bluuebunny/crossref_metadata_2025 huggingface dataset:
Transfer of blood urea nitrogen to cecal microbial nitrogen is increased by fructo‐oligosaccharide feeding in guinea pigs
Authors: Kawasaki Kiyonori , Min Xiao , Li Xiao , Hasegawa Ena , Sakaguchi Ei
Published: June 2014

The present study was conducted to determine the mechanism by which nitrogen (N) availability is improved by fructo‐oligosaccharide (FOS) in guinea pigs. Adult male guinea pigs were fed a commercial pellet diet (50 g/day) with either 5% glucose or 5% FOS for 7 days in individual metabolism cages. After 7 days of feeding the diet, 15N‐urea was administered intravenously 1 h before slaughter under anesthesia. The amount and concentration of total, protein, bacterial, ammonia and urea N and the 15N atom % excess were measured in blood, liver, gut contents and urine. The 15N atom % excess of total and protein N, and the amount of total, protein and bacteria N and 15N in the cecum were significantly increased by the consumption of FOS. Furthermore, the concentration and amount of short‐chain fatty acids were significantly increased by the consumption of FOS. In contrast, the amount of urinary 15N was significantly decreased by the consumption of FOS. These results suggest that consumption of FOS increases transfer of blood urea N into the large intestine for bacterial N synthesis, which is subsequently re‐absorbed by cecotrophy, and contributes to the increase of N utilization in guinea pigs.
Light microscopy and scanning electron microscopy studies on the reduction of the tongue microstructures in the white stork (Ciconia ciconia,Aves)
Authors: Jackowiak Hanna , Skieresz‐Szewczyk Kinga , Kwieciński Zbigniew , Godynicki Szymon , Jackowiak Katarzyna , Leszczyszyn Andrzej
Published: June 2014

The structure of the tongue in the white stork (Ciconia ciconia) is observed macroscopically and under light and scanning electron microscopy. Our observations of the tongue reveal a rare terminal reduction of the size of the tongue and microstructures of the lingual mucosa among the investigations of birds published so far. The short, triangular tongue with a pointed tip is approximately 2.5 cm long in the adult and is situated in the caudal part of the oral cavity close to the laryngeal prominence. On the dorsal surface of the tongue, no typical mucosa microstructures like lingual papillae, median groove or lingual prominence are observed. The main structure of the tongue is composed of rostral part of hyoid apparatus, that is, entoglossal cartilage connects with basihyoid. Very thin mucosa is composed of fibrous connective tissue covered with orthokeratinized epithelium. No lingual glands and muscles are observed in the lamina propria of mucosa. Even though the triangular shape of the tongue in the white stork is typical for birds, the inner structure of the reduced organ is composed only of flat cartilagineous entoglossum of hyoid apparatus. During feeding behaviour of the white stork, the food transportation in oral cavity called cranio‐inertial transport is undoubtedly affected by structural reduction of the tongue.
Clinical relevance of molecular aberrations in paediatric acute myeloid leukaemia at first relapse
Authors: Bachas Costa , Schuurhuis Gerrit Jan , Reinhardt Dirk , Creutzig Ursula , Kwidama Zinia J. , Zwaan C. Michel , van den Heuvel‐Eibrink Marry M. , De Bont Evelina S. J. M. , Elitzur Sarah , Rizzari Carmelo , de Haas Valérie , Zimmermann Martin , Cloos Jacqueline , Kaspers Gertjan J. L.
Published: June 2014

Outcome for relapsed paediatric acute myeloid leukaemia (AML) remains poor. Strong prognostic factors at first relapse are lacking, which hampers optimization of therapy. We assessed the frequency of molecular aberrations (FLT3, NRAS, KRAS, KIT, WT1 and NPM1 genes) at first relapse in a large set (n = 198) of relapsed non‐French‐American‐British M3, non‐Down syndrome AML patients that received similar relapse treatment. We correlated molecular aberrations with clinical and biological factors and studied their prognostic relevance. Hotspot mutations in the analysed genes were detected in 92 out of 198 patients (46·5%). In 72 of these 92 patients (78%), molecular aberrations were mutually exclusive for the currently analysed genes. FLT3‐internal tandem repeat (ITD) (18% of total group) mutations were most frequent, followed by NRAS (10·2%), KRAS (8%), WT1 (8%), KIT (8%), NPM1 (5%) and FLT3‐tyrosine kinase domain (3%) mutations. Presence of a WT1 aberration was an independent risk factor for second relapse (Hazard Ratio [HR] = 2·74, P = 0·013). In patients who achieved second complete remission (70·2%), WT1 and FLT3‐ITD aberrations were independent risk factors for poor overall survival (HR = 2·32, P = 0·038 and HR = 1·89, P = 0·045 respectively). These data show that molecular aberrations at first relapse are of prognostic relevance and potentially useful for risk group stratification of paediatric relapsed AML and for identification of patients eligible for personalized treatment.
Taphonomy or signal sensitivity in palaeoecological investigations ofNorselandnáminVatnahverfi, southernGreenland?
Authors: Ledger Paul M. , Edwards Kevin J. , Schofield J. Edward
Published: June 2014

Anthrosols (cf. plaggen soils) are commonly found across the homefields of Norse farms, yet the extent to which these taphonomically complex and heterogeneous deposits provide reliable archives of environmental change and vegetation history has rarely been investigated. This paper compares the palynological signature contained within an anthrosol located besideNorse farm ruins in theEasternSettlement ofGreenland, with that from a mire situated ∼400 m from the nearest archaeological remains. The investigation covers a period of ∼1000 years leading into, through, and beyond theGreenlandiclandnámofAD985. The results demonstrate that, as anticipated, the anthrosol contains a strong signal for human impact associated with settlement and occupation, although changes in both pollen percentages and accumulation rates (influx) through the profile appear smooth, not erratic, and radiocarbon dates are conformable. Thus the palynological signature contained in the anthrosol is broadly comparable to the patterns characteristic of stratified natural contexts (e.g. mires) with small pollen source areas that are located in close proximity to former Norse structures. Nevertheless, it is also demonstrated that secondary microfossils are a major component of the pollen assemblages within the anthrosol, and pollen influx is notably an order of magnitude higher when compared against the peat core taken from the mire. It is suggested that this may result from the addition of pollen contained in animal dung, augmenting that accumulating through the natural accretion of pollen derived from the surrounding vegetation and landing on the surface of the anthrosol. Although this complicates any palynological interpretation, by adopting a cautious approach we argue that anthrosols can be used to extract useful information about vegetation history at a local scale, as well as providing indirect evidence of landscape impacts and resource use around farmsteads.
Efficacy of interventions to improve feeding difficulties in children with autism spectrum disorders: a systematic review and meta‐analysis
Authors: Marshall J. , Ware R. , Ziviani J. , Hill R.J. , Dodrill P.
Published: June 2014

Background

Feeding difficulties are relatively common in children with autism spectrum disorders (ASD), but current evidence for their treatment is limited. This review systematically identifies, reviews and analyses the evidence for intervention in young children with ASD and feeding difficulties.

Methods

A comprehensive search strategy was used to identify studies from January 2000 to October 2013. Studies were included if they described interventions where the goal was to increase desirable eating behaviours or decrease undesirable eating behaviours using an experimental design, including single‐subject research methodology. Studies were reviewed for descriptive information, and research quality was appraised using a formal checklist. Individual study findings were compared using Improvement Rate Difference (IRD), a method for calculating effect size in single‐subject research.

Results

Overall, 23 papers were included. All studies reviewed had five or fewer participants, and reported on operant conditioning style intervention approaches, where the child is prompted to perform an action, and receives a contingent response. Where quality measures were not met, it was primarily due to lack of detail provided for the purposes of replication, or failure to meet social validity criteria. Meta‐analysis indicated a medium‐large effect size [mean = 0.69, 95% confidence interval (CI) 0.60 to 0.79] when the outcome measured was an increase in desirable behaviours (e.g. consuming food), but a small‐negligible effect size (mean = 0.39, 95% CI 0.18 to 0.60) when the outcome measured was a decrease in undesirable mealtime behaviours (e.g. tantrums). Only a small proportion of studies reported outcomes in terms of increased dietary variety rather than volume of food consumed.

Conclusions

The reviewed literature consisted primarily of low‐level evidence. Favourable intervention outcomes were observed in terms of increasing volume, but not necessarily variety of foods consumed in young children with ASD and feeding difficulties. Further research in the form of prospective randomized trials to further demonstrate experimental effect in this area is required.
ON THE DETERMINANTS OF LOCAL TAX RATES: NEW EVIDENCE FROM SPAIN
Authors: Delgado Francisco J. , Lago‐Peñas Santiago , Mayor Matías
Published: June 2014

This article examines the determinants of local tax rates. For the two main local taxes in Spain—the property tax and the motor vehicle tax—we test the existence of tax mimicking, yardstick competition, and political trends in a sample of 2,713 municipalities. Using various spatial models, the results support the hypothesis of tax mimicking, with coefficients above 0.40. We also show the relevance of political variables such as the ideology of incumbents and political fragmentation. The fact that incumbents with weaker political support exhibit stronger mimicking behavior is interpreted as evidence in favor of yardstick competition. Finally, we find that incumbents mimic neighboring municipalities ruled by the same political party, confirming the political trends hypothesis. (JEL C31, H71, H77)
Mocking Ads through Mobile Web Services
Authors: Gatti Lorenzo , Guerini Marco , Stock Oliviero , Strapparava Carlo
Published: June 2014

The need for creativity is ubiquitous, and mobile devices connected to Web services can help us. Linguistic creativity is widely used in advertisements to surprise us, to get our attention, and to stick concepts in our memory. However, creativity can also be used as a defense. When we walk in the street, we are overwhelmed by messages that try to get our attention with any persuasive device at hand. As messages get ever more aggressive, often our basic cognitive defenses—trying not to perceive those messages—are not sufficient. One advanced defensive technique is based on transforming the perceived message into something different (for instance, making use of irony or hyperbole) from what was originally meant in the message. In this article, we describe an implemented application for smartphones, which creatively modifies the linguistic expression in a virtual copy of a poster encountered on the street. The mobile system is inspired by the subvertising practice of countercultural art.
Photocrosslinked ultrathin anionic polysaccharide supports for accelerated growth of human mesenchymal stem cells
Authors: Mikulska A. , Filipowska J. , Osyczka A. M. , Szuwarzyński M. , Nowakowska M. , Szczubiałka K.
Published: June 2014

Objectives

Properties of cell culture supports obtained from ultrathin multilayer films containing anionic natural polysaccharides (PSacs) and a synthetic polycation were studied.

Materials and methods

Supports were prepared via a layer‐by‐layer (LbL) self‐assembly deposition method. Polymers used were: heparin (Hep), chondroitin sulphate (CS), hyaluronic acid (HA), and ι‐carrageenan (Car) as polyanions, and diazoresin (DR) as a polycation. PSac layers were crosslinked with DR layers by irradiation with UV light absorbed by DR resin.

Results

DR/PSac films are very efficient cell culture growth supports as found from experiments with human mesenchymal stem cells (hMSCs). Irradiation of the films resulted in changing zeta potential of outermost layers of both DR and PSac to more negative values, and in increased film hydrophobicity, as found from the contact angle measurements. Photocrosslinking of the supports led to their increased stability.

Conclusions

The supports allow for obtaining intact cell monolayers faster than when typical polystyrene tissue culture plates are used. Moreover, these monolayers spontaneously detach permitting formation of new cell layers on these surfaces relatively early during culture, compared to cells cultured on commonly used tissue culture plastic.
Supplier Selection Behavior Under Uncertainty: Contextual and Cognitive Effects on Risk Perception and Choice
Authors: Kull Thomas J. , Oke Adegoke , Dooley Kevin J.
Published: June 2014

Buyers often make supplier selection decisions under conditions of uncertainty. Although the analytical aspects of supplier selection are well developed, the psychological aspects are less so. This article uses supply chain management and behavioral decision theories to propose that attributes of the purchasing situation (category difficulty, category importance, and contingent pay) affect cognition that, in turn, affects a supply manager's choice. We conducted a supplier selection behavioral experiment with practicing managers to test the model's hypotheses. When the context involves an important or difficult sourcing category, higher risk perceptions exist that increase preference for a supplier with more certain outcomes, even when that choice has a lower expected payoff. However, the presence of contingent pay decreases risk perceptions through higher perceived supplier control. We also find that a manager's risk propensity increases preferences for a supplier with less certain outcomes regardless of perceived risk. Our model and results provide a theoretical framework for further study into the cognitive aspects of supplier selection behavior and provide insight into biases that influence practicing supply chain managers.
COMPETITION AND COOPERATION IN A PUBLIC GOODS GAME: A FIELD EXPERIMENT
Authors: Augenblick Ned , Cunha Jesse M.
Published: June 2014

We explore the effects of competitive and cooperative motivations on contributions in a field experiment. A total of 10,000 potential political donors received solicitations referencing past contribution behavior of members of the competing party (competition treatment), the same party (cooperative treatment), or no past contribution information (control). We first theoretically analyze the effect of these treatments on the contribution behavior of agents with different social preferences in a modified intergroup public good (IPG) game. Then, we report the empirical results: Contribution rates in the competitive, cooperative, and control treatments were 1.45%, 1.08%, and 0.78%, respectively. With the exception of one large contribution, the distribution of contributions in the competitive treatment first order stochastically dominates that of the cooperative treatment. Qualitatively, it appears that the cooperative treatment induced more contributions around the common monetary reference point, while the competitive treatment led to more contributions at twice this amount. These results suggest that eliciting competitive rather than cooperative motivations can lead to higher contributions in IPG settings. (JEL D72, H41, C93)
GONE FISHING! REPORTED SICKNESS ABSENTEEISM AND THE WEATHER
Authors: Shi Jingye , Skuterud Mikal
Published: June 2014

A fundamental challenge in informing employer–employee agency problems is measuring employee shirking activity. We identify the propensity of employees to misreport health in order to exploit favorable weather by linking Canadian weather data and survey data on short‐term spells of sickness absenteeism among indoor workers during the non‐winter months. The results point to a clear tendency for reported sickness absenteeism to rise with the recreational quality of the weather. Comparing across workers suggests larger marginal weather effects where shirking costs are higher, which we show is consistent with employees' marginal utility of outdoor leisure increasing in the interaction of their health and weather quality. We discuss the implications of our findings for flexible vacation policies and survey respondents' trust in the confidentiality guarantees of statistical agencies. (JEL D82, I10, J22)
FLIP‐FLOPPING: IDEOLOGICAL ADJUSTMENT COSTS IN THE UNITED STATES SENATE
Authors: DeBacker Jason Matthew
Published: June 2014

Using a long panel of roll call voting data, I find that “flip‐flopping” senators face significant electoral costs when changing positions. In models of electoral competition, as the costs to candidates changing position approach zero, the equilibrium prediction is the convergence of platforms. Such convergence is at odds with empirical observation. Using a dynamic, structural model of candidate positioning, I identify the nature of the costs associated with changing position that may result in such non‐convergence. (JEL D72, C61, H11)
THE LABOR MARKET IN THE ART SECTOR OF BAROQUE ROME
Authors: Etro Federico , Marchesi Silvia , Pagani Laura
Published: June 2014

We analyze the labor market for painters in Baroque Rome using unique data on primary sales of portraits, still lifes, genre paintings, landscapes, and figurative paintings. In line with the traditional artistic hierarchy of genres, average price differentials between them were high. The matched painter‐patron nature of the dataset allows us to evaluate the extent to which price heterogeneity is related to unobservable characteristics of painters and patrons. We find that the market allocated artists between artistic genres to the point of equalizing the marginal return of each genre. Residual price differences at the employer level can be explained in terms of incentive mechanisms to induce effort in the production of artistic quality and compensating wage differentials. (JEL C23, D8, J3, Z11)
Collateral projections from vestibular nuclear and inferior olivary neurons to lobules I/II and IX/X of the rat cerebellar vermis: a double retrograde labeling study
Authors: Lee Ray X. , Huang Jian‐Jia , Huang Chiming , Tsai Meng‐Li , Yen Chen‐Tung
Published: June 2014

Axon collateral projections to various lobules of the cerebellar cortex are thought to contribute to the coordination of neuronal activities among different parts of the cerebellum. Even though lobules I/II and IX/X of the cerebellar vermis are located at the opposite poles in the anterior–posterior axis, they have been shown to receive dense vestibular mossy fiber projections. For climbing fibers, there is also a mirror‐image‐like organisation in their axonal collaterals between the anterior and posterior cerebellar cortex. However, the detailed organisation of mossy and climbing fiber collateral afferents to lobules I/II and IX/X is still unclear. Here, we carried out a double‐labeling study with two retrograde tracers (FluoroGold and MicroRuby) in lobules I/II and IX/X. We examined labeled cells in the vestibular nuclei and inferior olive. We found a low percentage of double‐labeled neurons in the vestibular nuclei (2.1 ± 0.9% of tracer‐labeled neurons in this brain region), and a higher percentage of double‐labeled neurons in the inferior olive (6.5 ± 1.9%), especially in its four small nuclei (18.5 ± 8.0%; including the β nucleus, dorsal cap of Kooy, ventrolateral outgrowth, and dorsomedial cell column), which are relevant for vestibular function. These results provide strong anatomical evidence for coordinated information processing in lobules I/II and IX/X for vestibular control.
An unknown quantity—The worldwide prevalence of epilepsy
Authors: Bell Gail S. , Neligan Aidan , Sander Josemir W.
Published: June 2014

The reported incidence (rate of new cases in a population) of epilepsy is consistently lower in high‐income than in lower‐income economies, whereas opinions vary regarding comparative prevalence rates (proportion of the population with epilepsy). For any condition that does not influence mortality, lifetime prevalence should approximate to the cumulative incidence. We suspected that epilepsy prevalence might be uniform throughout the world, whereas incidence is higher in resource‐poor countries. To test whether our suspicion was reasonable, we conducted a Medline search to estimate the prevalence of active and lifetime epilepsy in different economic areas throughout the world. We found that the range of estimated prevalence of epilepsy may be broadly similar throughout the world, but comparison is limited by lack of door‐to‐door studies in high‐income economies and by variations in the definitions of active epilepsy. We contend that any inconsistencies between incidence and prevalence are due largely to the excess premature death rate in people with epilepsy in lower‐income economies. Much of the variability in epidemiologic indices arises from differences in study methodology, definitions, and risk factors. The epidemiology of epilepsy, and particularly its mortality, needs thorough investigation using uniform definitions that do not include antiepileptic drug use; causes of death should be identified and actions, including treatment and education, should be taken to avoid preventable deaths.

A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
Safety and effectiveness of long‐term treatment with diazepam auto‐injector administered by caregivers in an outpatient setting for the treatment of acute repetitive seizures
Authors: Rogin Joanne , Wheless James , Abou‐Khalil Bassel , Wolter Kevin D. , Pixton Glenn C. , Sherman Nancy A. , Shukla Rajesh B. , Roland Carl L. , Sommerville Kenneth W.
Published: June 2014

Objective

Part 1 of this phase III study was a randomized, double‐blind, parallel‐group, placebo‐controlled, multicenter study of caregiver administered diazepam auto‐injector (AI) in subjects with acute repetitive seizures (ARS) and demonstrated that diazepam AI was well‐tolerated and significantly more effective than placebo AI in delaying the time to next seizure or rescue. Part 2 of this study, presented herein, was an open‐label continuation to assess the long‐term safety and effectiveness of diazepam AI for the treatment of ARS.

Methods

Of the 234 subjects randomized in part 1, 161 continued into part 2 and were provided open‐label diazepam AI. Effectiveness measures were time to next seizure or rescue, number of subsequent rescues by type (rescue medication, emergency room visit, or other medical care), and number of subsequent seizures during the 12‐h follow‐up period. Safety data (adverse events and respirations <8/min) were also collected.

Results

During the open‐label part 2 study, 129 subjects were administered a total of 1,380 diazepam AI treatments (median 4.5; range 1–118), of which 1,071 (77.6%) were effective with no subsequent seizure or rescue during the 12‐h follow‐up period. Median number of subsequent seizures experienced by subjects was one (range 0–20). Of the 1,380 administrations, 79 (5.7%) required use of rescue medication, 18 (1.3%) required a visit to an emergency room, and 6 (0.4%) required other rescue medical care. In most (75%) of subjects with treatment‐emergent adverse events (TEAEs), TEAEs were mild or moderate in severity. Commonly reported treatment‐related TEAEs were injection‐site pain (10.9%), injection‐site hemorrhage (7%), and injection‐site bruising (6.3%). Although three subjects met the predefined respiratory rate threshold, none were considered clinically significant or reported as AEs.

Significance

Long‐term treatment with diazepam AI administered by trained caregivers in an outpatient setting to treat ARS is a safe and effective option.

A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
Automated versus manual hippocampal segmentation in preoperative and postoperative patients with epilepsy
Authors: Germeyan Sierra C. , Kalikhman David , Jones Lucy , Theodore William H.
Published: June 2014

Objective

To compare manual and automated preoperative and postoperative hippocampal volume measurements in patients with intractable epilepsy.

Methods

We studied 34 patients referred to the Clinical Epilepsy Section, National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH) for evaluation of intractable epilepsy and 21 normal volunteers who received 1.5 or 3 T GE Signa magnetic resonance imaging (MRI) scans. Hippocampal volumes were traced manually on each slice and assembled into three‐dimensional volumes by investigators who were blinded to other data. Automated volumetric measurements were obtained using FreeSurfer. Statistical analysis was performed with GraphPad Prism.

Results

Automated hippocampal volumes were larger than manual volumes in both patients and normal volunteers (p < 0.05). Right to left hemisphere hippocampal ratio and percent of hippocampus resected did not differ significantly by segmentation method. It was not possible to obtain accurate total resection volumes with the automated method.

Significance

Values such as side‐to‐side ratio and percent resected may be more directly translatable between manual and automated methods than absolute measures of volume. Accurate determination of resection volumes is important for studies of the effects of surgery on both seizure control and postoperative neuropsychological deficits. Our preliminary data suggest that FreeSurfer may provide an accurate and simple method for quantitating hippocampal resections. However, it may be less valuable for large or extratemporal resections, or when distortions of normal anatomy are present.

A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
Changes in quality of life after epilepsy surgery: The role of reprioritization response shift
Authors: Sajobi Tolulope T. , Fiest Kirsten M. , Wiebe Samuel
Published: June 2014

Objectives

Health‐related quality of life (HRQOL) is an important indicator of well‐being in patients with epilepsy. When assessing changes in HRQOL over time, some of the changes observed may be due to the patients' change in the internal standards, value, or meaning that they attribute to the HRQOL domain being measured, rather than actual change, a phenomenon known as response shift. Response shifts are increasingly recognized as an important explanation for the seemingly paradoxical or counterintuitive HRQOL results often observed in chronic conditions. We investigated the presence of changing values (reprioritization response shift) in data from a surgical randomized controlled trial of patients with temporal lobe epilepsy (TLE).

Methods

Eighty patients with TLE, who were randomized to surgical and medical treatment, provided data on the epilepsy‐specific 31‐item Quality of life in Epilepsy (QOLIE‐31) questionnaire at baseline and 1 year after randomization. Reprioritization response shift among the seven QOLIE‐31 domains was assessed using changes in the relative importance weights derived from logistic regression and discriminant analysis.

Results

The relative importance analysis showed a statistically significant increase over time in the importance of social function, but a significant decrease in the relative importance of seizure worry. There were no significant changes in the relative importance of the remaining five domains in distinguishing between surgical and medical group over time.

Significance

Patients that receive surgical treatment are more likely to experience a decrease in the valuation of seizure worry and an increase in the value of social function as compared to patients who are medically treated. Changes in expectation about seizure freedom and social function may confound the assessment of longitudinal change on these outcomes, and highlight the importance of assessing response shift and the limitations of assessing HRQOL at a single point in time or without a control group.

A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
Long‐term prognosis of patients with Ehlers‐Danlos syndrome and epilepsy
Authors: Verrotti Alberto , Spartà Maria Valentina , Monacelli Debora , Porto Rossella , Castagnino Miriam , Russo Raucci Annalisa , Compagno Francesca , Viglio Simona , Foiadelli Thomas , Nicita Francesco , Grosso Salvatore , Spalice Alberto , Chiarelli Francesco , Marseglia Gianluigi , Savasta Salvatore
Published: June 2014

Objective

Epilepsy in Ehlers‐Danlos syndrome (EDS) has been reported in the literature, but there are no studies that have investigated in detail clinical and electroencephalography (EEG) features in patients with EDS, and that have compared the outcome of epilepsy in subjects with or without brain lesions. We report a series of 42 patients with EDS and epilepsy, including data that concern clinical characteristics, EEG abnormalities, brain malformations at magnetic resonance imaging (MRI) and long‐term outcome.

Methods

EEG, clinical information, and neuroimaging characteristics in 42 patients with EDS were analyzed at the onset of epilepsy and after long‐term follow‐up (at least 5 years). We subdivided the patients into two groups: group A, 26 patients without brain abnormalities; group B, 16 patients with brain lesions, often with periventricular heterotopia (PH).

Results

Group A patients: Most cases (19 of 26) presented focal epilepsy, whereas 7 of 26 were affected by generalized epilepsy; interictal EEG showed temporal or temporoparietal spikes in most cases. Twenty‐three patients received antiepileptic drug (AED) monotherapy; three patients were treated with polytherapy. During follow‐up, all patients were seizure‐free for at least 2 years, and only one continued to receive AEDs. Group B patients: the majority presented focal epilepsy (9 of 16), but many patients had generalized epilepsy (7 of 16); interictal EEG showed usually frontal or frontotemporal spikes and waves. Many patients (12 of 16) received AED polytherapy. During follow‐up, 12 patients were seizure‐free, and all patients continued pharmacologic treatment.

Significance

All patients without brain lesions showed a favorable response to AED monotherapy and were seizure‐free after a few years of treatment. Patients with central nervous system abnormalities had a worse outcome, suggesting that the presence of brain lesions could influence the long‐term evolution in these patients.

A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
Stability and folding of amphibian ribonuclease A superfamily members in comparison with mammalian homologues
Authors: Arnold Ulrich
Published: June 2014

Comparative studies on homologous proteins can provide knowledge on how limited changes in the primary structure find their expression in large effects on catalytic activity, stability or the folding behavior. For more than half a century, members of the ribonuclease A superfamily have been the subject of a myriad of studies on protein folding and stability. Both the unfolding and refolding kinetics as well as the structure of several folding intermediates of ribonuclease A have been characterized in detail. Moreover, the RNA‐degrading activity of these enzymes provides a basis for their cytotoxicity, which renders them potential tumor therapeutics. Because amphibian ribonuclease A homologues evade the human ribonuclease inhibitor, they emerged as particularly promising candidates. Interestingly, the amphibian ribonuclease A homologues investigated to date are more stable than the mammalian homologues. Nevertheless, despite the generation of numerous genetically engineered variants, knowledge of the folding of amphibian ribonuclease A homologues remains rather limited. An exception is onconase, a ribonuclease A homologue from Rana pipiens, which has been characterized in detail. This review summarizes the data on the unfolding and refolding kinetics and pathways, as well on the stability of amphibian ribonuclease A homologues compared with those of ribonuclease A, the best known member of this superfamily.
Statin‐induced inhibition of breast cancer proliferation and invasion involves attenuation of iron transport: intermediacy of nitric oxide and antioxidant defence mechanisms
Authors: Kanugula Anantha Koteswararao , Gollavilli Paradesi Naidu , Vasamsetti Sathish Babu , Karnewar Santosh , Gopoju Raja , Ummanni Ramesh , Kotamraju Srigiridhar
Published: June 2014

Accumulating evidence from in vitro, in vivo, clinical and epidemiological studies shows promising results for the use of statins against many cancers including breast carcinoma. However, the molecular mechanisms responsible for the anti‐proliferative and anti‐invasive properties of statins still remain elusive. In this study, we investigated the involvement of nitric oxide, iron homeostasis and antioxidant defence mechanisms in mediating the anti‐proliferative and anti‐invasive properties of hydrophobic statins in MDA‐MB‐231, MDA‐MB‐453 and BT‐549 metastatic triple negative breast cancer cells. Fluvastatin and simvastatin significantly increased cytotoxicity which was reversed with mevalonate. Interestingly, fluvastatin downregulated transferrin receptor (TfR1), with a concomitant depletion of intracellular iron levels in these cells. Statin‐induced effects were mimicked by geranylgeranyl transferase inhibitor (GGTI‐298) but not farnesyl transferase inhibitor (FTI‐277). Further, it was observed that TfR1 downregulation is mediated by increased nitric oxide levels via inducible nitric oxide synthase (iNOS) expression. NOS inhibitors (asymmetric dimethylarginine and 1400W) counteracted and sepiapterin, a precursor of tetrahydrobiopterin, exacerbated statin‐induced depletion of intracellular iron levels. Notably, fluvastatin increased manganese superoxide dismutase (by repressing the transcription factor DNA damage‐binding protein 2), catalase and glutathione which, in turn, diminished H2O2 levels. Fluvastatin‐induced downregulation of TfR1, matrix metalloproteinase‐2, ‐9 and inhibition of invasion were reversed in the presence of aminotriazole, a specific inhibitor of catalase. Finally, we conclude that fluvastatin, by altering iron homeostasis, nitric oxide generation and antioxidant defence mechanisms, induces triple negative breast cancer cell death.
Geography's role in nurturing postgraduate students
Authors: Gillen Jamie , Ziegler Alan D , Friess Daniel A , Wasson Robert
Published: June 2014

In this commentary we encourage thinking about how to effectively nurture postgraduate students in geography. We offer some ideas and strategies for supervisors who mentor postgrads for careers in and beyond the academy. We also consider how supervisor–student relationships can take advantage of geography's interdisciplinarity to develop a well rounded postgraduate experience.
Insulin resistance in patients with post‐adolescent acne
Authors: Balta Ilknur , Ekiz Ozlem , Ozuguz Pınar , Ustun Ihsan , Karaca Semsettin , Dogruk Kacar Seval , Eksioglu Meral
Published: June 2014

Background

Post‐adolescent acne has been defined as acne in a patient aged >25 years. Acne vulgaris first develops at the onset of puberty as a result of hormonal changes. During puberty, there is a transient decline in insulin sensitivity. We hypothesized that insulin resistance might persist after puberty in patients with post‐adolescent acne.

Objectives

This study was conducted in order to investigate the relationship between post‐adolescent acne and insulin resistance.

Methods

The study population comprised 35 patients with post‐adolescent acne and 35 healthy control subjects. The parameters measured were fasting blood glucose, insulin, aspartate aminotransferase (AST) and alanine aminotransferase (ALT), total cholesterol (TC), triglycerides, high‐density lipoprotein cholesterol (HDL‐C), and low‐density lipoprotein cholesterol (LDL‐C). The homeostasis model assessment of insulin resistance (HOMA‐IR) index was calculated for each individual.

Results

No significant differences were observed between patients with post‐adolescent acne and control subjects in fasting blood glucose, fasting insulin, AST, ALT, triglyceride and HDL‐C levels, and HOMA‐IR index. There were also no correlations between these parameters and the severity of acne.

Conclusions

This study suggests that insulin resistance may not play a major role in the pathogenesis of post‐adolescent acne. Hormonal changes, genetic susceptibility, stress, the use of cosmetics, drugs, and environmental factors should be considered in the development of post‐adolescent acne.
Kaposi's sarcoma: a population‐based cancer registry descriptive study of 57 consecutive cases diagnosed between 1977 and 2009
Authors: Laresche Claire , Fournier Evelyne , Dupond Anne Sophie , Woronoff Anne Sophie , Drobacheff‐Thiebaut Christine , Humbert Philippe , Aubin Francois
Published: June 2014

Background

There are few epidemiological data available on rare skin cancer, including Kaposi's sarcoma (KS), which is a multifocal illness affecting the skin, mucosa, and viscera. Four different types of KS have been described: classic, AIDS‐associated, iatrogenic, and African. The purpose of this study was to describe the epidemiology and evolution of the different types of KS in the Doubs region of France.

Patients and methods

A retrospective population‐based study was conducted, including 57 patients with KS from the Doubs region between 1977 and 2009. These patients were identified by the tumor registry of the Doubs region.

Results

A larger proportion of AIDS‐associated KS (61%) was observed compared to classic KS (30%) and iatrogenic KS (9%). No cases of African KS were observed. Most new cases were observed between 1987 and 1996 due to the AIDS explosion. The patients were predominantly male, with a male/female ratio at 10 : 4 (34 for AIDS‐associated KS). The mean age of patients was 50.2 (higher in classic and iatrogenic KS and lower in AIDS‐associated KS). Mucosal and visceral forms were more common in AIDS‐associated KS, explaining the higher rate of mortality due to KS (21%).

Conclusion

KS, which used to be a rare illness, as it existed only in the classic form, has become more common since the appearance of HIV and, to a lesser extent, of immunosuppressive treatment.
PHACE syndrome, a series of six patients: clinical and morphological manifestations, propranolol efficacy, and safety
Authors: Bellaud Guillaume , Puzenat Eve , Billon‐Grand Nora Célia , Humbert Philippe , Aubin François
Published: June 2014

Introduction

PHACE syndrome is defined by infantile hemangiomas associated with a spectrum of malformations. These abnormalities involve posterior fossa of the brain, cerebral vasculature, heart or aorta, eyes, and midline thorax or sternum.

Case reports

Six cases of PHACE syndrome were diagnosed between 2002 and 2011 in the Department of Dermatology at Besançon Hospital. All patients were female and had an infantile hemangioma of the face over 5 cm in size. Five of them had encephalic abnormalities, and arterial malformations were found in three patients. Only one patient showed an ocular abnormality. None of them presented any cardiac, aortic, or sternal malformations. Two patients benefited from treatment with propranolol with good effectiveness and safety.

Discussion

The presence of an infantile hemangioma over 5 cm in size on the face should be a suspected sign for PHACE syndrome and requires the completion of a systematic evaluation. Diagnosis is based on recently revised criteria and demonstrates the large polymorphism of the malformations that may be encountered.

Conclusion

This descriptive series is in accordance with literature data including female preponderance and the frequent involvement of the posterior fossa. It also underlines the effectiveness of propranolol for this indication.
Cutaneous non‐tuberculous mycobacterial infections
Authors: Lamb Ruth C. , Dawn Goutam
Published: June 2014

Mycobacteria cause a range of diseases in both immunocompetent and immunosuppressed individuals and may affect many different organs. The most noticeable recent change in patterns of cutaneous mycobacterial infection refers to the increase in non‐tuberculous mycobacterial (NTM) infections. This review focuses on the clinical manifestations, diagnosis, and treatment of cutaneous NTM infections. The MEDLINE, PubMed, and Cochrane Library databases were searched using the keywords “non‐tuberculous mycobacteria,” “atypical mycobacteria,” and “mycobacteria other than tubercle bacilli”. Publications on cutaneous NTM infections written in English were included in this review. Literature published by the World Health Organization was examined for further material. References in review articles were screened for other studies not already identified. The clinical features, diagnosis, and treatment of NTM infections were reviewed. Cutaneous mycobacterial disease may take many guises and may appear benign in nature. Chronic cutaneous lesions at the sites of trauma or surgical procedures, especially those that fail to respond to standard antibiotic therapy, should prompt the consideration of mycobacterial disease. The lack of rapid techniques for the identification of pathogens makes diagnosis challenging. The lack of randomized controlled trials on the efficacy of antimicrobial agents means that empiric therapy may fail, necessitate prolonged combinations of antibiotics, and increase the probability of side effects and diminished compliance.
Identification of a novel U2HR mutation in a Korean woman with Marie Unna hereditary hypotrichosis
Authors: Yun Seok‐Kweon , Cho Yong‐Gon , Song Ki Hun , Hwang Su‐Ran , Kim Yoon Sung‐Joo , Choi Keon‐Woo , Kim Han‐Uk , Park Jin
Published: June 2014

Marie Unna hereditary hypotrichosis (MUHH) is a rare autosomal dominant genodermatosis characterized by progressive non‐scarring hair loss. Mutation of the U2HR gene, located in chromosome 8p21, is generally responsible for MUHH development. Until now, 17 mutations of U2HR have been identified from various ethnic backgrounds, but U2HR mutations have been identified mostly in Chinese families and only one Japanese patient with MUHH among Asian populations. Here, we report the first Korean case of MUHH with a novel heterozygous missense mutation (c.80C>T) in U2HR that has not been documented to date. Genetic analysis further revealed that this mutation is responsible for the hair morphology phenotype presented in this case. This finding contributes to expansion of the mutant spectrum of U2HR, supporting the possibility of racial differences in terms of genetic mutations of MUHH.
Hidradenitis suppurativa: the role of immune dysregulation
Authors: Kelly Genevieve , Sweeney Cheryl M. , Tobin Anne‐Marie , Kirby Brian
Published: June 2014

Hidradenitis suppurativa (HS) is a chronic relapsing inflammatory disease of follicular occlusion characterized by boils, sinus tracts, fistulae, and scarring. It has a significant underestimated morbidity. Antimicrobial, immunosuppressive, anti‐androgenic, and surgical approaches have been used with varying results. Knowledge of the pathogenesis of HS is fragmented, and treatment choices have hitherto been empiric without an exact understanding of the scientific basis for their use. Tumor necrosis factor‐α inhibitors have shown promise in the treatment of HS in recent years, and the concept of HS as an immunological condition has come to the fore. The focus of this review is to discuss the immunological abnormalities underpinning HS as elucidated to date.
Basosquamous carcinoma and metatypical basal cell carcinoma: a review of treatment with Mohs micrographic surgery
Authors: Allen Kattie J. , Cappel Mark A. , Killian Jill M. , Brewer Jerry D.
Published: June 2014

Background

Basosquamous carcinoma (BSC) and metatypical basal cell carcinoma (MBCC) are uncommon tumors poorly defined in the literature. Available studies suggest these tumors carry a greater risk of recurrence and metastases than basal cell carcinomas (BCCs) and, in some studies, squamous cell carcinomas. Formal treatment recommendations are not fully established.

Objective

To analyze BSC and MBCC separately, evaluate whether they are distinct tumor subtypes, and analyze Mohs micrographic surgery (MMS) efficacy for BSC and MBCC.

Methods

Retrospective review of medical records and histologic specimens was conducted for 293 patients with 303 biopsy‐proven BSCs or MBCCs treated with MMS between 1996 and 2004. In total, 32 BSCs and 128 MBCCs were identified. Surgical and follow‐up data were analyzed.

Results

Kaplan–Meier estimates of recurrence‐free survival after MMS were 100% at one year for both tumor subtypes and were 100% for BSC and 93.8% for MBCC at 5 years. Initial mean sizes were 1.5 cm for BSC and 1.3 cm for MBCC. Approximately 7% represented recurrent tumors at surgery. Of six patients with recurrences, none had known metastatic disease.

Limitations

Limitations include retrospective design, analysis of only head and neck sites, and small sample sizes.

Conclusion

BSC and MBCC showed no significant distinguishing characteristics to separate them into two BCC subtypes. Reported recurrence rates for BSC and MBCC are 12–45% with wide local excision; estimated recurrence rates are 4.1% with MMS. Our study showed recurrence‐free survival of 95.1% at five years. Hence, MMS is effective in treating these BCC subtypes.
Epidemiology of sarcoidosis in Afro‐Caribbean people: a 7‐year retrospective study in Guadeloupe
Authors: Coquart Nolwenn , Cadelis Gilbert , Tressières Benoît , Cordel Nadege
Published: June 2014

Background

There are no reliable epidemiological data on sarcoidosis in the French West Indies, although this disease is known to be more frequent and more severe in Black African‐Americans and West Indians.

Objectives

This retrospective study aimed to assess the incidence and prevalence of sarcoidosis in Guadeloupe over a 7‐year period and to determine its epidemiological, clinical, and evolutionary characteristics.

Methods

Patients were identified through the computerized databases of the three pathology laboratories and two hospitals on the islands of Guadeloupe. Histologically proven cases of sarcoidosis were selected. All patients were recalled at a single study time‐point.

Results

A total of 75 patients were identified. These included 44 women and 31 men (sex ratio: 1.4), with a mean ± standard deviation (SD) age of 47 ± 14 years and Fitzpatrick skin types IV–VI. The average incidence was 2.28 per 100,000 inhabitants per year (95% confidence interval [CI] 1.69–3.02). The prevalence of sarcoidosis in 2009 was 21.09 per 100,000 inhabitants (95% CI 16.00–26.18). Most patients (61/71, 85.9%) exhibited multiple organ involvement; the mean ± SD number of organs involved was 2.6 ± 1.1. The initiation of systemic therapy was required in 75.7% of cases. Several lines of treatment were necessary in 41.5% of affected patients. At the study time‐point, seven patients were found to have died. Four of these deaths were directly attributable to sarcoidosis (mortality rate: 5.3%).

Conclusions

This epidemiological study on sarcoidosis in Guadeloupe reveals a low incidence of the disease and a high degree of severity as evidenced by the average number of affected organs, the high frequency of extrathoracic organ involvement, the frequent use of corticosteroids, and a mortality rate of 5.3%.