohort considered as a whole (p < 0.005). The SNP rs1541160 genotype did not appear to have any effect on patient survival or on KIFAP3 expression. Conclusions: The incidence of the UMN-ALS phenotype in the CC patients of this cohort supports the hypothesis that the SNP rs1541160 within the KIFAP3 gene is a potential modifier of the ALS phenotype.
Relationship between Glycosylated Hemoglobin and Blood Glucose during Progression of Chronic Kidney Disease
Authors: Agarwal Rajiv , Light Robert P.
Published: June 2011

Background: The value of measurement of glycosylated hemoglobin (HgbA1C) in determining the degree of glycemic control in patients with chronic kidney disease (CKD) is unclear. Methods: A single-center, prospective cohort study was conducted in 128 veterans with diabetes mellitus and CKD. HgbA1C was measured as clinically indicated and its relationship with random blood glucose (RBG) measurement evaluated prospectively over up to 10 years in three groups (end-stage renal disease (ESRD), CKD and controls who had diabetes but no CKD). Results: Between 1995 and 2011, in the control group, glycemic control as assessed by HgbA1C was stable but improved when assessed by RBG. However, both the CKD and ESRD groups experienced declines in RBG and HgbA1C. Declining HgbA1C and RBG were noted prior to onset of dialysis. A fall in HgbA1C remained after adjustment for RBG. A strong inverse relationship was seen between CKD stage and HgbA1C even after adjusting for RBG such that the relationship between RBG levels and HgbA1C was modified by CKD. Conclusions: In diabetic patients with late-stage CKD, glycemic control shows an improvement. However, HgbA1C <7% may overestimate the degree of glycemic control. Therefore, reliance on HgbA1C without home blood glucose monitoring may result in poor diabetes control.
Rivaroxaban differentially influences ex vivo global coagulation assays based on the administration time
Authors: Hesse Christian , Gertrud Stratmann , Lindhoff-Last Edelgard , Mani Helen
Published: June 2011

It was the objective of this study to quantify the effects of rivaroxaban administration on global coagulation parameters associated with routine clinical procedures, we collected plasma samples from patients undergoing major orthopaedic surgery receiving rivaroxaban at various time points after drug administration. Forty-seven patients received rivaroxaban (10 mg daily) for venous thromboembolism prophylaxis. Blood samples were collected at four different time points: A) before surgery; B) before drug administration at day 4–5 after surgery (steady state of rivaroxaban); C) 2 hours (h) after drug administration and D) 12 h after drug administration. The prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin time (TT), antithrombin (AT) level, fibrinogen level by Clauss method (FibC), and derived fibrinogen (dFIB) level were assessed with various reagents. At 2 h after rivaroxaban administration, the PT and aPTT clotting times were significantly prolonged to different extents up to 1.4 fold, whereas 12 h after drug administration, no significant effect was observed. Rivaroxaban administration had no influence on the TT or the FibC concentration. The dFIB assay was differentially affected by rivaroxaban when different reagents were tested. The AT assay dependent on thrombin activity was not influenced by rivaroxaban, whereas the AT levels dependent on factor Xa activity were significantly increased by rivaroxaban. Clinicians should be aware of the time-dependent influence of rivaroxaban on factor Xa-dependent routine coagulation assays. Therefore, routine coagulation parameters should be assessed directly before drug administration to keep the interaction of rivaroxaban low.
Social Class Predicts Generalized Trust But Only in Wealthy Societies
Authors: Hamamura Takeshi
Published: June 2011

This research examined the relationship between social class and generalized trust, or a belief that others have a benign intention in social interactions, in a diverse set of societies represented in the World Values Survey. The strength of the relationship varied significantly across societies: Although social class was a positive predictor of generalized trust in wealthy countries, as reported in past research, among less wealthy countries social class was uncorrelated with trust. These results indicate that resources available to individuals of high social class may make a trusting belief more rewarding; nevertheless, in less wealthy societies, the socio-political-economic infrastructure that supports generalized trust is unavailable, and therefore even individuals of high social class are reluctant to trust others. This finding extends prior theorizing on trust in finding the interactive relationship between an individual-level factor and a society-level factor in shaping individuals’ inclination toward trust.
The Emergence of an Unmarkedness Effect in Mandarin Speech Errors: Nasals in a Coda Position
Authors: Hsu Hsiu-ling
Published: June 2011

This study aims to explore how the markedness effect shapes Mandarin slips of the tongue with respect to nasals in syllable-final positions. Data were collected via natural speech and elicitation tasks from 35 participants’ reading of 346 test items. Three hundred and eight slips in Mandarin from natural data and 360 slips from elicited data were investigated. This study shows that there exists a strong preference for unmarked coronal nasals over marked dorsal nasals in the syllable-final position in both spontaneous and elicited Mandarin slips. This tendency toward the unmarked [n] reveals that the influence of the unmarkedness effect is present in the coda position in Mandarin slips. Interestingly, this tendency is inconsistent with that found in a previous study by Wan of Mandarin slips in the onset position, which shows a tendency for coronals to be replaced by other sounds. This suggests an onset—coda distinction occurring in Mandarin speech errors, which implies that the markedness effect works differently in distinct syllable positions. More precisely, the unmarkedness effect emerges in the coda position in Mandarin slips, leading to the occurrence of unmarked segments, whereas it submerges in the onset position, resulting in marked segments.
Blended Sentencing, Early Release, and Recidivism of Violent Institutionalized Delinquents
Authors: Trulson Chad R. , Haerle Darin R. , DeLisi Matt , Marquart James W.
Published: June 2011

This study explores the recidivism outcomes of 1,804 serious and violent delinquents sentenced under a blended sentencing statute and released early by juvenile correctional authorities without continuing their blended sentence in adult prisons. Released at an average age of 19, roughly 50% of releases were rearrested for a felony-level offense postrelease. The remaining 50% of all releases did not incur a postrelease arrest or were rearrested for an offense no higher than a misdemeanor. Measures for assaultive institutional misconduct and prior delinquent adjudications were predictive of recidivism in models examining rearrest for any offense and rearrest for a felony only. Substance abusers, gang members, those with a gang-related commitment offense, and homicide-related state commitments were significantly more likely to be rearrested for any offense postrelease. This article ends with a discussion of implications specific to this high risk cohort of released delinquent offenders.
Power Fosters Context-Independent, Analytic Cognition
Authors: Miyamoto Yuri , Ji Li-Jun
Published: June 2011

The present research tested the hypothesis that power, defined as the capacity to influence others, promotes analytic cognitive processing, by examining the use of linguistic categories and the categorization of objects. Supporting the hypothesis, recalling instances of influencing others facilitated the use of adjectives and discouraged the use of verbs to describe others (Study 1). Recalling instances of influencing others also promoted taxonomic, instead of thematic, categorization (Study 2). Furthermore, the authors also examined the effect of power in a real-life context. They examined whether socioeconomic status (SES) differences in cognitive processing can be partly explained by sense of agency, an antecedent of power (Study 3); high SES individuals made more taxonomic categorization than did low SES individuals, and a sense of agency partially mediated the SES differences in categorization. These findings underscore the role of power in shaping cognitive processes.
A Grounded Theory of the Process of Adherence to Oral Chemotherapy in Hispanic and Caucasian Children and Adolescents With Acute Lymphoblastic Leukemia
Authors: Landier Wendy , Hughes Cynthia B. , Calvillo Evelyn R. , Anderson Nancy L. R. , Briseño-Toomey Deborah , Dominguez Leticia , Martinez Alex M. , Hanby Cara , Bhatia Smita
Published: June 2011

Children and adolescents with acute lymphoblastic leukemia (ALL) receive treatment that relies on daily self- or parent/caregiver-administered oral chemotherapy for approximately 2 years. Despite the fact that pediatric ALL is uniformly fatal without adequate treatment, nonadherence to oral chemotherapy has been observed in up to one third of patients. Little is known about the reasons for nonadherence in these patients. This study used Straussian grounded theory methodology to develop and validate a model to explain the process of adherence to oral chemotherapy in children and adolescents with ALL. Thirty-eight semistructured interviews (with 17 patients and 21 parents/caregivers) and 4 focused group discussions were conducted. Three stages were identified in the process of adherence: (a) Recognizing the Threat, (b) Taking Control, and (c) Managing for the Duration. Doing Our Part was identified as the core theme explaining the process of adherence and involves the parent (or patient) taking responsibility for assuring that medications are taken as prescribed. Understanding the association between taking oral chemotherapy and control/cure of leukemia ( Making the Connection) appeared to mediate adherence behaviors.
How family carers view hospital discharge planning for the older person with a dementia
Authors: Bauer Michael , Fitzgerald Les , Koch Susan , King Susan
Published: June 2011

This study reports on the experiences of 25 family carers of the hospital discharge planning process for their family member with a dementia. Analysis of the data indicates that the needs of family carers were not always addressed in the hospital discharge process and that discharge planning and execution is in need of improvement.
Impaired microvascular properties in uncomplicated type 1 diabetes identified by Doppler ultrasound of the ocular circulation
Authors: Lockhart Christopher J , McCann Aaron , Agnew Christina A , Hamilton Paul K , Quinn Cathy E , McClenaghan Vivienne , Patterson Christopher , Canice McGivern R. , Harbinson Mark T , McVeigh Gary E
Published: June 2011

Objective: Quantification of Doppler flow velocity waveforms has been shown to predict adverse cardiovascular outcomes and identify altered downstream haemodynamics and vascular damage in a number of organ beds. We employed novel techniques to quantify Doppler flow velocity waveforms from the retro bulbar circulation.

Methods and results: In total, 39 patients with uncomplicated Type 1 diabetes mellitus, and no other significant cardiovascular risk factors were compared with 30 control subjects. Flow velocity waveforms were captured from the ophthalmic artery (OA), central retinal artery (CRA) and the common carotid artery. The flow velocity profiles were analysed in the time domain to calculate the resistive index (RI), and time-frequency domain using novel discrete wavelet transform methods for comparison. Analysis of flow waveforms from the OA and CRA identified specific frequency band differences between groups, occurring independently of potential haemodynamic or metabolic confounding influences. No changes were identified in the calculated RI from any arterial site.

Conclusion: Novel analysis of the arterial flow velocity waveforms recorded from the retro bulbar circulation identified quantifiable differences in Doppler flow velocity waveform morphology in patients with diabetes prior to the development of overt retinopathy. The technique may be useful as an additional marker of cardiovascular risk.
Finite-element modelling of a novel flanging process on a cylinder with a large diameter–thickness ratio
Authors: Fan S Q , Zhao S D , Zhang Q , Wang C H
Published: June 2011

To solve existing problems of current processing methods for flanged cylinders with a large diameter–thickness ratio, a double-roller clamping spinning (DRCS) process is proposed as a novel flanging process on a cylinder. Finite-element (FE) numerical simulation is used to research the DRCS process. First, on the basis of non-linear FE analysis software Abaqus/Explicit, an FE model for the DRCS process is established. Second, some key problems in FE modelling of DRCS are solved, for example, the definition of contact, the treatment of meshing, the load curves applied on the rollers, the optimal clamping position, and the bending point on the defined initial workpiece. Then, the reliability of the FE model is verified using the energy-conservation principle and experimental results. Finally, the DRCS process of the flange is simulated using the verified FE model, and the equivalent stress field, equivalent strain field, and wall-thickness distribution on the flange during the whole forming process are obtained. The FE model established in this study can be used to explore and develop the DRCS process.
Ddx18 is essential for cell-cycle progression in zebrafish hematopoietic cells and is mutated in human AML
Authors: Payne Elspeth M. , Bolli Niccolò , Rhodes Jennifer , Abdel-Wahab Omar I. , Levine Ross , Hedvat Cyrus V. , Stone Richard , Khanna-Gupta Arati , Sun Hong , Kanki John P. , Gazda Hanna T. , Beggs Alan H. , Cotter Finbarr E. , Look A. Thomas
Published: June 2011

In a zebrafish mutagenesis screen to identify genes essential for myelopoiesis, we identified an insertional allele hi1727, which disrupts the gene encoding RNA helicase dead-box 18 (Ddx18). Homozygous Ddx18 mutant embryos exhibit a profound loss of myeloid and erythroid cells along with cardiovascular abnormalities and reduced size. These mutants also display prominent apoptosis and a G1 cell-cycle arrest. Loss of p53, but not Bcl-xl overexpression, rescues myeloid cells to normal levels, suggesting that the hematopoietic defect is because of p53-dependent G1 cell-cycle arrest. We then sequenced primary samples from 262 patients with myeloid malignancies because genes essential for myelopoiesis are often mutated in human leukemias. We identified 4 nonsynonymous sequence variants (NSVs) of DDX18 in acute myeloid leukemia (AML) patient samples. RNA encoding wild-type DDX18 and 3 NSVs rescued the hematopoietic defect, indicating normal DDX18 activity. RNA encoding one mutation, DDX18-E76del, was unable to rescue hematopoiesis, and resulted in reduced myeloid cell numbers in ddx18hi1727/+ embryos, indicating this NSV likely functions as a dominant-negative allele. These studies demonstrate the use of the zebrafish as a robust in vivo system for assessing the function of genes mutated in AML, which will become increasingly important as more sequence variants are identified by next-generation resequencing technologies.
Chromatin boundaries require functional collaboration between the hSET1 and NURF complexes
Authors: Li Xingguo , Wang Shaohua , Li Ying , Deng Changwang , Steiner Laurie A. , Xiao Hua , Wu Carl , Bungert Jörg , Gallagher Patrick G. , Felsenfeld Gary , Qiu Yi , Huang Suming
Published: June 2011

Chromatin insulators protect erythroid genes from being silenced during erythropoiesis, and the disruption of barrier insulator function in erythroid membrane gene loci results in mild or severe anemia. We showed previously that the USF1/2-bound 5′HS4 insulator mediates chromatin barrier activity in the erythroid-specific chicken β-globin locus. It is currently not known how insulators establish such a barrier. To understand the function of USF1, we purified USF1-associated protein complexes and found that USF1 forms a multiprotein complex with hSET1 and NURF, thus exhibiting histone H3K4 methyltransferase- and ATP-dependent nucleosome remodeling activities, respectively. Both SET1 and NURF are recruited to the 5′HS4 insulator by USF1 to retain the active chromatin structure in erythrocytes. Knock-down of NURF resulted in a rapid loss of barrier activity accompanied by an alteration of nucleosome positioning, increased occupancy of the nucleosome-free linker region at the insulator site, and increased repressive H3K27me3 levels in the vicinity of the HS4 insulator. Furthermore, suppression of SET1 reduced barrier activity, decreased H3K4me2 and acH3K9/K14, and diminished the recruitment of BPTF at several erythroid-specific barrier insulator sites. Therefore, our data reveal a synergistic role of hSET1 and NURF in regulating the USF-bound barrier insulator to prevent erythroid genes from encroachment of heterochromatin.
IL-17–producing invariant NKT cells in lymphoid organs are recent thymic emigrants identified by neuropilin-1 expression
Authors: Milpied Pierre , Massot Bérangère , Renand Amédée , Diem Séverine , Herbelin André , Leite-de-Moraes Maria , Rubio Marie-Thérèse , Hermine Olivier
Published: June 2011

Despite increasing knowledge on the mechanisms of invariant natural killer T (iNKT)–cell development in the thymus, the function of recent thymic emigrant (RTE) iNKT cells remains largely unexplored, principally because of a lack of bona fide markers to distinguish RTE from long-lived iNKT cells. Whether the recently described IL-17–producing iNKT cell subset is part of RTE has notably not been addressed. In the present study, we show that neuropilin-1 (Nrp-1), a transmembrane receptor mainly found on T-regulatory (Treg) cells in the murine immune system, is specifically expressed on RTE iNKT cells in naive mice. We used the Nrp-1 marker to discriminate RTE from mature iNKT cells and compare their functions. We show that RTE iNKT cells proliferate more than mature iNKT cells after in vitro activation; that, unlike mature iNKT cells, most RTE iNKT cells fail to rapidly produce IFN-γ and IL-4 after in vivo activation; and, most importantly, that IL-17–producing iNKT cells in lymphoid organs of naive mice are contained within the RTE iNKT cell pool. Our results establish an accurate marker of RTE iNKT cells and reveal that continuous thymic output is required for pro-inflammatory IL-17 secretion, a key function of adult iNKT cells.
ALAS2 acts as a modifier gene in patients with congenital erythropoietic porphyria
Authors: To-Figueras Jordi , Ducamp Sarah , Clayton Jerome , Badenas Celia , Delaby Constance , Ged Cecile , Lyoumi Said , Gouya Laurent , de Verneuil Hubert , Beaumont Carole , Ferreira Gloria C. , Deybach Jean-Charles , Herrero Carmen , Puy Herve
Published: June 2011

Mutations in the uroporphyrinogen III synthase (UROS) gene cause congenital erythropoietic porphyria (CEP), an autosomal-recessive inborn error of erythroid heme biosynthesis. Clinical features of CEP include dermatologic and hematologic abnormalities of variable severity. The discovery of a new type of erythroid porphyria, X-linked dominant protoporphyria (XLDPP), which results from increased activity of 5-aminolevulinate synthase 2 (ALAS2), the rate-controlling enzyme of erythroid heme synthesis, led us to hypothesize that the CEP phenotype may be modulated by sequence variations in the ALAS2 gene. We genotyped ALAS2 in 4 unrelated CEP patients exhibiting the same C73R/P248Q UROS genotype. The most severe of the CEP patients, a young girl, proved to be heterozygous for a novel ALAS2 mutation: c.1757 A > T in exon 11. This mutation is predicted to affect the highly conserved and penultimate C-terminal amino acid of ALAS2 (Y586). The rate of 5-aminolevulinate release from Y586F was significantly increased over that of wild-type ALAS2. The contribution of the ALAS2 gain-of-function mutation to the CEP phenotype underscores the importance of modifier genes underlying CEP. We propose that ALAS2 gene mutations should be considered not only as causative of X-linked sideroblastic anemia (XLSA) and XLDPP but may also modulate gene function in other erythropoietic disorders.
The impact of endometrioma and laparoscopic cystectomy on serum anti-Müllerian hormone levels
Authors: Hwu Yuh-Ming , Wu Frank Shao-Ying , Li Sheng-Hsiang , Sun Fang-Ju , Lin Ming-Huei , Lee Robert Kuo-Kuang
Published: June 2011

Background

Serum anti-Müllerian hormone (AMH) had been proposed as a marker of ovarian reserve. The aim of this study was to evaluate the impact of endometrioma and laparoscopic cystectomy on ovarian reserve as measured by serum AMH levels.

Methods

A total of 1,642 patients were recruited in this retrospective analysis. Control group (group 1) included 1,323 infertility patients without endometrioma. Endometrioma group (group 2) included 141 patients with ovarian endometrioma. Previous cystectomy group (group 3) included 147 patients who underwent unilateral or bilateral laparoscopic cystectomy due to ovarian endometrioma more than 6 months before enrollment. Current cystectomy group (group 4) included 31 patients who underwent cystectomy during study period. Serum anti-müllerian hormone (AMH) levels were measured upon enrollment with all patients. For patients in group 4, AMH levels were measured before and 3 months after cystectomy.

Results

Mean AMH level of patients in control group was significantly higher than that of endometrioma group or previous cystectomy group in each age subgroup, while the mean serum AMH level of the endometrioma group was also significantly higher than that of the previous cystectomy group in each age subgroup. The mean AMH level was significantly lower in patients with previous bilateral cystectomy compared to that of patients with unilateral cystectomy. The mean serum AMH level was also significantly lower in patients with bilateral endometrioma compared to that of patients with unilateral endometrioma. In group 4, mean AMH level significantly decreased from 3.95 +/- 0.42 preoperation to 2.01 +/- 0.21 ng/ml at 3-month postoperation.

Conclusions

Both ovarian endometrioma and cystectomy are associated with a significant reduction on ovarian reserve. Bilateral endometrioma exerts a more profound negative impact on ovarian reserve than unilateral endometrioma, regardless of either conservative or surgical intervention.
Endotoxemia in pediatric critical illness - a pilot study
Authors: Dholakia Shamik , Inwald David , Betts Helen , Nadel Simon
Published: June 2011

Introduction

The aim was to investigate the prevalence of endotoxemia in children admitted to pediatric intensive care unit (PICU), and its association with disease severity and outcome.

Methods

We conducted a prospective, observational cohort study of children admitted to PICU at St. Mary's Hospital, London over a 6-month period. One hundred consecutive patients were recruited. Demographic and clinical data were collected. Severity of illness was assessed by the pediatric index of mortality 2 (PIM2) score. The pediatric logistic organ dysfunction (PELOD) score was performed daily for the first 4 days. Patients were categorized according to primary reason for PICU admission. Blood samples were taken within 24 hours of admission and endotoxemia was measured using the endotoxin activity assay (EAA). Patients were stratified according to EAA level (high, EAA > 0.4, low, EAA < 0.4) and categorized as septic, post-surgical, respiratory or other. Data were analyzed using appropriate non-parametric tests.

Results

EAA level was significantly lower in PICU controls versus other PICU admissions (P = 0.01). Fifty-five children had endotoxemia on admission. Forty-one (75%) of these were eventually diagnosed with an infectious cause of admission. Nine children without infection had elevated EAA on admission. An infectious cause of admission was significantly associated with endotoxemia (P < 0.005). Of 15 children with gram-negative infection, only 9 (60%) had endotoxemia on admission. Endotoxemia on admission was not associated with shock or death. However, there was a tendency for increased PELOD score and length of stay in endotoxemic children.

Conclusions

Endotoxemia is common in children admitted to intensive care. Understanding the implications of endotoxemia and potential anti-endotoxin strategies may have the potential to reduce severity of illness and length of PICU stay in critically ill children.
Serial specification of diverse neuroblast identities from a neurogenic placode by Notch and Egfr signaling
Authors: Hwang Helen J. , Rulifson Eric
Published: June 2011

We used the brain insulin-producing cell (IPC) lineage and its identified neuroblast (IPC NB) as a model to understand a novel example of serial specification of NB identities in the Drosophila dorsomedial protocerebral neuroectoderm. The IPC NB was specified from a small, molecularly identified group of cells comprising an invaginated epithelial placode. By progressive delamination of cells, the placode generated a series of NB identities, including the single IPC NB, a number of other canonical Type I NBs, and a single Type II NB that generates large lineages by transient amplification of neural progenitor cells. Loss of Notch function caused all cells of the placode to form as supernumerary IPC NBs, indicating that the placode is initially a fate equivalence group for the IPC NB fate. Loss of Egfr function caused all placodal cells to apoptose, except for the IPC NB, indicating a requirement of Egfr signaling for specification of alternative NB identities. Indeed, both derepressed Egfr activity in yan mutants and ectopic EGF activity produced supernumerary Type II NBs from the placode. Loss of both Notch and Egfr function caused all placode cells to become IPC NBs and survive, indicating that commitment to NB fate nullified the requirement of Egfr activity for placode cell survival. We discuss the surprising parallels between the serial specification of neural fates from this neurogenic placode and the fly retina.
Chronic diseases influence major life changing decisions: a new domain in quality of life research
Authors: Bhatti ZU , Salek MS , Finlay AY
Published: June 2011

The purpose of this review is to identify knowledge about the influence of chronic disease on major life changing decisions (MLCDs). This review was carried out in three stages: identification of key search terms; selection of databases and searching parameters; and evaluation of references. Only two articles matched the main search term ‘major life changing decisions’. No article reviewed or measured the influence of chronic disease on major life changing decisions. However, 76 articles and various sections of seven books were identified that provided insight into this area and these are reviewed in detail. This literature review has brought together previously scattered information on chronic disease impact on important patient life decisions. These include decisions related to having children, marriage and divorce, job and career choice, social life, holidays, travelling and education. Lifestyle decisions viewed by patients as major decisions are also documented. The influence of cancer on life decisions is discussed, as are affected life decisions of other family members. Very little information is available about the long-term impact of chronic disease on patients' lives and methodology to assess long-term impact is incomplete. This review points to a novel dimension to health-related outcome research, the impact of chronic disease on major life changing decisions, and its possible implication for patients' future health.
The Atom Transfer Radical Polymerization of Methyl Methacrylate in Ionic Liquid Media of 1-Methylimidazolium Alkyltes
Authors: Ma Fumin , Li Wen , Lai Guoqiao , Guo Jianlin , Ruan Min , Qin Wanguang
Published: June 2011

Three brønsted acidic ionic liquids, 1-methylimidazolium acetate ([Hmim][CH3COO]), 1-methylimidazolium propionate ([Hmim][CH3CH2COO]) and 1-methylimidazolium butyrate ([Hmim][CH3CH2CH2COO]) were used as reaction medium for atom transfer radical polymerization of methyl methacrylate with ethyl 2-bromoisobutyrate (EBiB)/CuBr as the initiating system. Kinetic studies, chain extension and block copolymerization confirmed the well-controlled manner of these polymerizations in three brønsted acidic ionic liquids. The reactions were fast and the polydispersities of the polymers were quite narrow (1.10<Mw/Mn<1.34). The rate of polymerization in [Hmim][RCOO] decreased with the increasing alkyl chain lengths (n from 1 to 3), indicating a strong dependence of reaction rate on the length of the substituted groups of the anions in ionic liquids.
Reabilitação nomenclatural e taxonômica de Virola bicuhyba (Schott) Warb. (Myristicaceae)
Authors: Rodrigues William A
Published: June 2011

Esta nota trata da reabilitação da Miristicácea Virola bicuhyba (Schott) Warb., designação de lectótipo e sinonimização tanto de Virola oleífera (Schott) A.C. Smith como de Bicuiba oleífera (Schott) de Wilde.
Paving the Way to the Future? Education and Young Europeans' Paths to Work and Independence
Authors: Lundahl Lisbeth
Published: June 2011

This article discusses young people's transitions from school to work at a time when educational systems have become more closely connected to the economy than ever before. The serious situation of high unemployment, unstable employment conditions and poverty among young people and young adults in Europe is highlighted. Using Sweden as an illuminative example, it is argued that the increasing commercialisation and competition within the education sector add to the risks connected to school-to-work transitions. The associated shift to outcome-based curricula and focus on narrow competences and skills rather than a broad education including social, cultural and democratic elements, will provide young people with poor navigation instruments in this process. The need to analyse the long-term impact of the market-oriented culture on young people's self-understanding, orientations and choices is emphasized.
Multiple Embedded Inequalities and Cultural Diversity in Educational Systems: A Theoretical and Empirical Exploration
Authors: Verhoeven Marie
Published: June 2011

This article explores the social construction of cultural diversity in education, with a view to social justice. It examines how educational systems organize ethno-cultural difference and how this process contributes to inequalities. Theoretical resources are drawn from social philosophy as well as from recent developments in social organisation theory. It is argued that ethnic minority pupils face multiple sources of inequalities, of a social (redistributive) and cultural (recognition) nature. The first section argues that these dimensions are made more ‘durable’ through institutionalized patterns of norms and routines. The second section develops this proposal at three empirical levels: the macro-level of educational systems, where national narratives interact with structural educational patterns to shape ethnic inequalities; the intermediate level of local spaces of interdependency, where we scrutinize segregation and institutional discrimination; and, finally in this section, we look at single-school policies of difference as the product of educational ‘niches’. In the last section, the author argues that these multiple embedded inequalities significantly determine pupils' opportunities, identity and ‘capability’ building. New perspectives for multilevel approaches to inequality and qualitative international comparison are suggested.
Producing Dangerous Knowledge: Researching Knowledge Production in Belgium
Authors: Mangez Catherine , Mangez Eric
Published: June 2011

This is an article about the struggle for control of knowledge in a divided society. It starts off by describing Belgium as a consociational democracy — that is, a society organized around integrated pillars of society (Catholic, secular), each of which provides a wide range of services (educational, training, health, health insurance, social care, family planning, leisure) to ‘its' people. This special politico-institutional arrangement inherited from the past, though it has evolved, still has profound implications for the way knowledge circulates (or not) and for the way it is used (or not) and perceived both within pillars and across the policy realm: the co-existence of distinct communities requires a form of discretion. The article then goes on to describe the specific incidents that occurred in the course of a recent research project: the authors' written reports produced unexpected effects. They try to make sense of these events by reflecting on how the specific Belgian context can affect and be affected by the production of knowledge about itself. Finally, they extend their reflection to other contexts by emphasizing the general process behind their observation: the transformative effect of knowledge. Knowledge needs to be understood as an act (of construction) that can affect its very object of analysis.
Standardizing the European Education Policy Space
Authors: Lawn Martin
Published: June 2011

Countries in Europe, through the European Union, are creating, as part of the market and its governance, a new policy space in education. It is being formed through law, regulation, networking and harmonization. The development of standards across the different fields of policy, statistical calculation and commerce underpins and extends the creation of policy spaces. Europeanization processes in education have some subtle and yet powerful features created through measurement and standardization. They may have a technical form but they are knowledge based, policy driven and exclude politics. Europe is at the leading edge of new forms of governance in education.
The Role of Explanations and Prescriptions in the Science of Design: The Case of Educational Research
Authors: Penalva José
Published: June 2011

This article develops the idea that the sciences of the design perspective offer a more adequate solution for bridging the gap between explanations and prescriptions in educational research. This idea is developed over the following steps: first, the scope of the analysis and the problem of the relationship between explanations and prescription are expounded; second, the article examines the relevance of the science of the design perspective and the advantages compared with the traditional view in educational research; third, it considers educational research as a science of design; and finally, predictions and prescriptions are assigned a new role in the field of educational research.
Ownership Concentration, Managerial Ownership and Firm Performance: Evidence from Turkey
Authors: Mandacı Pınar , Gumus Guluzar
Published: June 2011

Ownership Concentration, Managerial Ownership and Firm Performance: Evidence from Turkey

This study examines the effects of ownership concentration and managerial ownership on the profitability and the value of non-financial firms listed on the Istanbul Stock Exchange (ISE) in the context of an emerging market. We measure the firm's performance by Return on Assets (ROA) and Tobin's Q ratios, where the former measures profitability and the latter the value of the firm. In addition, we give detailed information on the main characteristics of the ownership structures of the firms in our sample and find that ownership of Turkish firms is highly concentrated. In addition, the unlisted holding companies have the highest average percentage of shares, which supports the belief that individuals or families establish the holding companies in order to control their listed firms. After controlling for investment intensity, leverage, growth and size, we find that ownership concentration has a significantly positive effect on both firm value and profitability, while managerial ownership has a significantly negative effect on firm value.
Polish botanical and mycological studies of the Antarctic terrestrial and fresh water ecosystems in 1977-2009: An overview
Authors: Köhler Piotr , Olech Maria
Published: June 2011

Polish botanical and mycological studies of the Antarctic terrestrial and fresh water ecosystems in 1977-2009: An overview

This paper recapitulates Polish botanical and mycological research on terrestrial and freshwater Antarctic ecosystems carried out between 1977 and 2009. The main results are briefly summarized. The references encompass nearly 200 papers on floristics, taxonomy, biogeography, ecology, cytology, biochemistry, physiology and genetics of lichens, mosses, fungi, algae and vascular plants inhabiting soils, rocks and inland waters in the Antarctic.
On Two Nanocomposite Models: Differences, Similarities and Interpretational Possibilities Regarding Tsagaropoulos' Model and Tanaka's Model
Authors: Danikas Michael
Published: June 2011

On Two Nanocomposite Models: Differences, Similarities and Interpretational Possibilities Regarding Tsagaropoulos' Model and Tanaka's Model

Nanocomposites consist nowadays a major field of research. This paper investigates the interpretational possibilities of two models developed, namely, Tsagaropoulos' model and Tanaka's model. The two models were developed separately and independently from each other. They both consider various layers around the nanoparticles. They both consider well bonded and more loosen layers. Similarities and differences between the two models are pointed out as well as possibilities for further research in order to shed light on some aspects of electrical phenomena in these materials.
Real Time Motion Data Preprocessing
Authors: Benický Peter , Jurišica Ladislav
Published: June 2011

Real Time Motion Data Preprocessing

There is a lot of redundant data for image processing in an image, in motion picture as well. The more data for image processing we have, the more time is needed for preprocessing it. That is why we need to work with important data only. In order to identify or classify motion, data processing in real time is needed.
Vector Control with Optimal Torque of a Salient-Pole Double Star Synchronous Machine Supplied by Three-Level Inverters
Authors: Nezli Lazhari , Mahmoudi Mohand
Published: June 2011

Vector Control with Optimal Torque of a Salient-Pole Double Star Synchronous Machine Supplied by Three-Level Inverters

This work relates to the study of the vector control of the salient-pole double star synchronous machine drive (DSSM) fed by three-level inverters which are controlled by PWM hysteresis strategy. Indeed, it is a question of carrying out a decoupling between rotor flux and electromagnetic torque, by introducing a vector control with an optimal torque working and by imposing constant flux regime. Furthermore, to ensure a decoupled dynamic behavior of the machine as in the case of a DC machine, a regulation of the rotor current has been introduced through a chopper operating at 5 kHz chopping frequency, feeding the excitation circuit. The obtained results are very satisfactory and reveal the effectiveness of the proposed approach.