bluuebunny/crossref_metadata_2025 huggingface dataset:
Benign hereditary chorea related to NKX2.1: expansion of the genotypic and phenotypic spectrum
Authors: Peall Kathryn J , Lumsden Daniel , Kneen Rachel , Madhu Rajesh , Peake Deirdre , Gibbon Frances , Lewis Hilary , Hedderly Tammy , Meyer Esther , Robb Stephanie A , Lynch Bryan , King Mary D , Lin Jean‐Pierre , Morris Huw R , Jungbluth Heinz , Kurian Manju A
Published: October 2013

Aim

Benign hereditary chorea is a dominantly inherited, childhood‐onset hyperkinetic movement disorder characterized by non‐progressive chorea and variable degrees of thyroid and respiratory involvement. Loss‐of‐function mutations in NKX2.1, a gene vital to the normal development and function of the brain, lungs, and thyroid, have been identified in a number of individuals.

Method

Clinical data from individuals with benign hereditary chorea identified through paediatric neurology services were collected in a standardized format. The NKX2.1 gene was analysed by Sanger sequencing, multiplex ligation‐dependent probe amplification, and microarray analysis.

Results

Six of our cohort were female and four male, median age at assessment was 8 years 6 months (range 1y 6mo–18y). We identified 10 probands with NKX2.1 mutations; nine of these mutations are novel (including two whole‐gene deletions) and one has been previously reported. Of the 10 individuals, eight presented with muscle hypotonia and four had evidence of hypothyroidism or respiratory involvement. Only three out of the 10 individuals had the full triad of ‘brain–lung–thyroid syndrome’ symptoms. Additional clinical characteristics occurring in individual participants included growth hormone deficiency, pes cavus, kyphosis, duplex kidney, and obsessive–compulsive disorder.

Interpretation

Our data suggest that the neurological phenotype is prominent in this condition and that many patients with benign hereditary chorea do not have the classic triad of brain–lung–thyroid syndrome. The extended phenotype may include obsessive–compulsive disorder and skeletal abnormalities.
Ethnicity and long‐term vascular outcomes in Type 2 diabetes: a prospective observational study (UKPDS 83)
Authors: Davis T. M. E. , Coleman R. L. , Holman R. R. ,   UKPDS Group
Published: October 2013

Aims

Evidence of ethnic differences in vascular complications of diabetes has been inconsistent. The aim of this study was to examine the relationship between ethnicity and long‐term outcome in a large sample of individuals with newly diagnosed Type 2 diabetes.

Methods

In a prospective observational study of 4273 UK Prospective Diabetes Study participants followed for a median of 18 years, 3543 (83%) were White Caucasian, 312 (7%) Afro‐Caribbean and 418 (10%) Asian Indian. Relative risks for predefined outcomes were assessed comparing Afro‐Caribbean and Asian Indian with White Caucasian using accelerated failure time models, with adjustment for cardiovascular risk factors and other potentially confounding variables.

Results

During follow‐up, 2468 (58%) participants had any diabetes‐related end point, 1037 (24%) a myocardial infarction and 401 (9%) a stroke, and 1782 (42%) died. Asian Indian were at greater risk (relative risk, 95% confidence interval) for any diabetes‐related end point (1.18, 1.07–1.29), but at lower risk of all‐cause mortality (0.89, 0.80–0.97) and peripheral vascular disease (0.43, 0.23–0.82), vs. White Caucasian. Afro‐Caribbean participants were at lower risk for all‐cause mortality (0.84, 0.76–0.93), diabetes‐related death (0.75, 0.64–0.88), myocardial infarction (0.55, 0.43–0.71) and peripheral vascular disease (0.55, 0.33–0.93) vs. White Caucasian. No ethnicity‐related associations were found for stroke or microangiopathy.

Conclusions

Asian Indian ethnicity is associated with the greatest burden of disease, but not with an increased risk of major vascular complications or death. Afro‐Caribbean ethnicity is associated with reduced risk of all‐cause and diabetes‐related death, myocardial infarction and peripheral vascular disease, suggesting an ethnicity‐specific protective mechanism.
Height and the risk of gestational diabetes: variations by race/ethnicity
Authors: Brite J. , Shiroma E. J. , Bowers K. , Yeung E. , Laughon S. K. , Grewal J. G. , Zhang C.
Published: October 2013

Aims

Gestational diabetes is a common pregnancy complication affecting races/ethnicities disproportionally. Adult height, an indicator of both genetic and early‐life factors, is inconsistently associated with gestational diabetes risk. We examined the association and whether it varies by races in a nationally representative US cohort.

Methods

Analyses were conducted among 135 861 pregnancies in the Consortium on Safe Labor, 5567 of which were diagnosed with gestational diabetes based on medical records review. Generalized estimating equations were used to estimate odds ratios (95% confidence intervals) of gestational diabetes, controlling for other risk factors including body weight. Additionally, a meta‐analysis of 15 761 pregnancies with gestational diabetes and 205 828 without gestational diabetes was conducted to estimate the pooled mean difference in height between those with gestational diabetes and control subjects.

Results

Height was inversely associated with gestational diabetes risk across races/ethnicities, with the strongest association among Asians (P for interaction < 0.01). Comparing extreme quartiles (> 168 vs. < 157 cm), adjusted odds ratios (95% confidence intervals) were 0.18 (0.09–0.36) for Asians/Pacific Islanders, 0.33 (0.29–0.38) for non‐Hispanic white women, 0.39 (0.31–0.51) for Hispanics and 0.59 (0.47–0.75) for non‐Hispanic black women. Meta‐analysis found women with gestational diabetes to be significantly shorter than others.

Conclusions

Taller women are at lower risk of developing gestational diabetes, with the magnitude of association varying significantly across races/ethnicities.
Contrasting weight changes with LY2605541, a novel long‐acting insulin, and insulin glargine despite similar improved glycaemic control in T1DM and T2DM
Authors: Jacober S. J. , Rosenstock J. , Bergenstal R. M. , Prince M. J. , Qu Y. , Beals J. M.
Published: October 2013

Aims

The basal insulin analogue LY2605541, a PEGylated insulin lispro with prolonged duration of action, was previously shown to be associated with modest weight loss in Phase 2, randomized, open‐label trials in type 2 (N = 288) and type 1 (N = 137) diabetes mellitus (T2DM and T1DM), compared with modest weight gain with insulin glargine. Exploratory analyses were conducted to further characterize these findings.

Methods

Pearson correlations between change in body weight and other variables were calculated. Continuous variables were analysed using a mixed linear model with repeated measurements. Proportions of subjects with weight loss were analysed using Fisher's exact test for T2DM and Nagelkerke's method for T1DM.

Results

Weight loss was more common in LY2605541‐treated patients than in patients treated with insulin glargine (T2DM: 56.9 vs. 40.2%, p = 0.011; T1DM: 66.1 vs. 40.3%, p < 0.001). More LY2605541‐treated patients experienced ≥5% weight loss compared to patients treated with glargine (T2DM: 4.8 vs. 0%, p = 0.033; T1DM: 11.9 vs. 0.8%, p < 0.001). In both the T1DM and T2DM studies, weight change did not correlate with baseline body mass index (BMI), or change in HDL‐cholesterol in either treatment group. No consistent correlations were found across both studies between weight change and any of the variables assessed; however, weight change was significantly correlated with hypoglycaemia rate in glargine‐treated T2DM patients.

Conclusion

In two Phase 2 trials, improved glycaemic control with long‐acting basal insulin analogue LY2605541 is associated with weight loss in previously insulin‐treated patients. This weight change is independent of baseline BMI or hypoglycaemia.
Re‐starting a neural race: anti‐saccade correction
Authors: Noorani Imran , Carpenter R. H. S.
Published: October 2013

In the anti‐saccade task, a subject must make a saccadic eye movement in the opposite direction from a suddenly‐presented visual target. This sets up a conflict between the natural tendency to make a pro‐saccade towards the target and the required anti‐saccade. Consequently there is a tendency to make errors, usually corrected by a second movement in the correct anti‐saccade direction. In a previous paper, we showed that a very simple model, with racing LATER (Linear Approach to Threshold at Ergodic Rate) units for the pro‐ and anti‐directions, and a stop unit that inhibits the impending error response, could account precisely for the detailed distributions of reaction times both for correct and error responses. However, the occurrence and timing of these final corrections have not been studied. We propose a novel mechanism: the decision race re‐starts after an error. Here we describe measurements of all the responses in an anti‐saccade task, including corrections, in a group of human volunteers, and show that the timing of the corrections themselves can be predicted by the same model with one additional assumption, that initiation of an incorrect pro‐saccade also resets and initiates a corrective anti‐saccade. No extra parameters are needed to predict this complex aspect of behaviour, adding weight to our proposal that we correct our mistakes by re‐starting a neural decision race. The concept of re‐starting a decision race is potentially exciting because it implies that neural processing of one decision can influence the next, and may be a fruitful way of understanding the complex behaviour underlying sequential decisions.
The potential for bioenergy crops to contribute to meeting GB heat and electricity demands
Authors: Wang Shifeng , Hastings Astley , Wang Sicong , Sunnenberg Gilla , Tallis Matthew J. , Casella Eric , Taylor Simon , Alexander Peter , Cisowska Iwona , Lovett Andrew , Taylor Gail , Firth Steven , Moran Dominic , Morison James , Smith Pete
Published: October 2013

The paper presents a model system, which consists of a partial equilibrium model and process‐based terrestrial biogeochemistry models, to determine the optimal distributions of both Miscanthus (Miscanthus × giganteus) and short rotation coppice willow (SRC) (Salix. viminalis L. x S. viminalis var Joruun) in Great Britain (GB), as well as their potential contribution to meet heat and electricity demand in GB. Results show that the potential contribution of Miscanthus and SRC to heat and electricity demand is significant. Without considering farm‐scale economic constraints, Miscanthus and SRC could generate, in an economically competitive way compared with other energy generation costs, 224 800 GWh yr−1 heat and 112 500 GWh yr−1 electricity, with 8 Mha of available land under Miscanthus and SRC, accounting for 66% of total heat demand and 62% of total electricity demand respectively. Given the pattern of heat and electricity demand, and the relative yields of Miscanthus and SRC in different parts of GB, Miscanthus is mainly favoured in the Midlands and areas in the South of GB, whereas SRC is favoured in Scotland, the Midlands and areas in the South of GB.
Helicobacter pylori Antigen HP0986 (TieA) Interacts with Cultured Gastric Epithelial Cells and Induces IL8 Secretion via NF‐κB Mediated Pathway
Authors: Devi Savita , Ansari Suhail A. , Vadivelu Jamuna , Mégraud Francis , Tenguria Shivendra , Ahmed Niyaz
Published: October 2013

Background

The envisaged roles and partly understood functional properties of Helicobacter pylori protein HP0986 are significant in the context of proinflammatory and or proapoptotic activities, the two important facilitators of pathogen survival and persistence. In addition, sequence analysis of this gene predicts a restriction endonuclease function which remained unknown thus far. To evaluate the role of HP0986 in gastric inflammation, we studied its expression profile using a large number of clinical isolates but a limited number of biopsies and patient sera. Also, we studied antigenic role of HP0986 in altering cytokine responses of human gastric epithelial (AGS) cells including its interaction with and localization within the AGS cells.

Materials and Methods

For in vitro expression study of HP0986, 110 H. pylori clinical isolates were cultured from patients with functional dyspepsia. For expression analysis by qRT PCR of HP0986, 10 gastric biopsy specimens were studied. HP0986 was also used to detect antibodies in patient sera. AGS cells were incubated with recombinant HP0986 to determine cytokine response and NF‐κB activation. Transient transfection with HP0986 cloned in pEGFPN1 was used to study its subcellular localization or homing in AGS cells.

Results

Out of 110 cultured H. pylori strains, 34 (31%) were positive for HP0986 and this observation was correlated with in vitro expression profiles. HP0986 mRNA was detected in 7 of the 10 biopsy specimens. Further, HP0986 induced IL‐8 secretion in gastric epithelial cells in a dose and time‐dependent manner via NF‐κB pathway. Serum antibodies against HP0986 were positively associated with H. pylori positive patients. Transient transfection of AGS cells revealed both cytoplasmic and nuclear localization of HP0986.

Conclusion

HP0986 was moderately prevalent in clinical isolates and its expression profile in cultures and gastric biopsies points to its being naturally expressed. Collective observations including the induction of IL‐8 via TNFR1 and NF‐κB, subcellular localization, and seropositivity data point to a significant role of HP0986 in gastroduodenal inflammation. We propose to name the HP0986 gene/protein as ‘TNFR1 interacting endonuclease A (TieA or tieA)’.
HIV nucleoside reverse transcriptase inhibitors efavirenz and tenofovir change the growth and differentiation of primary gingival epithelium
Authors: Mitchell D , Israr M , Alam S , Dinello D , Kishel J , Jia R , Meyers C
Published: October 2013

Objectives

An increasing number of HIV‐infected patients are combating HIV infection through the use of antiretroviral drugs, including reverse transcriptase inhibitors. Oral complications associated with these drugs are becoming a mounting cause for concern. In our previous studies, both protease inhibitors and reverse transcriptase inhibitors have been shown to change the proliferation and differentiation state of oral tissues. This study examined the effect of a nonnucleoside and a nucleoside reverse transcriptase inhibitor on the growth and differentiation of gingival epithelium.

Methods

Organotypic (raft) cultures of gingival keratinocytes were treated with a range of efavirenz and tenofovir concentrations. Raft cultures were immunohistochemically analysed to determine the effect of these drugs on the expression of key differentiation and proliferation markers, including cytokeratins and proliferating cell nuclear antigen (PCNA).

Results

These drugs dramatically changed the proliferation and differentiation state of gingival tissues when they were present throughout the growth period of the raft tissue as well as when drugs were added to established tissue on day 8. Treatment with the drugs increased the expression of cytokeratin 10 and PCNA and, conversely, decreased expression of cytokeratin 5, involucrin and cytokeratin 6. Gingival tissue exhibited increased proliferation in the suprabasal layers, increased fragility, and an inability to heal itself.

Conclusions

Our results suggest that efavirenz and tenofovir treatments, even when applied in low concentrations for short periods of time, deregulated the cell cycle/proliferation and differentiation pathways, resulting in abnormal epithelial repair and proliferation. Our system could be developed as a potential model for studying the effects of HIV and highly active antiretroviral therapy (HAART) in vitro.
Pathobiological properties of the ubiquitin ligase Nedd4L in melanoma
Authors: Kito Yusuke , Bai Juncheng , Goto Naoe , Okubo Hiroshi , Adachi Yoshihiro , Nagayama Tomoko , Takeuchi Tamotsu
Published: October 2013

A recent global gene expression profiling study unexpectedly showed that activated oncogenic NRAS may recruit neural precursor cell expressed, developmentally downregulated 4L (Nedd4L; a human homologue of Nedd4‐2) in cultured melanoma cells. However, whether Nedd4L was expressed in melanoma tissues or participated in melanoma carcinogenesis remains to be clarified. Here, we investigated the expression status of Nedd4L in human melanocytes, benign nevi and melanoma tissue specimens and subsequently attempted to determine the role of Nedd4L in melanoma cell growth. Immunohistochemical staining revealed that Nedd4L was not present in any non‐tumorous melanocytes or in 18 benign nevi tissues, but it was detected in 34 of 79 cutaneous melanomas and 9 of 32 nodal metastatic melanomas. Downregulation of Nedd4L significantly reduced the growth of cultured G361 melanoma cells in vitro. Moreover, exogenous Nedd4L expression significantly promoted the growth of A2058 melanoma cells in vivo in a xenograft assay. The present findings indicate that Nedd4L expression may be increased to facilitate tumour growth in many melanomas.
Is Firm‐specific Return Variation a Measure of Information Efficiency?
Authors: Bae Kee‐Hong , Kim Jin‐Mo , Ni Yang
Published: October 2013

The issue of whether firm‐specific return variation measures the private information reflected in stock returns or trading noise is controversial. Using a firm's geographic proximity to its investors as a proxy for a firm's private information, we investigate the relation between firm‐specific return variation and price informativeness. We find that firms located in metropolitan areas experience higher firm‐specific return variation and that holdings and trading by local institutional investors positively affect firm‐specific return variation. These findings suggest that higher firm‐specific return variation is indicative of more informative stock prices.
Comparing the hydrosurgery system to conventional debridement techniques for the treatment of delayed healing wounds: a prospective, randomised clinical trial to investigate clinical efficacy and cost‐effectiveness
Authors: Liu Jing , Ko Jason H , Secretov Erwin , Huang Eric , Chukwu Christiana , West Julie , Piserchia Katherine , Galiano Robert D
Published: October 2013

In these uncertain times of high health care costs, clinicians are looking for cost‐effective devices to employ in their everyday practices. In an effort to promote cost‐effective and proper wound repair, the hydrosurgical device allows accurate debridement of only unwanted tissue while precisely conserving viable structures for eventual repair. This prospective, randomised study compared procedures using the hydrosurgery system (VERSAJET™) with conventional debridement in order to assess clinical efficacy and cost‐effectiveness when treating subjects with chronic wounds. A total of 40 subjects were recruited. There was no difference in time to achieve stable wound closure between the treatment groups (P = 0·77). There were no significant differences between the two groups in terms of cost of the first operative procedure (P = 0·28), cost of surgical procedures during the study (P = 0·51), cost of study treatment (P = 0·29) or cost to achieve stable wound closure (P = 0·85). There were no differences in quantitative bacterial counts after debridement with either methods (P = 0·376). However, the time taken for the first excision procedure was significantly faster using the hydrosurgery system (VERSAJET) when compared with conventional debridement (P < 0·001). The total excision time for all procedures was significantly less for the Hydrosurgery group than for the conventional group (P = 0·005). Also, the Hydrosurgery group demonstrated significantly less intraoperative blood loss than conventional group for all procedures (P = 0·003). In this study, although there were no differences in time to stable wound closure or bacterial reduction between the two groups, the hydrosurgery system (VERSAJET) did offer advantages in terms of operative times and intraoperative blood loss and was cost‐neutral, despite the handpiece cost.
Board Effectiveness and Short Termism
Authors: Gonzalez Angelica , André Paul
Published: October 2013

We examine whether more effective boards in terms of size, experience, shareholding and independence, as discussed in the 2010 UK Corporate Governance Code, limit excessive short‐term risk taking or short‐termism. We use a state‐of‐the‐art asset pricing model that enables the disentangling of short‐term risk (related to short‐term returns) and long‐term risk (related to long‐term returns), and use the former as a proxy for short‐termism, where the short‐term component not only represents the time horizon for which we are interested but also the risk that is not related to fundamentals. We examine 916 firms in the UK over a possible horizon of 18 years, January 1992–December 2010, and find that more effective boards are associated with lower levels of short‐term risk and this result is robust to various types of short‐term risk (overall, downside) and specifications.
Epithelial Sodium Channel Inhibition by Amiloride on Blood Pressure and Cardiovascular Disease Risk in Young Prehypertensives
Authors: Bhagatwala Jigar , Harris Ryan A. , Parikh Samip J. , Zhu Haidong , Huang Ying , Kotak Ishita , Seigler Nichole , Pierce Gary L. , Egan Brent M. , Dong Yanbin
Published: October 2013

Overactivity of the epithelial sodium channel (ENaC) is considered to be one mechanism underlying obesity‐related blood pressure (BP) elevation. In an open‐labeled, nonplacebo‐controlled clinical trial (Clinicaltrials.gov: NCT‐01308983), the authors aimed to comprehensively evaluate the effects of amiloride monotherapy, an ENaC blocker, on BP and cardiovascular risk in young adults with prehypertension (n=17). The mean body mass index of participants was 28.45±1.30 kg/m2. Following 10 mg daily amiloride for 4 weeks, peripheral systolic BP (SBP), central SBP, and carotid‐radial pulse wave velocity were significantly reduced by −7.06±2.25 mm Hg, −7.68±2.56 mm Hg, and −0.72±0.33 m/s, respectively, whereas flow‐mediated dilation was significantly increased by 2.2±0.9%. Following amiloride monotherapy for 4 weeks, a significant increase in serum aldosterone was observed (5.85±2.45 ng/dL). ENaC inhibition by amiloride may be used as an early intervention to halt the progression to full hypertension and cardiovascular disease in young adults with prehypertension.
T lymphocytes export proteasomes by way of microparticles: a possible mechanism for generation of extracellular proteasomes
Authors: Bochmann Isabel , Ebstein Frédéric , Lehmann Andrea , Wohlschlaeger Jeremias , Sixt Stephan Urs , Kloetzel Peter‐Michael , Dahlmann Burkhardt
Published: October 2013

The 20S proteasome is almost exclusively localized within cells. High levels of extracellular proteasomes are also found circulating in the blood plasma of patients suffering from a variety of inflammatory, autoimmune and neoplastic diseases. However, the origin of these proteasomes remained enigmatic. Since the proteome of microparticles, small membrane enclosed vesicles released from cells, was shown to contain proteasomal subunits, we studied whether intact proteasomes are actively released into the extracellular space. Using human primary T lymphocytes stimulated with CaCl2 and the calcium ionophore A23187 to induce membrane blebbing we demonstrate that microparticles contain proteolytically active 20S proteasomes as well as the proteasome activator PA28 and subunits of the 19S proteasome regulator. Furthermore, our experiments reveal that incubation of in vitro generated T lymphocyte‐microparticles with sphingomyelinase results in the hydrolysis of the microparticle membranes and subsequent release of proteasomes from the vesicles. Thus, we here show for the first time that functional proteasomes can be exported from activated immune cells by way of microparticles, the dissolution of which may finally lead to the generation of extracellular proteasomes.
Roles of microRNAs in prenatal chondrogenesis, postnatal chondrogenesis and cartilage‐related diseases
Authors: Shang Jin , Liu Huan , Zhou Yue
Published: October 2013

Cartilage has limited repair and regeneration capacity, thus damage of cartilage often results in its dysfunction and even chronic diseases like osteoarthritis (OA). Chondrogenesis induced by tissue‐engineering methods is essential to treating cartilage‐related diseases. MicroRNAs (miRNAs) are a class of small non‐coding single‐stranded RNAs which exert their biological effects by binding to the target messenger RNAs (mRNAs), resulting in decay or translation suppression of target mRNAs. There are emerging evidence indicating that miRNAs may play important roles in regulating both prenatal and postnatal chondrogenesis. During embryonic skeletal development, prenatal chondrogenesis is thought to be a precondition for formation of cartilage in developing limbs. Plenty of studies on different types of stem cells have undoubtedly proven their capacity of differentiating into chondrocytes. MiRNAs are found to comprehensively modulate these processes by establishing an interaction network with target genes, transcription factors and cytokines et al. In addition, translational application of miRNA technology has also been explored. In this review, we focus on the up‐dated progress on regulatory mechanisms of miRNAs in prenatal and postnatal chondrogenesis. In addition, several miRNA target genes and roles of miRNAs in cartilage‐related diseases are also discussed. This will contribute to studies of chondrogenesis mechanisms and development of new treating methods.
Genetic markers associated with progression in early mycosis fungoides
Authors: Johnson V.E. , Vonderheid E.C. , Hess A.D. , Eischen C.M. , McGirt L.Y.
Published: October 2013

Background

Mycosis fungoides (MF) is a rare, but potentially devastating malignancy. It classically presents with cutaneous patches and plaques and can progress to tumours on the skin with lymph node, blood and visceral involvement. While most patients with MF have a relatively benign disease course, a subset of patients will develop progressive disease that is often fatal.

Objective

The aim of this study was to identify genetic markers in early MF limited to the skin (stages IA‐IIA) that distinguish those patients who will have progressive disease from those who will not, so that early appropriate treatment may be instituted.

Methods

The study includes 18 patients who were diagnosed with early stage MF at the time of biopsy and had follow‐up to determine which patients developed progressive disease. RNA was extracted from skin biopsy specimens and analysed for expression of CD3, FOXP3, IFNγ, Interleukin (IL)‐4, IL‐13, KIR3DL2, MICB, PLS3 and STAT4 by quantitative real‐time polymerase chain reaction.

Results/Conclusions

Reduced expression of FOXP3 and STAT4 and increased expression of IL‐4 relative to CD3 expression levels were significantly associated with MF progression. Further studies will be needed to fully assess the usefulness of these genetic markers to predict disease progression and guide treatment options in patients diagnosed with early MF.
Clinical features of patients with Guillain‐Barré syndrome at seven hospitals on the East Coast of Australia
Authors: Blum Stefan , Reddel Stephen , Spies Judy , McCombe Pamela
Published: October 2013

To document the clinical features of Guillain‐Barré syndrome (GBS) in Australia, we performed a retrospective analysis of all patients admitted to several hospitals along the East Coast of Australia from 2000 to 2012. Using hospital records, we reviewed all patients with a diagnosis of GBS admitted to seven hospitals. From these, we report information of subjects who fulfilled standard diagnostic criteria. We excluded patients where inadequate information was available or who were under the age of 18. We report the features of 335 patients, in 228 of whom neurophysiological data were available. There were 168 cases of acute inflammatory demyelinating polyneuropathy (AIDP), 17 of acute motor axonal neuropathy (AMAN), 4 of acute motor and sensory axonal neuropathy (AMSAN), and 35 of Miller‐Fisher syndrome (MFS). The median age at onset was 52.5 years (18–89 years) with a male : female ratio of 1.61 : 1. Upper respiratory tract infections were the most frequently identified trigger (151 subjects, 44.5%). Most patients were severely affected, with 42.7% of subjects bedbound, and an additional 24% requiring ventilatory support. GBS affects adults of all ages and usually follows a severe clinical course. In contrast to other autoimmune diseases, males are more frequently affected. A wide variety of triggering factors leads to a relatively stereotypical clinical syndrome. The most common variant of GBS in Australia is AIDP. This study shows that the clinical features of GBS in Australia are similar to that previously reported and confirms the male predominance, increased incidence with age, and frequent evidence of peripheral nerve demyelination as features of GBS.
A controlled trial of intravenous immunoglobulin in multifocal motor neuropathy
Authors: Hahn Angelika F. , Beydoun Said R. , Lawson Victoria ,   for The IVIG in MMN Study Team, Oh MyungShin , Empson Victoria G. , Leibl Heinz , Ngo Leock Y. , Gelmont David , Koski Carol L.
Published: October 2013

Intravenous immunoglobulin (IVIG) has become the standard treatment for multifocal motor neuropathy (MMN) based on limited data. To critically assess the efficacy, safety, and tolerability of 10% liquid IVIG (IVIG), 44 adults with MMN were randomized 1 : 1 to either double‐blind treatment of IVIG followed by placebo for 12 weeks each or the reverse. Open‐label IVIG was administered for 12 weeks at the beginning and end of the study for clinical stabilization, and between double‐blinded periods to prevent a carry‐over effect. To avoid potential worsening, switching to open‐label IVIG was permitted if deterioration occurred during blinded treatment. Mean maximal grip strength of the more affected hand declined 31.38% during placebo and increased 3.75% during IVIG (p = 0.005). In 35.7% of participants, Guy's Neurological Disability scores for upper limbs worsened during placebo and not during IVIG, whereas the converse was true in 11.9% (p = 0.021). Sixty‐nine percent (69.0%) switched prematurely from placebo to open‐label IVIG and 2.4% switched from blinded to open‐label IVIG (p < 0.001). One serious adverse reaction (pulmonary embolism) and 100 non‐serious reactions (69 mild, 20 moderate, and 11 severe) to IVIG occurred. IVIG was effective in improving disability and muscle strength, and was safe and well tolerated in adults with MMN.
Does learning style influence academic performance in different forms of assessment?
Authors: Wilkinson Tracey , Boohan Mairead , Stevenson Michael
Published: October 2013

Educational research on learning styles has been conducted for some time, initially within the field of psychology. Recent research has widened to include more diverse disciplines, with greater emphasis on application. Although there are numerous instruments available to measure several different dimensions of learning style, it is generally accepted that styles differ, although the qualities of more than one style may be inherent in any one learner. But do these learning styles have a direct effect on student performance in examinations, specifically in different forms of assessment? For this study, hypotheses were formulated suggesting that academic performance is influenced by learning style. Using the Honey and Mumford Learning Style Questionnaire, learning styles of a cohort of first year medical and dental students at Queen's University Belfast were assessed. Pearson correlation was performed between the score for each of the four learning styles and the student examination results in a variety of subject areas (including anatomy) and in different types of assessments – single best answer, short answer questions and Objective Structured Clinical Examinations. In most of the analyses, there was no correlation between learning style and result and in the few cases where the correlations were statistically significant, they generally appeared to be weak. It seems therefore from this study that although the learning styles of students vary, they have little effect on academic performance, including in specific forms of assessment.
Exploring young people's dignity: A qualitative approach
Authors: Narayan Kruthika , Hooker Claire , Jarrett Carmen , Bennett David
Published: October 2013

Aim

Human dignity as an important consideration in health care has been primarily investigated from an adult perspective. This paper explores young people's perceptions of dignity and how it impacts on their health‐care experience.

Method

A qualitative pilot study was undertaken at the Children's Hospital, Westmead in from 2010 to 2011. Semistructured interviews were conducted with five inpatients, and data were analysed using a grounded theory approach.

Results

The adolescents interviewed perceived dignity as a way of protecting their personhood. Privacy and maintaining integrity were the means by which dignity could be preserved in a health‐care setting.

Conclusions

The study found that young people had unique perceptions of privacy and personhood with regards to dignity. Of the concepts of dignity in the existing literature, the dignity of identity was most applicable to adolescents' conceptions. This understanding of young people's views of dignity could prevent dignity violations in health care and beneficially impact their development.
Reassessing the taxonomic status of two enigmatic Desmos species (Annonaceae): Morphological and molecular phylogenetic support for a new genus, Wangia
Authors: GUO Xing , WANG Jing , XUE Bine , THOMAS Daniel C. , SU Yvonne C. F. , TAN Yun‐Hong , SAUNDERS Richard M. K.
Published: October 2013

The systematic position of two enigmatic Annonaceae species from China, Desmos saccopetaloides (W. T. Wang) P. T. Li and Desmos yunnanensis (Hu) P. T. Li, has been controversial, with both species having been transferred between several different genera within subfamilies Annonoideae and Malmeoideae. Phylogenetic analyses of eight chloroplast regions (matK, ndhF, ndhF‐rpl32, psbA‐trnH, rbcL, rpl32‐trnL, trnL‐F, and ycf1; ca. 9.2 kb, 66 taxa) unambiguously placed D. saccopetaloides in a subclade of tribe Miliuseae, nested among the genera Monoon, Neo‐uvaria, Phaeanthus, Sageraea, and Stelechocarpus. This relationship was also supported by endosperm rumination patterns in the seed; other morphological characters furthermore indicated that D. saccopetaloides has closer affinities with Monoon, Neo‐uvaria, and Phaeanthus rather than either Sageraea or Stelechocarpus. Desmos saccopetaloides is distinguished from these genera by its leaf‐opposed inflorescences, sepaloid outer petals, saccate inner petals with basal glandular tissue, moniliform monocarps with uniseriate seeds, and rectangular disulculate pollen with two “cryptoapertures.” On the basis of the combined molecular phylogenetic and morphological data, we propose a new genus, Wangia, to accommodate D. saccopetaloides. The molecular phylogenetic analyses furthermore indicated that D. yunnanensis belongs to the genus Dasymaschalon: examination of the type collections revealed that it is conspecific with Dasymaschalon obtusipetalum, although the combination Dasymaschalon yunnanense has nomenclatural priority.
Phylogeography of the widespread plant Ailanthus altissima (Simaroubaceae) in China indicated by three chloroplast DNA regions
Authors: LIAO Yi‐Ying , GUO You‐Hao , CHEN Jin‐Ming , WANG Qing‐Feng
Published: October 2013

Ailanthus altissima (Mill.) Swingle, a temperate tree species, has a wide distribution in China. To infer its refugia and patterns of migration during past climatic changes in China, genetic variations among different populations were studied. Gene sequences of three chloroplast DNA spacer regions, psbA‐trnH, trnL‐trnF, and trnD‐trnT, were obtained from 440 individuals of 44 populations. The distribution of haplotype and the relationships among them were investigated by haplotype network. In addition, the genetic diversity of the sampled regions was inferred, and the biogeographic history was also reconstructed. Twelve haplotypes were identified, among which, five were unique. The phylogenetic analysis and geographical distribution of haplotypes indicate that multiple glacial refugia existed in mainland China during the Quaternary oscillations. Due to the combined effects of contiguous range expansion and allopatric fragmentation, significant genetic structure was not found in this study. Based on biogeographic and demographic analysis, three main dispersal routes were identified for the major haplotypes, whereas others were more likely localized demographic expansion.
Sectoral Money Demand Behaviour and the Welfare Cost of Inflation in the UK
Authors: Ashworth John , Barlow David , Evans Lynne
Published: October 2013

In this paper, we estimate separate UK money demand functions for the household and corporate sectors; and calculate estimates of the welfare cost of inflation. We find that the household sector bears most of the welfare burden which is in sharp contrast to previous (US) evidence. Also, we find aggregate welfare cost estimates that are much smaller than previous (largely US) estimates—sufficiently smaller as to challenge the oft‐quoted Lucas finding that shoe leather costs are by no means trivial. For the UK, we find welfare costs no greater than one tenth of a per cent of real income.
Which Words are Hard for Autistic Children to Learn?
Authors: SCHAFER GRAHAM , WILLIAMS TIM I. , SMITH PHILIP T.
Published: October 2013

Motivated by accounts of concept use in autistic spectrum disorder (ASD) and a computational model of weak central coherence (O'Loughlin and Thagard, 2000) we examined comprehension and production vocabulary in typically‐developing children and those with ASD and Down syndrome (DS). Controlling for frequency, familiarity, length and imageability, Colorado Meaningfulness played a hitherto unremarked role in the vocabularies of children with ASD. High Colorado Meaningful words were underrepresented in the comprehension vocabularies of 2‐ to 12‐year‐olds with ASD. The Colorado Meaningfulness of a word is a measure of how many words can be associated with it. Situations in which high Colorado Meaningfulness words are encountered are typically highly variable, and words with High Colorado Meaningfulness often involve extensive use of context. Our data suggest that the number of contexts in which a particular word can appear has a role in determining vocabulary in ASD. This suggestion is consistent with the weak central coherence theory of autism.
Structural basis for arabinoxylo‐oligosaccharide capture by the probiotic Bifidobacterium animalis subsp. lactis Bl‐04
Authors: Ejby Morten , Fredslund Folmer , Vujicic‐Zagar Andreja , Svensson Birte , Slotboom Dirk Jan , Abou Hachem Maher
Published: October 2013

Glycan utilization plays a key role in modulating the composition of the gut microbiota, but molecular insight into oligosaccharide uptake by this microbial community is lacking. Arabinoxylo‐oligosaccharides (AXOS) are abundant in the diet, and are selectively fermented by probiotic bifidobacteria in the colon. Here we show how selectivity for AXOS uptake is established by the probiotic strain Bifidobacterium animalis subsp. lactis Bl‐04. The binding protein BlAXBP, which is associated with an ATP‐binding cassette (ABC) transporter that mediates the uptake of AXOS, displays an exceptionally broad specificity for arabinosyl‐decorated and undecorated xylo‐oligosaccharides, with preference for tri‐ and tetra‐saccharides. Crystal structures of BlAXBP in complex with four different ligands revealed the basis for this versatility. Uniquely, the protein was able to recognize oligosaccharides in two opposite orientations, which facilitates the optimization of interactions with the various ligands. Broad substrate specificity was further enhanced by a spacious binding pocket accommodating decorations at different mainchain positions and conformational flexibility of a lid‐like loop. Phylogenetic and genetic analyses show that BlAXBP is highly conserved within Bifidobacterium, but is lacking in other gut microbiota members. These data indicate niche adaptation within Bifidobacterium and highlight the metabolic syntrophy (cross‐feeding) among the gut microbiota.
Lonergan's philosophy as grounding for cross‐disciplinary research
Authors: Kane Anne
Published: October 2013

Increasingly, nurses conduct scientific inquiry into complex health‐care problems by collaborating on teams with researchers from other highly specialized fields. As cross‐disciplinary research proliferates and becomes institutionalized globally, researchers will increasingly encounter the need to integrate their particular research perspectives within inquiries without sacrificing the potential contributions of their discipline‐specific expertise. The work of the philosopher Bernard Lonergan (1904–1984) offers the necessary philosophical grounding. Here, I defend a role for philosophy in cross‐disciplinary research and present selected ideas in Lonergan's work. These include: (1) a dynamic, normative pattern that each inquirer operates uniquely also forms the common core, or unity, in knowing; (2) the possibility of cross‐disciplinary knowledge development is dependent on each researcher's consciousness of her or his attentiveness, intelligence, reasonableness, and responsibleness; and (3) shifts in researchers' viewpoints, or horizons, facilitate their collaborative inquiry and their grasp of the unity in knowing. The desire to know, shared by team members, drives their inquiry. Lonergan's stance is consistent with nursing values because it respects, but does not unconditionally privilege, any researcher or discipline. Arguments support a claim that Lonergan's perspective is well suited to guide nurse researchers participating on cross‐disciplinary health research teams.
What is nursing in the 21st century and what does the 21st century health system require of nursing?
Authors: Scott P. Anne , Matthews Anne , Kirwan Marcia
Published: October 2013

It is frequently claimed that nursing is vital to the safe, humane provision of health care and health service to our populations. It is also recognized however, that nursing is a costly health care resource that must be used effectively and efficiently. There is a growing recognition, from within the nursing profession, health care policy makers and society, of the need to analyse the contribution of nursing to health care and its costs. This becomes increasingly pertinent and urgent in a situation, such as that existing in Ireland, where the current financial crisis has lead to public sector employment moratoria, staff cuts and staffing deficits, combined with increased patient expectation, escalating health care costs, and a health care system restructuring and reform agenda. Such factors, increasingly common internationally, make the identification and effective use of the nursing contribution to health care an issue of international importance. This paper seeks to explore the nature of nursing and the function of the nurse within a 21st century health care system, with a focus on the Irish context. However, this analysis fits into and is relevant to the international context and discussion regarding the nursing workforce. This paper uses recent empirical studies exploring the domains of activity and focus of nursing, together with nurses perceptions of their role and work environment, in order to connect those findings with core conceptual questions about the nature and function of nursing.
Ruptured thought: rupture as a critical attitude to nursing research
Authors: Beedholm Kirsten , Lomborg Kirsten , Frederiksen Kirsten
Published: October 2013

In this paper, we introduce the notion of ‘rupture’ from the French philosopher Michel Foucault, whose studies of discourse and governmentality have become prominent within nursing research during the last 25 years. We argue that a rupture perspective can be helpful for identifying and maintaining a critical potential within nursing research. The paper begins by introducing rupture as an inheritance from the French epistemological tradition. It then describes how rupture appears in Foucault's works, as both an overall philosophical approach and as an analytic tool in his historical studies. Two examples of analytical applications of rupture are elaborated. In the first example, rupture has inspired us to make an effort to seek alternatives to mainstream conceptions of the phenomenon under study. In the second example, inspired by Foucault's work on discontinuity, we construct a framework for historical epochs in nursing history. The paper concludes by discussing the potential of the notion of rupture as a response to the methodological concerns regarding the use of Foucault‐inspired discourse analysis within nursing research. We agree with the critique of Cheek that the critical potential of discourse analysis is at risk of being undermined by research that tends to convert the approach into a fixed method.
Validation of the Persian version of the Brief Assessment of Cognition in Schizophrenia in patients with schizophrenia and healthy controls
Authors: Mazhari Shahrzad , Parvaresh Nooshin , Eslami Shahrbabaki Mahin , Sadeghi Mohammad M. , Nakhaee Nouzar , Keefe Richard S. E.
Published: October 2013

Aims

The Brief Assessment of Cognition in Schizophrenia (BACS) is designed for assessment of cognitive function in patients with schizophrenia. Versions of the BACS in English and other languages have been shown to be as sensitive to cognitive dysfunction as a standard test battery, with the advantage of brief administration and scoring time. The present study aimed to test the concurrent validity of the Persian version of the BACS (Persian‐BACS).

Methods

A group of 50 patients with schizophrenia‐spectrum disorders and a group of 50 healthy controls received the Persian‐BACS in a first session, and in a second session a standard neurocognitive battery.

Results

Cronbach's alpha for the Persian‐BACS was 0.74. All the Persian‐BACS subscales weresignificantly correlated with the corresponding standard neurocognitive subscales and the Pearson correlation of the composite scores from the two instruments was 0.71. Moreover, a one‐factor solution was found that accounted for 67.9% of the variance. Finally, the Persian‐BACS demonstrated high ability to discriminate patients with schizophrenia from healthy controls.

Conclusion

Good psychometric properties of the Persian‐BACS suggest that it is a useful tool for assessing cognition in schizophrenic patients with Persian as their primary language.
Factors associated with mental well‐being of homeless people in Japan
Authors: Ito Kae , Morikawa Suimei , Okamura Tsuyoshi , Shimokado Kentaro , Awata Shuichi
Published: October 2013

Aim

This study aimed to determine the frequency of low mental well‐being and associated factors among homeless people in Japan.

Methods

A community‐based cross‐sectional study was conducted. Data were collected through in‐person interviews of 423 homeless persons living in two areas of Tokyo. Mental well‐being was assessed using the Japanese version of the World Health Organization‐Five Well‐being Index.

Results

The overall sample comprised 392 (92.7%) men and 31 (7.3%) women. Average age was 60.6 ± 11.9 years. The mean score on the World Health Organization‐Five Well‐being Index for the 396 participants with no missing values was 11.81 ± 5.35. Based on a cut‐off criterion of 12/13, the frequency of low mental well‐being among the participants was 57.1%. In multiple logistic regression analyses, thesubjective perception of poor health (odds ratio [OR] = 3.88, 95% confidence interval [CI] = 2.32–6.49), lack of perceived emotional social support (OR = 2.77, 95%CI = 1.70–4.49), dwelling without roof (OR = 2.70, 95%CI = 1.47–4.97), and pain (OR = 1.96, 95%CI = 1.12–3.42) were significantly associated with low mental well‐being in this population.

Conclusion

The findings suggest that comprehensive intervention programs that provide supportive housing, emotional social support, and health‐care services, may be needed to improve the mental well‐being of homeless people.