bluuebunny/crossref_metadata_2025_split huggingface dataset:
Pachyonychia Congenita: A Spectrum of KRT6a Mutations in Australian Patients
Authors: Forrest Charlotte E. , Casey Genevieve , Mordaunt Dylan A. , Thompson Elizabeth M. , Gordon Lynne 
Published: April 2016

AbstractBackgroundPachyonychia congenita (PC) is a rare inherited disorder of keratinization characterised by hypertrophic nail dystrophy, painful palmoplantar blisters, cysts, follicular hyperkeratosis and oral leukokeratosis. It is associated with mutations in five differentiation‐specific keratin genes, KRT6A, KRT6B, KRT6C, KRT16, or KRT17.ObjectivesLiving with Pachyonychia Congenita can be isolating. The aim of this paper is to document a single patient's experience within a national context.MethodWe report the case of a 2 year old female with an atypical presentation of PC due to a mutation in KRT6A with severely hypertrophic follicular keratoses, skin fragility, relative sparing of nail hypertrophy on one hand and failure to thrive in early infancy. In collaboration with the International Pachyonychia Congenita Research Registry (IPCRR), a database search was performed using Australian residency and KRT6A mutation as inclusion criteria. The IPCRR database was also searched for a matching KRT6A mutation. Six Australian patients were identified in addition to one patient with an identical mutation residing in the United States. The detailed standardized patient questionnaire data was manually collated and analysed.ResultsFingernail hypertrophy and oral leukokeratosis were the most common features. There was no recording of asymmetric distribution in any other Australian patient. Trouble nursing as an infant and follicular hyperkeratosis also occurred in the American patient, however they did not have asymmetric distribution and the oral leukokeratosis appeared later in life.ConclusionThis case has unique features. Sharing information can assist patients navigating life with this condition.
Diaper Dermatitis: A Review of 63 Children
Authors: Ersoy‐Evans Sibel , Akıncı Hande , Doğan Sibel , Atakan Nilgün 
Published: April 2016

AbstractBackground/ObjectiveDiaper dermatitis (DD) is a common infantile dermatosis with a highly variable prevalence and incidence. This study aimed to present the demographic and clinical features of babies with DD.MethodsData, including demographic and clinical features, obtained from DD patient forms were retrospectively analyzed.ResultsThe study included 63 babies with DD (female: n = 35 [55.6%]; male: n = 28 [44.4%]; mean age 11.6 mos). Most of the patients (43.5%) were 0 to 6 months of age. In all, 76.3% were breastfed, of whom 31.7% were exclusively breastfed. The number of previous episodes of DD was significantly lower in breastfed babies. The most common diaper area cleansing method was wet wipes (54.1%). Diaper creams were used in 86.2% of patients. DD was severe in 22.2% of patients, moderate in 57.4%, and mild in 20.4%. Candida infection was noted in 77.4% of patients. The median number of previous episodes of DD was significantly higher in patients with Candida infection than in those without (p = 0.02). Treatment yielded greater than 50% improvement (complete and moderate response) in 59.4% of patients at the first follow‐up visit (3–5 days) and 82.6% at the second follow‐up visit (10 days).ConclusionMost babies with DD were 0 to 6 months of age. Breastfed babies had fewer previous episodes of DD, so mothers should be encouraged to breastfeed their babies to reduce the incidence of DD. Candida infection was common; clinicians should be aware of its association with DD.
Narrow endemics on coastal plains: Miocene divergence of the critically endangered genus Avellara (Compositae)
Authors: Fernández‐Mazuecos M. , Jiménez‐Mejías P. , Martín‐Bravo S. , Buide M. L. , Álvarez I. , Vargas P. 
Published: April 2016

AbstractCritically endangered species representing ancient, evolutionarily isolated lineages must be given priority when allocating resources for conservation projects. Sound phylogenetic analyses and divergence time estimations are required to detect them, and studies on their population genetics, ecological requirements and breeding system are needed to understand their evolutionary history and to design efficient conservation strategies. Here we present the paradigmatic case of Avellara, a critically endangered monotypic genus of Compositae inhabiting a few swamps in the west–southwest Iberian coastal plains. Our phylogenetic and dating analyses based on nuclear (ITS) and plastid (matK) DNA sequences support a Miocene (>8.6 Ma) divergence between Avellara and closely related genera, resulting in marked morphological and ecological differentiation. We found alarmingly low levels of genetic diversity, based on AFLPs and plastid DNA sequences, and confirmed the prevalence of clonal reproduction. Species distribution modelling suggested a large macroclimatically suitable area for Avellara in the western Iberian Peninsula, but its apparently narrow microecological requirements restrict its distribution to peatlands with low‐mineralised waters. Although five populations have been recorded from Spain and Portugal in the past, its current distribution may be reduced to only one population, recurrently found in the last decade but threatened by herbivory and habitat degradation. All this confirms the consideration of Avellara as a threatened species with high phylogenetic singularity, and makes it a flagship species for plant conservation in both Spain and Portugal that should be given priority in the design of in situ and ex situ conservation programmes.
The Power of Nonprofits: Mechanisms for Nonprofit Policy Influence
Authors: Fyall Rachel 
Published: April 2016

AbstractThe dramatic increase in public funding for nonprofit organizations has raised concerns about the potential disadvantages of a nonprofit sector that is too reliant on government funding. Using nonprofits to deliver public programs also presents risks for the public sector, but the question of nonprofit policy influence is largely absent from discussions of public–nonprofit service collaborations. The motivation for this article stems from the contradiction between the perceived weakness of publicly funded nonprofits and their potential for policy influence. This research asks, how do nonprofits exert policy influence? Using a grounded theory approach, the research draws on the attitudes and experiences of professionals and elected officials involved in policy making and policy implementation in the area of low‐income housing. The findings indicate a variety of mechanisms through which the government–nonprofit relationship can strengthen the power of nonprofit organizations, sometimes while weakening their government counterparts.
Breeding and culture status of Hilsa (Tenualosa ilisha, Ham. 1822) in South Asia: a review
Authors: Sahoo Amiya Kumar , Wahab Md. Abdul , Phillips Michael , Rahman Anisur , Padiyar Arun , Puvanendran Velmurugu , Bangera Rama , Belton Ben , De Dilip Kumar , Meena Dharmendra Kumar , Behera Bijay Kumar , Sharma Anil Prakash , Bhaumik Utpal , Mohanty Bimal Prasanna , Choudhury Sampurna Roy , Mohan Chadag Vishnumurthy 
Published: April 2016

AbstractThe Hilsa shad, Tenualosa ilisha popularly known as ‘Hilsa’, is one of the most commercially important fish species in South Asian countries. The species is widely distributed from the Persian Gulf to Bay of Bengal and ascends into estuaries, rivers and brackish‐water lagoons of the Indo‐Pacific region. Recently, the availability of hilsa has drastically dwindled in aquatic systems across this region, due to anthropogenic pressures, mainly intensive fishing and river obstruction by dams and barrages. Climate change may also be contributing to the declining populations. Hence, there is an urgent need for conservation of the species. In addition, hilsa is of great cultural and economic importance among the peoples of Ganga‐Brahmaputra‐Meghna basin, with very high market demand and increasingly high prices, presently an average of around US$ 12 per kg for consumers both in India and Bangladesh. Many attempts at hilsa breeding and culture have been made across South Asia, particularly in India and Bangladesh, since the 1900s. To date, no breakthrough has been achieved in completing the life cycle in captivity. This review provides an insight into the status of artificial breeding, larval rearing and culture of this species, and identifies research gaps that need to be addressed by future aquaculture development programmes for hilsa in South Asia.
Effect of Follicle Size on In Vitro Maturation of Pre‐Pubertal Porcine Cumulus Oocyte Complexes
Authors: Töpfer D , Ebeling S , Weitzel JM , Spannbrucker AC 
Published: April 2016

ContentsVery small follicles (<3.0 mm diameter) are over‐represented on the surface of ovaries of non‐cycling pigs, and the oocytes collected from these follicles generally have reduced developmental competence in vitro. This study examined the effect of follicle size on the nuclear maturation (n = 608), the potential of parthenogenetic activation (n = 243) and the cyclic AMP (cAMP) content of pre‐pubertal porcine oocytes (n = 480). In addition, the influence of follicle size on steroid hormone synthesis was analysed. Cumulus oocyte complexes (COCs) flushed from small (2.5–4.0 mm) or large (4.5–6.0 mm) ovarian follicles were cultured for 0, 28 and 46 h. After 46 h of IVM, a greater proportion of oocytes from 4.5‐ to 6.0‐mm follicles reach metaphase II (MII) compared with those from follicles with 2.5–4.0 mm of diameter (96.1 vs 77.0%, respectively; p < 0.001). Parthenogenetic activation of oocytes from large follicles produced higher developmental rates than oocytes from large follicles (p < 0.05). At 28 h, the IVM medium with oocytes from large follicles contained significantly more 17ß‐oestradiol (E2) than the medium with oocytes from small follicles (5.55 vs 3.45 ng/ml, respectively; p < 0.05) and at 46 h, the medium with oocytes from small follicles contained significantly more progesterone (P4) than the medium with oocytes from large follicles (276.7 vs 108.2 ng/ml, respectively, p < 0.05). Porcine oocytes from large follicles have higher nuclear and cytoplasmic maturation capacities, but the differences did not appear to be cAMP‐mediated. Our findings also suggest that COCs from small follicles undergo more intensive luteinization than COCs from large follicles. The results show that oocytes from follicles with a diameter greater than 4.0 mm are more suitable for in vitro studies.
Skills, Tasks and the Scarcity of Talent in a Global Economy
Authors: Koch Michael 
Published: April 2016

AbstractThe scarcity of talent is a tremendous challenge for firms in the globalized world. This paper investigates the role of labor market imperfection in open economies for the usage of talent in the production process of firms. For this purpose, I set up a heterogeneous firms model, where production consists of a continuum of tasks that differ in complexity. Firms hire low‐skilled and high‐skilled workers to perform these tasks. How firms assign workers to tasks depends on factor prices for the two skill types and the productivity advantage of high‐skilled workers in the performance of complex tasks. I study the firms’ assignment problem under two labor market regimes, which capture the polar cases of fully flexible wages and a binding minimum wage for low‐skilled workers. Since the minimum wage lowers the skill premium, it increases the range of tasks performed by high‐skilled workers, which enhances the stock of knowledge within firms to solve complex tasks and reduces the mass of active firms. In a setting with fully flexible wages trade does not affect the firm‐internal assignment of workers to tasks. On the contrary, if low‐skilled wages are fixed by a minimum wage, trade renders high‐skilled workers a scarce resource and reduces the range of tasks performed by this skill type with negative consequences for the human capital stock within firms. In this case, trade leads to higher per‐capita income for both skill types and thus to higher welfare in the open than in the closed economy, whereas – somewhat counter‐intuitive – inequality between the two skill types decreases, as more low‐skilled workers find employment in the production process.
The SKG Mutation in ZAP‐70 also Confers Arthritis Susceptibility in C57 Black Mouse Strains
Authors: Guerard S. , Boieri M. , Hultqvist M. , Holmdahl R. , Wing K. 
Published: April 2016

AbstractVarious rodent models of arthritis are essential to dissect the full complexity of human rheumatoid arthritis (RA), a common autoimmune disease affecting joints. The SKG model of arthritis originates from a spontaneous mutation in ZAP‐70 found in a BALB/c colony. This mutation affects T cell selection due to reduced TCR signalling, which allows leakage of self‐reactive T cells from the thymus. To further expand the practical applicability of this unique model in arthritis research, we investigated the arthritogenicity of the SKG mutation in two common black mouse strains C57BL/6.Q and C57BL/10.Q and compared to BALB/c.Q. Mice retained the reduced TCR signalling characteristic of SKG.BALB/c mice, which leads to similar alteration in thymic selection. Importantly, mice also retained susceptibility to chronic arthritis after a single injection of mannan from Saccharomyces cerevisiae, with comparable prevalence and severity regardless of the genetic background. Further characterization of CD4+ T cells revealed a similar bias towards IL‐17 production and activated T cell phenotype in all SKG strains compared to respective wild type controls. Finally, transfer of SKG thymocytes conferred susceptibility to recipients, which confirm the intrinsic defect and pathogenicity of T cells. Overall, these results underline the strong impact that the W163C ZAP‐70 mutation has on T cell‐driven arthritis, and they support the use of the SKG model in black mice, which is useful for further investigations of this distinctive arthritis model to better understand autoimmunity.
Distinguishing the subjective and the objective aspects of self‐concept clarity
Authors: Guerrettaz Jean , Arkin Robert M. 
Published: April 2016

AbstractSince its introduction, self‐concept clarity has been viewed as a construct related to the structure and organization of a person's self‐concept. We argue, however, that self‐concept clarity may best be understood as a combination of subjective, metacognitive beliefs about the self‐concept and objective structure and organization. We consider the unique influences of both objective and subjective clarity and offer some suggestions and novel hypotheses for investigating this proposed distinction.
Enhancing the Conceptualization and Measurement of Implicit and Explicit Affective Evaluations: A Case Study in Disordered Eating
Authors: Woodward Halley E. , Cameron C. Daryl , Treat Teresa A. 
Published: April 2016

AbstractAffective evaluations (i.e., evaluations of affectively evocative stimuli) play important roles in many behaviors, including clinically relevant behaviors like disordered eating. Understanding automatic and controlled affective evaluations can enhance prediction and treatment of more deliberate versus more impulsive clinical behaviors, respectively. However, methodological and theoretical shortcomings cloud the implicit affective evaluation literature, particularly as applied in clinical psychology. This article aims to improve the clinical science of implicit and explicit affective evaluation by capitalizing on theoretical and methodological advancements drawn from implicit social cognition. We recommend three key enhancements to the affective evaluation literature: improve the structural comparability between indirect and direct measures, assess evaluations on both valence and arousal dimensions of affect, and carefully characterize and select stimuli. Additional improvements to analytic approaches (e.g., mixed effects modeling and process dissociation) are also recommended. Such efforts will advance our theoretical understanding of the relative contributions of automatic and controlled processes to clinically relevant affective evaluation, thereby informing assessment and improving intervention. Applying advanced methodologies of implicit cognition to clinical phenomena will also reiterate and reinforce the use of these advances in social psychology.
The Visual Guide to Morality: Vision as an Integrative Analogy for Moral Experience, Variability and Mechanism
Authors: Schein Chelsea , Hester Neil , Gray Kurt 
Published: April 2016

AbstractAnalogies help organize, communicate and reveal scientific phenomena. Vision may be the best analogy for understanding moral judgment. Although moral psychology has long noted similarities between seeing and judging, we systematically review the “morality is like vision” analogy through three elements: experience, variability and mechanism. Both vision and morality are experienced as automatic, durable and objective. However, despite feelings of objectivity, both vision and morality show substantial variability across biology, culture and situation. The paradox of objective experience and cultural subjectivity is best understood through constructionism, as both vision and morality involve the flexible combination of more basic ingredients. Specifically, both vision and morality involve a mechanism that demonstrates Gestalt, combination and coherence. The “morality is like vision” analogy not only provides intuitive organization and compelling communication for moral psychology but also speaks to debates in the field, such as intuition versus reason, pluralism versus universalism and modularity versus constructionism.
Gondwana breakup: no evidence for a Davie Fracture Zone offshore northern Mozambique, Tanzania and Kenya
Authors: Klimke Jennifer , Franke Dieter 
Published: April 2016

AbstractPlate tectonic reconstructions assume a major inactive transform fault, the Davie Fracture Zone, in the West Somali Basin, along which Madagascar is thought to have migrated southwards following Gondwana breakup in the Mesozoic. Based on the interpretation of reflection seismic data, we show that the Walu Ridge offshore Kenya and the Kerimbas Basin offshore northern Mozambique are tectonically unrelated to the southward motion of Madagascar and correlate with Late Cretaceous volcanism and inversion in Kenya and the evolution of the East African Rift System respectively. Offshore Tanzania, geophysical data do not show basement structures indicating the presence of a major transform fault. These results challenge the commonly supported transform margin concept and imply a more southerly pre‐breakup position of Madagascar within Gondwana. Opening of the West Somali Basin by SW‐propagating oblique rifting and seafloor spreading is proposed.
Blood graft cellular composition and posttransplant outcomes in myeloma patients mobilized with or without low‐dose cyclophosphamide: a randomized comparison
Authors: Valtola Jaakko , Silvennoinen Raija , Ropponen Antti , Siitonen Timo , Säily Marjaana , Sankelo Marja , Terävä Venla , Putkonen Mervi , Kuittinen Taru , Pelkonen Jukka , Mäntymaa Pentti , Remes Kari , Varmavuo Ville , Jantunen Esa 
Published: April 2016

BACKGROUNDAutologous stem cell transplantation is a standard treatment in multiple myeloma (MM). Blood grafts are usually collected after mobilization with granulocyte–colony‐stimulating factor (G‐CSF) alone or in a combination with cyclophosphamide (CY). There is limited knowledge of the possible effects of different mobilization regimens on blood graft characteristics and posttransplant outcomes.STUDY DESIGN AND METHODSThirty‐eight patients with MM were included in this study. The patients were randomly assigned at registration to mobilization with either low‐dose CY plus G‐CSF (Arm A) or G‐CSF alone (Arm B) and received three cycles of lenalidomide, bortetzomib, and dexamethasone induction. Flow cytometry analysis of lymphocyte subsets in the blood grafts after cryopreservation was performed. Hematologic and immune recovery were evaluated up to 12 months posttransplant.RESULTSThe blood grafts in Arm A contained significantly more CD34+ cells but in Arm B there was a greater proportion of CD34+CD38– cells and higher numbers of T and B lymphocytes as well as natural killer (NK) cells. The engraftment was comparable but lymphocyte count at 15 days posttransplant was higher in Arm B (0.8 × 109/L vs. 0.5 × 109/L, p = 0.033). At 3 and 6 months posttransplant the total number of NK cells was also higher in G‐CSF–mobilized patients. There was no difference in progression‐free survival between the study arms.CONCLUSIONCY plus G‐GSF yields more CD34+ cells but seems to diminish lymphocyte and NK cell counts in the grafts and hampers immune recovery after transplantation. Thus G‐CSF alone might be a preferred mobilization method due to more rapid immune recovery posttransplant.
ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 1. Structural and functional characterization in vitro
Authors: Ostertag Eric M. , Kacir Stephen , Thiboutot Michelle , Gulendran Gayathri , Zheng X. Long , Cines Douglas B. , Siegel Don L. 
Published: April 2016

BACKGROUNDAcquired thrombotic thrombocytopenia purpura (TTP) is a life‐threatening illness caused by autoantibodies that decrease the activity of ADAMTS13, the von Willebrand factor–cleaving protease. Despite efficacy of plasma exchange, mortality remains high and relapse is common. Improved therapies may come from understanding the diversity of pathogenic autoantibodies on a molecular or genetic level. Cloning comprehensive repertoires of patient autoantibodies can provide the necessary tools for studying immunobiology of disease and developing animal models.STUDY DESIGN AND METHODSAnti‐ADAMTS13 antibodies were cloned from four patients with acquired TTP using phage display and characterized with respect to genetic origin, inhibition of ADAMTS13 proteolytic activity, and epitope specificity. Anti‐idiotypic antisera raised to a subset of autoantibodies enabled comparison of their relatedness to each other and to polyclonal immunoglobulin (Ig)G in patient plasma.RESULTSFifty‐one unique antibodies were isolated comprising epitope specificities resembling the diversity found in circulating patient IgG. Antibodies directed both to the amino terminal domains and to those requiring the ADAMTS13 cysteine‐rich/spacer region for binding inhibited proteolytic activity, while those solely targeting carboxy‐terminal domains were noninhibitory. Anti‐idiotypic antisera raised to a subset of antibody clones crossreacted with and reduced the inhibitory activity of polyclonal IgG from a set of unrelated patients.CONCLUSIONSAnti‐ADAMTS13 autoantibodies isolated by repertoire cloning display the diversity of epitope specificities found in patient plasma and provide tools for developing animal models of acquired TTP. Shared idiotypes of inhibitory clones with circulating IgG from multiple patients suggest common features of pathogenic autoantibodies that could be exploited for developing more targeted therapies.