bluuebunny/crossref_metadata_2025 huggingface dataset:
Detection of paroxysmal nocturnal hemoglobinuria clones to exclude inherited bone marrow failure syndromes
Authors: DeZern Amy E. , Symons Heather J. , Resar Linda S. , Borowitz Michael J. , Armanios Mary Y. , Brodsky Robert A.
Published: February 2014

Background

Inherited bone marrow failure syndromes (IBMFS) and acquired aplastic anemia (AA) are life‐threatening marrow failure disorders. These entities can be difficult to distinguish because they present similarly. Correct diagnosis is imperative for proper therapy.

Design and methods

This is a retrospective, single‐center study of patients <40 yr of age, evaluated for bone marrow failure, and assayed for the presence of aPNHclone in the pediatric or adult hematology/oncology clinics from 2001 to present. Patients were also evaluated forIBMFS.

Results

We present results from 156 patients with marrow failure, 20 of whom haveIBMFS. None of theIBMSFpatients had paroxysmal nocturnal hemoglobinuria (PNH) clones.

Conclusions

Although further studies are needed, our results suggest that the detection of aPNHclone can be a useful diagnostic tool to exclude the diagnosis ofIBMFSand focus the work‐up and treatment on an acquired form of marrow failure.
Male Courtship Pheromones Affect Female Behaviour in the Swordtail Characin (Corynopoma riisei)
Authors: Amcoff Mirjam , Hallsson Lára R. , Winberg Svante , Kolm Niclas
Published: February 2014

Pheromones constitute an important cue used by both males and females during courtship. Here, we investigate the effect of male pheromones on female behaviour in the swordtail characin (Corynopoma riisei), a species of fish where males have a caudal pheromone gland which has been suggested to affect female behaviour during courtship. We subjected female C. riisei to male courtship pheromones and investigated the effect on both female behaviour and brain serotonergic activity levels compared to a control group. While no difference in serotonergic activity was found, the pheromone‐treated females showed lower stress levels compared to the control group. Furthermore, pheromone‐treated females increased locomotor activity over time, while a decrease in locomotor activity was observed in the control group. These results suggest that the male courtship pheromones may serve to reduce female stress and increase female activity, possibly to aid males in gaining access to females and facilitating sperm transfer.
Biochemical, histopathological and behavioral alterations caused by intrastriatal administration of quinolic acid to young rats
Authors: Pierozan Paula , Fernandes Carolina G. , Dutra Márcio F. , Pandolfo Pablo , Ferreira Fernanda , de Lima Bárbara O. , Porciúncula Lisiane , Wajner Moacir , Pessoa‐Pureur Regina
Published: February 2014

Quinolinic acid (QUIN) is a neuroactive metabolite of the kinurenine pathway, and is considered to be involved in aging and some neurodegenerative disorders, including Huntington's disease. QUIN was injected intrastriatally into adolescent rats, and biochemical and histopathological analyses in the striatum, cortex, and hippocampus, as well as behavioral tests, were carried out in the rats over a period of 21 days after drug injection. Decreased [3H]glutamate uptake and increased 45Ca2+ uptake were detected shortly after injection in the striatum and cerebral cortex. In the hippocampus, increased 45Ca2+ uptake preceded the decreased [3H]glutamate uptake, without histopathological alterations. Also, corticostriatal astrogliosis was observed 7 days later, progressing to neuronal death at day 14. QUIN‐treated rats also showed cognitive deficits 24 h after injection, concurrently with striatal astrogliosis. Motor deficits appeared later, after corticostriatal neurodegeneration. We assume that glutamate excitotoxicity could represent, at least in part, a molecular mechanism associated with the cognitive and motor impairments, corticostriatal astrogliosis and neuronal death observed in the QUIN‐treated rats. We propose that our findings could be relevant for understanding the pathophysiology of human neurodegenerative diseases affecting young people, such as the juvenile form of Huntington's disease, and for the design of potential therapeutic strategies to slow down the progression of the disease.
Estimation of bone marrow cellularity using digital image nucleated cell counts in patients receiving chemotherapy
Authors: Kim Y. , Kim M. , Kim Y. , Han J. H. , Han K.
Published: February 2014

Introduction

Bone marrow cellularity under microscopic examination has a wide range of variation. The number of nucleated cells in a certain volume of bone marrow could represent more accurate cellularity. In this study, we compared the cellularity under the microscope with the nucleated cell counts on the biopsy sections to assess the usefulness of nucleated cell count as a representative cellularity marker.

Methods

Three hundred and twenty‐five bone marrow biopsies were analyzed. Two pathologists determined bone marrow cellularity using digital images with ×100 magnification from biopsy sections. Nucleated cell counts on the digital images with ×100 magnification were determined using automated cell counting software.

Results

A wide range of variation was observed in the nucleated cell counts among the biopsies with the same cellularity. A good correlation was observed between the cellularity under the microscope (×100 magnification) and the nucleated cell counts (r = 0.816, P < 0.05). The nucleated cell counts, using the automated cell counting software, had no significant interobserver difference (F = 0.001, P = 0.972). The intraclass correlation coefficient (ICC) of the nucleated cell count between two observers was 0.952.

Conclusion

The nucleated cell counts in the bone marrow biopsy could represent the number of residual hematopoietic cells more accurately than the method of cellularity under the microscope. The nucleated cell counts in the bone marrow biopsy could be especially helpful to determine cellularity after initial chemotherapy.
Clinical research potential in Victorian hospitals: the Victorian clinician researcher needs analysis survey
Authors: Hiscock H. , Ledgerwood K. , Danchin M. , Ekinci E. , Johnson E. , Wilson A.
Published: February 2014

Background

The 2012 McKeon Review highlighted the role of clinician researchers in patient based research and the need to foster this capacity. While anecdotal evidence suggests that clinician researchers are under threat and underfunded, Australian data on barriers and enablers of clinician‐led research are scant.

Aims

To describe (i) characteristics of clinician researchers; (ii) for research‐active clinicians: areas of research, barriers/enablers of research and factors associated with funding success; and (iii) for research‐inactive clinicians: enablers of future research.

Methods

An online survey distributed through the Bio21 Cluster to clinicians (doctors, nurses, allied health professionals) in 15 Victorian hospitals between November 2011 and January 2012.

Results

Seven hundred and seventy of 1027 (75%) of respondents were research‐active and were more likely to be male, medical doctors, aged 45–54 years, to work full‐time and have a higher degree (all P < 0.01). Of clinicians with a higher degree, 28% were research‐inactive. Clinicians identified protected research time (50%), designated research space (42%), clinical trial coordinators (35%), institutional funding (34%) and mentoring (33%) as critical enablers of research. Research‐inactive clinicians identified protected research time as the key enabler of future research.

Conclusions

To realise recommendations in the McKeon Review, hospitals and research bodies will need to protect research time and provide space and funding. Engaging research‐inactive clinicians will build research capacity.
Early predictors of colectomy and long‐term maintenance of remission in ulcerative colitis patients treated using anti‐tumour necrosis factor therapy
Authors: Subramaniam K. , Richardson A. , Dodd J. , Platten J. , Shadbolt B. , Pavli P.
Published: February 2014

Background

Anti‐tumour necrosis factor (TNF) agents are used as induction and maintenance therapy in ulcerative colitis (UC) refractory to standard therapy and as rescue therapy in acute severe UC (ASUC).

Aims

To determine long‐term outcomes including colectomy rates, predictors of maintenance of response and remission, risk of serious adverse events by reviewing 12‐year clinical experience from a single centre in Australia.

Methods

Seventy‐one patients with moderate‐severe UC (Mayo score ≥6) (n = 52) and ASUC (n = 19) treated with anti‐TNF agents were included. Primary end‐points were colectomy at 12 weeks and colectomy‐free survival at last follow up. Secondary endpoints included clinical response (decrease in Mayo score of ≥3) and remission (Mayo score ≤2).

Results

Colectomy at 12 weeks was 1%, and colectomy‐free survival was 69%. Using full Mayo score, at 3 months, 32/37 (87%) refractory and 9/12 (75%) ASUC patients responded to anti‐TNF therapy; 19/37 (51%) refractory and 8/12 (67%) ASUC patients were in remission. Long‐term response rates (mean follow up 37.4 months) were 24/44 (55%) and 11/15 (73%) in refractory and ASUC groups respectively. Long‐term remission rates were 43% in refractory and 60% in ASUC patients. Twenty two of 71 (31%) underwent colectomy (mean time 50.4 months). Clinical non‐response at 3 months was a predictor of colectomy (hazard ratio = 9.346; P = 0.001). ASUC predicted long‐term maintenance of response (odds ratio 19.4; P = 0.013) and remission (odds ratio 6.13; P = 0.037). Two of 71 patients had serious infections.

Conclusions

Anti‐TNF therapy is effective in both refractory and ASUC. We argue that early anti‐TNF therapy may improve outcome in UC.
Multilocus phylogeography and morphology give insights into the recent evolution of a Mexican endemic songbird:Vireo hypochryseus
Authors: Arbeláez‐Cortés Enrique , Roldán‐Piña Diego , Navarro‐Sigüenza Adolfo G.
Published: February 2014

We used multilocus phylogeographic analyses, morphometric measurements, and environmental niche models (ENMs) to analyze the recent evolution of the golden vireoVireo hypochryseus, a Mexican endemic species.Vireo hypochryseusis made up of two phylogeographically structured mitochondrial DNA clades that probably diverged 132 000 yr ago. One clade comprised individuals from mainland Sinaloa and the Tres Marías islands in the northwest, and the other included individuals from the remaining range of the species. This marked phylogeographic structure contrasts with the low genetic structure reported for temperate North American vireos. The nuclear DNA markers also showed some geographic differences in allele frequency, but did not exhibit a clear phylogeographic structure. The morphometric analyses suggested a decreasing north to south cline, with the largest individuals located in the Tres Marías islands. The ENMs did not support a scenario of geographic fragmentation of the environmental conditions of the area in whichV. hypochryseushas inhabited over the last 130 000 yr. However, a model of isolation by resistance based on the actual configuration of climatic conditions in western Mexico did explain a major proportion of both the mitochondrial DNA distances and the differences in size, while a model of isolation by distance explain a low proportion of such differences. Therefore, the recent history ofV. hypochryseuswas likely shaped by historical habitat fragmentation due to fluctuating environmental conditions in the mainland that produced a phylogeographic print, and natural selection on morphological traits in the insular population, suggesting an active diversification of endemic lineages in the Mexican dry forest.
Body composition of north and southbound migratory blackcaps is influenced by the lay‐of‐the‐land ahead
Authors: Wojciechowski Michał S. , Yosef Reuven , Pinshow Berry
Published: February 2014

The annual migration of small birds depends on the optimal management of time and energy. Since refueling at stopovers between flights consumes most of the birds’ time and energy, selection of food‐rich sites, and timely departure therefrom are likely crucial to success. We examined this concept quantifying body composition of 200 migrating blackcaps, Sylvia atricapilla, in Eilat, Israel, using dual‐energy x‐ray absorptiometry and generated a model to predict body composition as it changes with body mass (mb). We then back‐calculated body composition of > 20 000 blackcaps ringed between 1984 and 2005, and tested the hypothesis that the amount of fuel that a bird stores determines the length of its stopover. We predicted that 1) if time‐constrained in spring, birds at the stopover site carry less than a maximum fuel load, but 2) if not time‐constrained, as in autumn, their fuel load is much higher than in spring. We found the change in body composition of blackcaps to be biphasic and correlated with increasing mb. At mb < ˜ 17.8 g, increasing mb is due to increasing lean mass (ml), while at mb > ˜ 17.8 g increasing mb results from increasing fat mass (mf), which is accompanied by decreasing ml. Body composition of blackcaps at a spring stopover site indicates that blackcaps leave stopovers as soon as they regain functionality of their digestive systems, but before laying down much mf. In autumn blackcaps arrive with fuel stores much larger than in spring. For these birds, the Eilat stopover apparently serves to complete fat accumulation before crossing the deserts ahead. We conclude that in spring, the decision to depart is not determined by the bird's fuel stores, especially when early arrival at the breeding site, and therefore time, is of the essence. In autumn, accumulating enough fuel to ensure successful crossing of the deserts ahead probably dictates stopover time.
Consistency and reciprocity of indirect interactions between tree species mediated by frugivorous birds
Authors: Martínez Daniel , García Daniel , Herrera José M.
Published: February 2014

Despite the recognized importance of indirect plant–plant interactions for community structure, we still need to improve our current knowledge on how their outcomes are consistent in space and time, as well as reciprocal between participating species. These caveats are especially relevant in the case of indirect interactions mediated by animals, whose behavior may show high variability. We studied consistency and reciprocity of frugivore‐mediated interactions between fleshy‐fruited trees. For three years we examined the influence of crop size and neighborhood characteristics (con‐ and heterospecific fruit abundance and forest cover) on frugivory rates onCrataegus monogynaandIlex aquifolium, two coexisting species in the secondary forests of the Cantabrian range that share a guild of frugivorous birds. Crop size and neighborhood characteristics influenced frugivory onC. monogynaandI. aquifolium. Both con‐ and heterospecific fruit abundance affected frugivory, evidencing the occurrence of indirect interactions between trees, although the strength and sign of these effects varied between tree species as well as across years within species. By showing complex temporal patterns in the consistency and reciprocity of indirect interactions, this study emphasizes the need for multispecific, long‐term studies to assess the actual contribution of animal‐mediated plant–plant indirect interactions to community dynamics.
Self‐organization of background habitat determines the nature of population spatial structure
Authors: Jackson Doug , Allen David , Perfecto Ivette , Vandermeer John
Published: February 2014

A substantial literature treats the dynamics of populations in response to spatial patterning of underlying habitats, the most common formulations being source/sink populations and metapopulations living in a patchwork of habitats. A separate and growing literature on the self‐organization of spatial pattern focuses on how local interactions give rise to regional patterns that can be described with scale‐free distributions. Motivated by the potential ubiquity of the coupling of these processes (spatial self organization and the dynamics of organisms in spatially structured habitats), we link these two lines of thought into a theory of populations in fragmented habitats. Using a combined analytical and computational approach, we show that self organization can generate a background into which an independent population fits, and that the scale‐free nature of such habitat creates conditions that influence both the persistence versus extinction properties of the embedded population and where it lies on the continuum between source/sink populations and metapopulations. Both the analytical framework and the computer simulations demonstrate the potential for populations to persist on either end of the metapopulation–source/sink continuum while failing to persist under intermediate conditions. These results provide a new perspective on ecological theory related to habitat fragmentation and population persistence, suggesting that under some conditions intermediate states between these two extremes may maximize risk of extinction of the population. These implications could be of particular importance for managing self‐organized systems of conservation concern.
Rapid Molten Salt Synthesis of Isotropic Negative Thermal Expansion ScF3
Authors: Hu Lei , Chen Jun , Fan Longlong , Deng Jinxia , Yu Ranbo , Xing Xianran
Published: February 2014

With a simple ReO3‐type structure, ScF3 exhibits a rare property of isotropic negative thermal expansion over a large temperature range. In this study, a rapid and low‐temperature synthesis route has been developed to prepare pure phase of ScF3 in which NaNO3 or KNO3 as reaction media and Sc(NO3)3 and NH4HF2 as precursors (i.e., 30 min and 310°C). The sample of ScF3 has relatively regular morphology and shows high crystallinity as well as single‐crystalline nature. The type of molten salts has obvious impact on morphologies of the particles. Substituting NaNO3 with KNO3, cubes of ScF3 turns to be nanosticks. The thermal stability of the as‐prepared ScF3 was investigated by thermal analysis. Molten salts play a significant role in eliminating the nonstoichiometric impurity of ScF2.76 which is a common impurity during the conventional chemical reaction. This study reveals that molten salt is in favor of preparing those fluorides and relatives which is inert with moisture.
Influence of Hydrogen Peroxide Addition on Photoluminescence of Y2O3:Eu3+ Nanophosphors Prepared by Laser Ablation in Water
Authors: Al‐Mamun Sharif Abdullah , Ishigaki Takamasa
Published: February 2014

Y2O3:Eu3+ red phosphors were synthesized by laser irradiation of Eu‐doped Y2O3 sintered targets immersed in water. The photoluminescence (PL) of the nanoparticles (NPs) was significantly enhanced by adding hydrogen peroxide to the water. Targets were synthesized using three methods, namely, sol–gel (SG), homogeneous‐precipitation (HP), and solid‐state reaction (SSR) followed by sintering. The sintered SG target contained 8.6 wt% of hexagonal oxygen‐deficient byproduct, while the other targets were composed of pure cubic phase. The amount of hexagonal oxygen‐deficient and monoclinic by‐products in the nanophosphors increased to 33 wt% and 27 wt% in irradiated SG‐ and HP‐derived targets, respectively. The SSR‐derived NPs were almost pure cubic phase. Addition of H2O2 to the water minimized the amount of hexagonal oxygen‐deficient and monoclinic byproducts (5.7 wt% and 6.4 wt% for SG‐ and HP‐derived NPs respectively). Moreover, the PL of the NPs was enhanced by the formation of a pure cubic phase, and the optimum amount of added H2O2 for maximum luminescence was 5 wt%.
Low‐Temperature Phase Transition in AgNbO3
Authors: Zhang Tingsong , Zhang Chenyang , Wang Ling , Chai Yisheng , Shen Shipeng , Sun Young , Yuan Hongming , Feng Shouhua
Published: February 2014

AgNbO3 is a weak ferroelectric with antiferroelectricity due to Ag displacements at room temperature. A dielectric anomaly at 250 K, which has not been observed previously, reveals a transition between the weak ferroelectric phase (M1 phase) at the higher temperature and a new ferroelectric phase (M0 phase) at the lower temperature in AgNbO3. This transition was further verified by the pyroelectric current and differential scanning calorimetry measurements. The spontaneous polarization value is found to be much larger in the M0 phase than that of the M1 phase. A well‐defined saturating ferroelectric hysteresis loop can also be observed at 77 K, showing a remnant polarization value of 2.4 μC/cm2 and a coercive field of 25 kV/cm. All the above results indicate that the larger polarization of the M0 phase mainly comes from the alignment of the antiferroelectric displacements of the Ag atoms.
Intergroup friendship and children's intentions for social exclusion in integrative classrooms: the moderating role of teachers' diversity beliefs
Authors: Grütter Jeanine , Meyer Bertolt
Published: February 2014

We examine children's intentions to socially exclude other children with special educational needs in integrative classrooms. Based on developmental and diversity research, we hypothesize that intergroup friendship leads to lower intentions to socially exclude. We also examine the moderating role of teachers' diversity beliefs on the proposed relationship. We obtained social network and questionnaire measures from 439 primary school pupils from 20 school classes, and analyzed them with multilevel modeling. In support of the moderation hypothesis, results show that intergroup friendship was only associated with lower intentions for social exclusion if teachers saw value in diversity for their class. The results offer important insights into intergroup contact theory and its use for designing implications for integrative education.
Phylogeny of the Poorly Known Ciliates, Microthoracida, a Systematically Confused Taxon (Ciliophora), with Morphological Reports of Three Species
Authors: Fan Xinpeng , Pan Hongbo , Li Lifang , Jiang Jiamei , Al‐Rasheid Khaled A. S. , Gu Fukang
Published: February 2014

Three species of Microthoracids, Lopezoterenia paratorpens n. sp., Trochiliopsis australis Foissner et al., 1988 and Discotricha papillifera Tuffrau, 1954, collected from Chinese coastal waters, were investigated using live observation and protargol staining methods. Lopezoterenia paratorpens n. sp. was characterized by its squarely shaped cortical papillae and by dorsal kineties which contained loosely distributed basal bodies. Trochiliopsis australis was revealed to have two oral membranelles, which was not recorded in the original report. Phylogenetic analyses were carried out based on SSU rRNA gene sequence data from each of the three species, and on other available data for microthoracids. The results showed that the order Microthoracida is not monophyletic because the family Discotrichidae, which contains L. paratorpens and D. papillifera, forms a clade separated from the “core” Microthoracids clade. The topologies of the maximum likelihood and Bayesian inference trees, along with the distinct morphological characteristics found previously, suggest that the family Discotrichidae should not be assigned to the order Microthoracida. We propose to designate a new order, Discotrichida n. ord. which diagnosed as: flattened ciliates with conspicuous cortical papillae on both dorsal and ventral faces, rod‐shaped mucocysts, and an asymmetric cytopharyngeal basket. Also, the fact that Leptopharynx clusters with the assemblage including T. australis, and Pseudomicrothorax is located distantly from Leptopharynx indicates that the classification of Pseudomicrothoracidae and Microthoracidae by Foissner (1985) is justified.
Dopamine denervation of the prefrontal cortex increases expression of the astrocytic glutamate transporter GLT‐1
Authors: Vollbrecht Peter J. , Simmler Linda D. , Blakely Randy D. , Deutch Ariel Y.
Published: February 2014

Both dopamine and glutamate are critically involved in cognitive processes such as working memory. Astrocytes, which express dopamine receptors, are essential elements in the termination of glutamatergic signaling: the astrocytic glutamate transporter GLT‐1 is responsible for > 90% of cortical glutamate uptake. The effect of dopamine depletion on glutamate transporters in the prefrontal cortex (PFC) remains unknown. In an effort to determine if astrocytes are a locus of cortical dopamine–glutamate interactions, we examined the effects of chronic dopamine denervation on PFC protein and mRNA levels of glutamate transporters. PFC dopamine denervation elicited a marked increase in GLT‐1 protein levels, but had no effect on levels of other glutamate transporters; high‐affinity glutamate transport was positively correlated with the extent of dopamine depletion. GLT‐1 gene expression was not altered. Our data suggest that dopamine depletion may lead to post‐translational modifications that result in increased expression and activity of GLT‐1 in PFC astrocytes.

image

The glutamate transporter GLT‐1 is expressed by astrocytes, which also express dopamine receptors. Regulation of prefrontal cortical (PFC) GLT‐1 potentially offers a novel treatment approach to the cognitive deficits of schizophrenia. Partial PFC dopamine deafferentation increased membrane expression of GLT‐1 protein and glutamate uptake, but did not alter levels of the other two neocortical glutamate transporters, GLAST and EAAC1.
Susceptibility Weighted Imaging Features of Nonketotic Hyperglycemia: Unusual Cause of Hemichorea‐Hemiballismus
Authors: Atay Musa , Yetis Huseyin , Kurtcan Serpil , Aralasmak Ayse , Alkan Alpay
Published: February 2014

Nonketotic hyperglycemia has been described as a metabolic cause of Hemiballism‐hemichorea (HB‐HC), especially in elderly patients with poorly controlled diabetes. Pathophysiology is not known yet. MRI features tend to be hyperintense in the putamen on T1‐weighted images. We present conventional MRI with diffusion weighted imaging (DWI) and susceptibility weighted imaging (SWI) features of two cases with nonketotic hyperglycemia. T1 hyperintensity without diffusion restriction on DWI and minimal putaminal hypointensity without phase shift on SWI were compatible with either pathological mineralization or petechial microhemorrhage or protein denaturation. In the type 2 diabetic patients with HC‐HB, conventional MRI together with SWI and DWI will guide to clinician to plan treatment approach.
High‐flow nasal cannula oxygen therapy for infants with bronchiolitis: Pilot study
Authors: Mayfield Sara , Bogossian Fiona , O'Malley Lee , Schibler Andreas
Published: February 2014

Aim

To obtain data on the safety and clinical impact of managing infants with bronchiolitis on the ward with high‐flow nasal cannula (HFNC) treatment.

Methods

A prospective pilot study was conducted of 61 infants aged <12 months with bronchiolitis and oxygen requirement presenting to the emergency department. HFNC was commenced at 2 L/kg/min, and fraction of inspired oxygen was titrated to oxygen saturation > 94%. A standard‐treatment group (n = 33) managed with standard low‐flow subnasal oxygen during the same time period was retrospectively identified.

Results

Admission demographics, heart rate (HR) and respiratory rate (RR) were similar in test and standard‐treatment groups. Responders and non‐responders to HFNC were identified within 60 min of treatment. Non‐responders to HFNC requiring paediatric intensive care unit (PICU) admission showed no change in HR and RR, whereas responders showed decreases in HR and RR (P < 0.02). Patients receiving HFNC were four times less likely to need PICU admission than the standard treatment group (OR 4.086, 95%CI 1.0–8.2; P = 0.043). No adverse events such as pneumothorax, bradycardia, bradypnoea, emergency intubation or cardiopulmonary resuscitation were observed. No patients admitted to the PICU required intubation.

Conclusions

HFNC treatment in the paediatric ward is safe. Non‐responders requiring PICU admission can be identified within the first hour of HFNC treatment by monitoring HR and RR. It is feasible to undertake a randomised controlled trial based on this pilot with the aim of decreasing PICU admissions.
Utility of peritoneal dialysis in neonates affected by inborn errors of metabolism
Authors: Bilgin Leyla , Unal Sevim , Gunduz Mehmet , Uncu Nermin , Tiryaki Tugrul
Published: February 2014

Aim

Some inborn errors of metabolism induce metabolic encephalopathy through accumulation of neurotoxic metabolites. Rapid elimination of these metabolites by peritoneal or extracorporeal dialysis is crucial to prevent neuronal damage or death. In this retrospective study, we evaluated the outcomes of nine neonates with metabolic crisis treated with peritoneal dialysis.

Method

Six neonates with hyperammonemic coma (four with organic acidemias, two with urea cycle disorders) and three with leucine accumulation due to maple syrup urine disease (MSUD) were managed with peritoneal dialysis in conjunction with dietary and pharmacological therapy.

Results

Three patients with organic acidemia survived. One of the patients was normal; others had moderate and severe neurological impairments. One neonate with organic acidemia and both neonates with urea cycle disorders died. Two of the three patients withMSUDsurvived without neurological impairment; the other had severe neurological damage and died at 9 months of age due to sepsis.

Conclusion

Theoretically, extracorporeal dialysis should be the first dialysis treatment of choice; however, this report demonstrates that peritoneal dialysis has a chance to prevent neurological damage in some patients. Therefore, in developing countries without extracorporeal dialysis opportunities, it can be still a life‐saving procedure, if it is applied with skilled staff and standard procedures.
Paediatricians' perceptions of a potential online psychosocial intervention for children with recurrent abdominal pain
Authors: Boixadós Mercè , Hernández Encuentra Eulàlia , Nieto Luna Ruben , Huguet Anna , Aumatell Eva
Published: February 2014

Aims

To evaluate paediatricians' perceived effectiveness of an online psychosocial intervention for children with recurrent abdominal pain (RAP). Also, to explore which elements of this intervention they would consider necessary when applied in the clinical context.

Methods

A total of 131 paediatricians affiliated to Catalan and Balearic paediatric societies completed an online survey about how effective they would perceive an online psychosocial intervention for RAP (for reducing pain intensity, reducing disability and preventing chronicity) and how this intervention should be carried out. They were asked about the perceived effectiveness of the standard medical treatments they routinely applied for RAP and also their opinion of face‐to‐face psychosocial interventions.

Results

A face‐to face psychosocial intervention was considered better (to reduce pain intensity, reduce disability and prevent chronic pain) than an online psychological intervention and the standard medical treatment. Online and face‐to‐face psychosocial interventions are considered equally useful for children with mild disability, but a face‐to‐face psychosocial intervention is considered better for those with moderate and severe levels of disability. Paediatricians considered that an online psychosocial intervention for children with RAP should be simple and consistent; it should provide easy access for users; and its interface should be easy to use and attractive.

Conclusions

Paediatricians show a positive attitude towards a potential online psychosocial intervention for children and adolescents with RAP. However, they do not use the Internet for offering health care, and they would prefer a face‐to‐face psychosocial intervention.
A sixth personality domain that is independent of the Big Five domains: The psychometric properties of the HEXACO Personality Inventory in a Japanese sample
Authors: Wakabayashi Akio
Published: February 2014

We investigated the reliability of the HEXACO‐Personality Inventory (HEXACO‐PI) using the NEO Five‐Factor Inventory (NEO‐FFI) and the International Personality Item Pool (IPIP) Big Five scales in a Japanese sample of 492 respondents (mean age = 20.4 years). The psychometric properties of the HEXACO‐PI were examined and found to be satisfactory. The results of the factor analysis showed that the six hypothesized personality domains were found independently. A total of 307 participants responded to the NEO‐FFI and the IPIP Big Five scales. The results showed that the five domains of the HEXACO model corresponded to the Five‐Factor domains and were relative to the Big Five personality domains as a whole, but the sixth domain, Honesty‐Humility, showed weak to moderate correlations with some factors of the NEO‐FFI and the IPIP Big Five scales. These results suggest that the Honesty‐Humility domain is independent of the five fundamental personality factors and that the HEXACO‐PI is a useful and reliable questionnaire for investigating personality structure in cross‐cultural and other context.
Fish oil supplementation increases concentration of adiponectin in healthy dogs
Authors: Mazaki‐Tovi M. , Abood S. K. , Schenck P. A.
Published: February 2014

OBJECTIVE

To determine the effect of fish oil supplementation on circulating concentrations of adiponectin, leptin, insulin, glucose, triglyceride and cholesterol in healthy dogs.

METHODS

Twenty healthy adult dogs were administered 220 mg/kg of a fish oil supplement once daily for 30 days. At baseline, on supplement and 10 to 20 weeks off supplement, dogs were examined, body condition scores determined (range: 4 to 6), body measurements recorded for % body fat calculation and fasted blood samples collected.

RESULTS

Serum concentrations of the measured individual and total n‐3 polyunsaturated fatty acids increased following supplementation (P<0·001). Mean serum adiponectin concentration on supplement was 3·4 µg/mL(95% confidence interval: 0·8 to 6·0; P=0·006) higher than baseline, and 5·3 µg/mL(2·0 to 8·7; P<0·001) higher than off supplement. Concentrations of adiponectin off supplement were not different from baseline. There were no significant differences in weight, body condition scores, % body fat and concentrations of other measured analytes between baseline and on supplement.

CLINICAL SIGNIFICANCE

Fish oil supplementation significantly increased circulating concentration of adiponectin in healthy non‐obese dogs. Further investigation is warranted to determine whether this effect may be extended to obese dogs and to evaluate the potential role of fish oil supplementation in the management of disorders associated with low circulating adiponectin concentrations.
An Increased Arginase Activity Is Associated with Corpus Cavernosum Impairment Induced by Hypercholesterolemia
Authors: Fraga-Silva Rodrigo A. , Costa-Fraga Fabiana P. , Faye Younoss , Sturny Mikaël , Santos Robson A.S. , da Silva Rafaela F. , Stergiopulos Nikolaos
Published: February 2014

Introduction

Hypercholesterolemia is a prevalent risk factor for the development of erectile dysfunction (ED), mostly due to an increase in oxidative stress and impaired nitric oxide (NO) bioavailability within the penis. Arginase is an enzyme that shares the common substrate L-arginine with NO synthase. Augmented arginase activity reduces NO production and is associated with ED development. However, the contribution of arginase hyperactivity in hypercholesterolemia-induced ED is unknown.

Aim

In the present study, we investigated the activity and role of arginase in the corpus cavernosum of hypercholesterolemic mice.

Methods

Apolipoprotein E (ApoE) gene-deleted mice fed with a Western-type diet for 11 weeks were treated with the selective arginase inhibitor, N-ω-Hydroxy-L-norarginine (NOHA), or vehicle (saline 0.9%) during the last 9 weeks. Arginase activity and expression were measured in penis protein extraction. Reactive oxygen species (ROS) content within the corpus cavernosum was measured by dihydroethidium staining. Functional in vitro studies were performed using cavernosal strips mounted in an isometric organ bath to evaluate NO production.

Main Outcome Measure

Arginase activity and its role in modulating NO and ROS production within the corpus cavernosum of hypercholesterolemic mice is the main outcome measure.

Results

Total arginase activity and arginase type II protein expression were increased in hypercholesterolemic mice compared with wild-type mice. The long-term treatment with NOHA normalized this alteration. Moreover, pharmacological arginase inhibition by NOHA attenuated the augmented ROS production within the corpus cavernosum of ApoE−/− mice, which increased the NO-dependent response in cavernosal strips.

Conclusion

These evidences indicate that arginase hyperactivity is associated with ED induced by hypercholesterolemia, suggesting that this enzyme is a potential target for treating ED.
Boceprevir and telaprevir‐based triple therapy for chronic hepatitis C: virological efficacy and impact on kidney function and model for end‐stage liver disease score
Authors: Virlogeux V. , Pradat P. , Bailly F. , Funingana G. , Gonçalves F. , Maynard M. , Hartig‐Lavie K. , Amiri M. , Zoulim F.
Published: February 2014

Triple therapy using telaprevir or boceprevir [hepatitis C virus (HCV)‐NS3/NS4A protease inhibitors (PI)] in association with PEG‐IFN/ribavirin has recently become the new standard of care (SOC) for treatment of HCV genotype 1 patients. Our objective was to assess the efficacy and tolerance of triple therapy in routine clinical practice. A total of 186 consecutive HCV patients initiating triple therapy were enrolled in a single centre study. Clinical, biological and virological data were collected at baseline and during follow‐up as well as tolerance and side effect details. Among 186 HCV patients initiating triple therapy, 69% received telaprevir and 31% boceprevir. Sixty‐one per cent of patients had cirrhosis. The overall extended rapid virological response (eRVR) rate and sustained virological response (SVR) rate were 57.0% and 59.7%, respectively. IL28B CC phenotype was associated with increased probability of achieving eRVR and SVR, whereas previous non‐response was associated with low eRVR and SVR rates. The SVR rate increased from 30.8% in previously non‐responders to 59.1% in partial non‐responders and 75% in relapsers. SVR rate in naive patients was 62.5%. Glomerular filtration rate assessed by MDRD after 12 weeks of therapy was significantly reduced for both PI (P < 0.001). The model for end‐stage liver disease (MELD) score was significantly increased at W12 for telaprevir (P = 0.008) and at W24 for boceprevir (P = 0.027). PI‐based triple therapy leads to high rates of virological response even in previously non‐responder patients. Renal function after triple therapy is impaired as well as MELD score in all patients. Cautious clinical monitoring should focus not only on haematological and dermatological side effects but also on renal function.
Specialists may thrive in small habitats: the case of high genetic diversity within a confined gecko population
Authors: Peled E. , Ben‐Shlomo R. , Shanas U.
Published: February 2014

Maintaining a meta‐population structure significantly contributes to species viability and is often the basis for defining the difference between a naturally patchy and a fragmented landscape. However, a heterogeneous landscape may be patchy for habitat generalists and fragmented for specialists, preventing the formation of meta‐population structures in habitat specialists. We examined this hypothesis on the generalist Lichtenstein's short‐fingered geckoStenodactylus sthenodactylusand the endangered specialist Middle Eastern short‐fingered geckoS. doriae, two species of the genusStenodactylus, inhabiting the southernAravaValley inIsrael. We compared the genetic structure of the populations of these two geckos by amplified fragment length polymorphism analysis, expecting to find decreased gene diversity within the small populations that fail to form a meta‐population structure. Indeed, we found that among populations, the habitat specialistS. doriaehad a low level of gene flow, whereas the habitat generalistS. sthenodactylushad a relatively high level of gene flow. However, unexpectedly, the most isolated population of the specialistS. doriae, located in theSamar dune (a small patch of 2.3 km2), exhibited the highest level of gene diversity of all the populations studied (expected heterozygosity = 0.4286). Moreover, the results showed that theSamar population is genetically unique when compared with its neighboring populations. Gene flow between two populations located to the north and to the south bypass theSamar population. The generalistS. sthenodactylus, in contrast, did not exhibit an exceptional level of gene diversity. The origin of the exceptional diversity and genetic uniqueness of theSamar population ofS. doriaemay be associated with traits that make this gecko highly adaptive to this specific landscape unit. It also emphasizes the need to establish special conservation efforts for the protection of high‐quality habitats that provide adequate conditions for a source population of specialist species.
The Acquisition of Polysynthetic Languages
Authors: Kelly Barbara , Wigglesworth Gillian , Nordlinger Rachel , Blythe Joseph
Published: February 2014

One of the major challenges in acquiring a language is being able to use morphology as an adult would, and thus, a considerable amount of acquisition research has focused on morphological production and comprehension. Most of this research, however, has focused on the acquisition of morphology inisolatinglanguages, or languages (such as English) with limited inflectional morphology. The nature of the learning task is different, and potentially more challenging, when the child is learning apolysyntheticlanguage – a language in which words are highly morphologically complex, expressing in a single word what in English takes a multi‐word clause. To date, there has been no cross‐linguistic survey of how children approach this puzzle and learn polysynthetic languages. This paper aims to provide such a survey, including a discussion of some of the general findings in the literature regarding the acquisition of polysynthetic systems.
Soil and geography are more important determinants of indigenous arbuscular mycorrhizal communities than management practices in Swiss agricultural soils
Authors: Jansa Jan , Erb Angela , Oberholzer Hans‐Rudolf , Šmilauer Petr , Egli Simon
Published: February 2014

Arbuscular mycorrhizal fungi (AMF) are ubiquitous soil fungi, forming mutualistic symbiosis with a majority of terrestrial plant species. They are abundant in nearly all soils, less diverse than soil prokaryotes and other intensively studied soil organisms and thus are promising candidates for universal indicators of land management legacies and soil quality degradation. However, insufficient data on how the composition of indigenous AMF varies along soil and landscape gradients have hampered the definition of baselines and effect thresholds to date. Here, indigenous AMF communities in 154 agricultural soils collected across Switzerland were profiled by quantitative real‐time PCR with taxon‐specific markers for six widespread AMF species. To identify the key determinants of AMF community composition, the profiles were related to soil properties, land management and site geography. Our results indicate a number of well‐supported dependencies between abundances of certain AMF taxa and soil properties such as pH, soil fertility and texture, and a surprising lack of effect of available soil phosphorus on the AMF community profiles. Site geography, especially the altitude and large geographical distance, strongly affected AMF communities. Unexpected was the apparent lack of a strong land management effect on the AMF communities as compared to the other predictors, which could be due to the rarity of highly intensive and unsustainable land management in Swiss agriculture. In spite of the extensive coverage of large geographical and soil gradients, we did not identify any taxon suitable as an indicator of land use among the six taxa we studied.
Regulation of hematopoiesis by activators and inhibitors of Wnt signaling from the niche
Authors: Schreck Christina , Bock Franziska , Grziwok Sandra , Oostendorp Robert A.J. , Istvánffy Rouzanna
Published: February 2014

Hematopoietic stem cells (HSCs) are a rare population of somatic stem cells that have the ability to regenerate the entire mature blood system in a hierarchical way for the duration of an adult life. Adult HSCs reside in the bone marrow niche. Different niche cell types and molecules regulate the balance of HSC dormancy and activation as well as HSC behavior in both normal and malignant hematopoiesis. Here, we describe the interplay of HSCs and their niche, in particular the involvement of the Wnt signaling pathway. Although the prevailing notion has been that malignant transformation of HSCs is the main cause of leukemia, evidence is mounting that disruption of niche regulation by transformed hematopoietic cells, which may overexpress Wnt signaling or intrinsic stromal defects in gene expression, is at least a collaborative factor in leukemogenesis. Thus, insights into the normal and altered functions of niche components will help to obtain a better understanding of normal and malignant hematopoiesis and how environmental factors affect these processes.
Transgenic expression of the rice Xa21 pattern‐recognition receptor in banana (Musa sp.) confers resistance to Xanthomonas campestris pv. musacearum
Authors: Tripathi Jaindra N. , Lorenzen Jim , Bahar Ofir , Ronald Pamela , Tripathi Leena
Published: February 2014

Banana Xanthomonas wilt (BXW), caused by the bacterium Xanthomonas campestris pv. musacearum (Xcm), is the most devastating disease of banana in east and central Africa. The spread of BXW threatens the livelihood of millions of African farmers who depend on banana for food security and income. There are no commercial chemicals, biocontrol agents or resistant cultivars available to control BXW. Here, we take advantage of the robust resistance conferred by the rice pattern‐recognition receptor (PRR), XA21, to the rice pathogen Xanthomonas oryzae pv. oryzae (Xoo). We identified a set of genes required for activation of Xa21‐mediated immunity (rax) that were conserved in both Xoo and Xcm. Based on the conservation, we hypothesized that intergeneric transfer of Xa21 would confer resistance to Xcm. We evaluated 25 transgenic lines of the banana cultivar ‘Gonja manjaya’ (AAB) using a rapid bioassay and 12 transgenic lines in the glasshouse for resistance against Xcm. About 50% of the transgenic lines showed complete resistance to Xcm in both assays. In contrast, all of the nontransgenic control plants showed severe symptoms that progressed to complete wilting. These results indicate that the constitutive expression of the rice Xa21 gene in banana results in enhanced resistance against Xcm. Furthermore, this work demonstrates the feasibility of PRR gene transfer between monocotyledonous species and provides a valuable new tool for controlling the BXW pandemic of banana, a staple food for 100 million people in east Africa.