bluuebunny/crossref_metadata_2025_split huggingface dataset:
Resourcing for a resilient post‐disaster reconstruction environment
Authors: Chang Yan , Wilkinson Suzanne , Seville Erica , Potangaroa Regan 
Published: February 2010

PurposeThe purpose of this paper is to understand the resourcing issues that concern the provision of resources required for reconstruction projects after a disaster and to enable them to be integrated into a holistic planning process.Design/methodology/approachTriangulation methodology is adopted in this paper including both quantitative and qualitative methods. The quantitative approach, namely statistic analysis with the aid of questionnaires and SPSS is employed to identify the key factors affecting resource availability in post‐disaster reconstruction situations. The qualitative semi‐structured interviews and desk reviews of government and media documents are conducted to further interpret outcomes in the questionnaire session.FindingsBased on empirical research, the major finding of the paper is that in order to arrive at a resilient and sustainable built environment after a disaster, resourcing efforts should be made around four components – resourcing facilitator: legislation and policy; resourcing implementer: construction industry; resourcing platform: construction market; and resourcing access: transportation system.Originality/valueThe original part of this paper is in raising the importance of resourcing for achieving a resilient post‐disaster built environment, and in presenting a thorough overhaul of the resourcing components. The paper also offers a vision of comprehensive planning and preparedness to facilitate resourcing operations in post‐disaster reconstruction; pinpoints possible constraints inherent in post‐disaster resourcing environment; and provides a direction‐setting framework to achieve the vision with built environment resilience considerations incorporated.
An examination of decision making in post disaster housing reconstruction
Authors: Hayles Carolyn S. 
Published: February 2010

PurposeThe purpose of this paper is to explore the key challenges facing non‐governmental organisations (NGOs) during decision making in post disaster housing reconstruction.Design/methodology/approachAn extensive literature review was undertaken to establish the key challenges facing disaster management practitioners. Following this review, practitioners from leading NGOs were asked to discuss their experiences of post disaster housing reconstruction including the issues of hazard risk and appropriate mitigation including increasing vulnerability due to climate change, end‐users and stakeholder consultation in planning and design, technological solutions, and constructing the build; as these were identified in the literature as the principal challenges being faced by practitioners in the pursuit of sustainable construction.FindingsThe research results presented in this paper provide clear insights into the decision‐making practices of these NGOs and establish where improvements need to be made. The results also establish that knowledge management activities need to be more focused to ensure that lessons learnt previously are implemented elsewhere; critical in hazard mitigation and meeting the challenges of increased vulnerability due to climate change.Research limitations/implicationsThe paper includes recommendations for improved knowledge transfer and dissemination of “lessons learnt” in order to capture knowledge gained on projects. Future research will build on this through a detailed examination of the project management process applied to recently completed case studies. Key intervention points in the life cycle of projects will be identified and anticipated knowledge requirements for each stage mapped.Originality/valueInterviews with practitioners reporting first hand on the challenges they face in the field. Useful for those supporting practitioners through research and development as well as donor organisations as it has been revealed that a lot of issues arise as a result of the way projects are funded.
Review of phenotypic markers used in flow cytometric analysis of MGUS and MM, and applicability of flow cytometry in other plasma cell disorders
Authors: Raja Karthick R. M. , Kovarova Lucie , Hajek Roman 
Published: March 2010

SummaryFlow cytometric immunophenotyping is considered an indispensable tool for the diagnosis, classification and monitoring of disease in monoclonal gammopathies. The clinical sensitivity of flow cytometry is comparable with advanced molecular methods. Clinical application of flow cytometry in monoclonal gammopathies has various dimensions, such as differential diagnosis of malignant plasma cell disorder from reactive plasmacytosis, identifying the progression risk in monoclonal gammopathy of undetermined significance (MGUS) and asymptomatic multiple myeloma (MM), and minimal residual disease detection. Flow cytometry‐based clonality assessment with immunophenotyping encourages and enables the most stringent method of diagnosis and follow‐up. The objective of this review is to update the malignant plasma cells phenotypic profile of MGUS and MM. The most comprehensive antigens, such as CD19, CD27, CD28, CD45, CD56 and CD117, play a significant role in the characterization of normal and malignant plasma cells. Several research groups described the putative phenotype of myeloma cell progenitors, but no remarkable suggestion could be made because of disparity. This review also focuses on the association of malignant phenotypic markers and chromosomal aberrations that identify the specific prognostic features in monoclonal gammopathies.
Asparaginase‐related venous thrombosis in UKALL 2003‐ re‐exposure to asparaginase is feasible and safe
Authors: Qureshi Amrana , Mitchell Chris , Richards Sue , Vora Ajay , Goulden Nick 
Published: March 2010

SummaryWe report the incidence and outcome of venous thrombosis (VT) in the UK acute lymphoblastic leukaemia (ALL) 2003 trial. VT occurred in 59/1824 (3·2%) patients recruited over 5 years with 90% occurring during a period of Asparagine depletion. Pegylated Escherichia Coli Asparaginase (Peg‐ASP) 1000 units/m2 was used throughout. Thirty‐four children received further Peg‐ASP, most with concurrent heparin prophylaxis. There were no episodes of bleeding or recurrent thrombosis. Optimal Asparagine depletion is central to success of modern regimes for treatment of ALL. This report confirms a significant risk of thrombosis with such therapy, but demonstrates that re‐exposure to Asparaginase is feasible and safe.
Ant nest location, soil nutrients and nutrient uptake by ant‐associated plants: does extrafloral nectar attract ant nests and thereby enhance plant nutrition?
Authors: Wagner Diane , Fleur Nicklen E. 
Published: March 2010

Summary1.As central place foragers, ants accumulate organic debris near their nests. Consequently, soil nutrient stocks are often enriched near the nest site. We investigated the hypothesis that plant‐derived food sources, such as extrafloral nectar (EFN), can encourage soil‐dwelling ant colonies to nest near the plant, thereby inadvertently providing the plant with an additional source of mineral nutrients. The study focused on a population ofAcacia constricta, a North American shrub bearing EFNs.2.Several lines of evidence supported the notion that food rewards drew ant nests close toA. constrictaplants. Firstly, ant species that visit EFNs nested significantly closer toA. constrictaplants than would be expected by chance, whereas this was not the case for two ant species that do not visit EFNs. Secondly,A. constrictaplants with an ant nest occurring naturally underneath the canopy had greater foliar volume, more EFNs per leaf and more EFNs per cm of leaf rachis than plants lacking an ant nest under the canopy. Thirdly, experimental supplementation of the nectar resources on acacias led to the establishment of significantly more new nests near the plant, relative to controls. However, nectar supplementation did not affect acacia seed production within the year of the study.3.Soil from the nests of three, EFN‐visiting ant species contained higher average stocks of most mineral nutrients than nearby soils outside the influence of the nest.4.To test whetherA. constrictacan assimilate the nutrients in ant nests, we fed15N‐labelled food toDorymyrmexsp. (smithicomplex) workers nesting near acacias. Twenty‐four days later, the leaves of acacias with an experimentally fed ant colony under the canopy contained significantly higher15N and %N than acacias without a nest under the canopy, indicating that acacias assimilated and benefited from nutrients derived from ants.5.Synthesis.The results indicate that nectar resources can attract the nests of some ant species, and that plants may benefit from access to soil nutrients derived from ant nests. Our data support the hypothesis that EFNs may confer nutritive, as well as protective, benefits.
Complex interplay between the LysR‐type regulator AtzR and its binding site mediates atzDEF activation in response to two distinct signals
Authors: Porrúa Odil , Platero Ana Isabel , Santero Eduardo , Del Solar Gloria , Govantes Fernando 
Published: March 2010

SummaryAtzR is a LysR‐type regulator responsible for activation of the cyanuric acid utilization operon atzDEF. AtzR binds the PatzDEF promoter region at a strong recognition element, designated the repressor binding site, and a weaker binding determinant, the activator binding site (ABS). AtzR activates transcription in response to two dissimilar signals, nitrogen limitation and cyanuric acid. In the present work we analyse the structure and function of the cis‐acting elements involved in AtzR activation of atzDEF. Hydroxyl radical footprinting assays revealed that the ABS is composed of three functional subsites spaced at one helix‐turn intervals. Two modes of interaction with the ABS are detected in vitro: AtzR binds at the ABS‐2 and ABS‐3 subsites in the absence of inducer, and relocates to interact with the ABS‐1 and ABS‐2 subsites in the presence of cyanuric acid. In vivo mutational analysis indicates that ABS‐1 and ABS‐2 are required for full PatzDEF activation in all conditions. In contrast, ABS‐3 acts as a ‘subunit trap’ that hinders productive AtzR interactions with ABS‐1 and ABS‐2. Our results strongly suggest an activation model in which cyanuric acid and nitrogen limitation cooperate to reposition AtzR from an inactive, ABS‐3 bound configuration to an active, ABS‐1‐ and ABS‐2‐bound configuration.
Genetics professionals' experiences with grief and loss: implications for support and training
Authors: Geller G , Rushton CH , Francomano C , Kolodner K , Bernhardt BA 
Published: February 2010

Geller G, Rushton CH, Francomano C, Kolodner K, Bernhardt BA. Genetics professionals' experiences with grief and loss: implications for support and training.This study was designed to determine the degree to which clinical genetics professionals are comfortable with grief and loss, whether discomfort with grief and loss is associated with clinician distress, and what factors predict comfort with grief and loss for the purpose of developing recommendations for support and training. We surveyed 300 clinical geneticists (MDs), genetic counselors (GCs) and genetic nurses randomly selected from their professional associations. Out of 225 eligible clinicians, 172 completed surveys (76% response rate). The vast majority of respondents have clinical interactions with patients and families who are experiencing grief, loss and/or death. However, nearly 20% of respondents reported that they did not feel ‘comfortable in the presence of grief and loss'. Twenty‐nine percent of respondents disagree or strongly disagree that they ‘have been adequately trained to address issues of death, dying, grief/bereavement, and end of life care’. Reported discomfort with grief and loss was strongly correlated with clinician distress. Predictors of comfort with grief and loss included perceived adequacy of training, tolerance for uncertainty, significant personal experiences of loss and deriving meaning from patient care. In conclusion, as follows. A significant minority of clinical genetics professionals experience discomfort in the presence of grief and loss, and feel inadequately prepared for such experiences. Greater attention should be paid to training clinicians in how to deal with grief and loss, and supporting them through such difficult experiences in an effort to reduce their distress.
Refining the phenotype associated with MEF2C haploinsufficiency
Authors: Novara F , Beri S , Giorda R , Ortibus E , Nageshappa S , Darra F , Dalla Bernardina B , Zuffardi O , Van Esch H 
Published: February 2010

Novara F, Beri S, Giorda R, Ortibus E, Nageshappa S, Darra F, dalla Bernardina B, Zuffardi O, Van Esch H. Refining the phenotype associated with MEF2C haploinsufficiency.Recently, submicroscopic deletions of the 5q14.3 region have been described in patients with severe mental retardation (MR), stereotypic movements, epilepsy and cerebral malformations. Further delineation of a critical region of overlap in these patients pointed to MEF2C as the responsible gene. This finding was further reinforced by the identification of a nonsense mutation in a patient with a similar phenotype. In brain, MEF2C is essential for early neurogenesis, neuronal migration and differentiation. Here we present two additional patients with severe MR, autism spectrum disorder and epilepsy, carrying a very small deletion encompassing the MEF2C gene. This finding strengthens the role of this gene in severe MR, and enables further delineation of the clinical phenotype.
Novel splice‐site mutations and a large intragenic deletion in PLA2G6 associated with a severe and rapidly progressive form of infantile neuroaxonal dystrophy
Authors: Tonelli A , Romaniello R , Grasso R , Cavallini A , Righini A , Bresolin N , Borgatti R , Bassi MT 
Published: February 2010

Tonelli A, Romaniello R, Grasso R, Cavallini A, Righini A, Bresolin N, Borgatti R, Bassi MT. Novel splice‐site mutations and a large intragenic deletion in PLA2G6 associated with a severe and rapidly progressive form of infantile neuroaxonal dystrophy.Infantile neuroaxonal dystrophy, INAD, is a severe progressive psychomotor disorder with infantile onset and characterized by the presence of axonal spheroids throughout the central and peripheral nervous systems. A subset of INAD patients shows also brain iron accumulation which represents instead the distinctive feature of the idiopathic neurodegeneration with brain iron accumulation, NBIA. These diseases share the same causative gene, PLA2G6, encoding iPLA2‐VIA, a calcium‐independent phospholipase. Mutations that lead to a complete absence of protein are associated with a severe INAD profile, while compound heterozygous mutations with possibly a residual protein activity are instead associated with the less severe NBIA phenotype. Here we describe two INAD patients both with an unusually rapid disease progression and a peculiar neuroradiological presentation in one of them. Compound heterozygosity for a large intragenic deletion and a nonsense mutation was found in one of them while the other is carrying two novel splice‐site mutations. Breakpoint‐sequence analysis suggests a non‐allelic‐homologous‐recombination (NAHR) event, probably underlying the rearrangement. These findings, while supporting the genotype–phenotype correlation already observed in INAD patients, provide the first sequence characterization of a genomic rearrangement in PLA2G6 gene, thus orienting the search for missing mutant alleles in PLA2G6 related diseases.
Parental origin of apparently balanced de novo complex chromosomal rearrangements investigated by microdissection, whole genome amplification, and microsatellite‐mediated haplotype analysis
Authors: Grossmann V , Höckner M , Karmous‐Benailly H , Liang D , Puttinger R , Quadrelli R , Röthlisberger B , Huber A , Wu L , Spreiz A , Fauth C , Erdel M , Zschocke J , Utermann G , Kotzot D 
Published: February 2010

Grossmann V, Höckner M, Karmous‐Benailly H, Liang D, Puttinger R, Quadrelli R, Röthlisberger B, Huber A, Wu L, Spreiz A, Fauth C, Erdel M, Zschocke J, Utermann G, Kotzot D. Parental origin of apparently balanced de novo complex chromosomal rearrangements investigated by microdissection, whole genome amplification, and microsatellite‐mediated haplotype analysis.Complex chromosomal rearrangements (CCRs) are rare findings in clinical cytogenetics. As a result of the high risk of unbalanced segregation, familial cases are even rarer and maternal transmission occurs more frequently than paternal transmission. Analogous to Drosophila and mice, as well as to CCRs involving the Y chromosome or a clinically relevant associated deletion, a preferential origin in spermatogenesis has been assumed but not proven directly and systematically thus far. Here, we investigated three healthy adults, one healthy child, and one child with multiple congenital anomalies and various balanced de novo CCRs. The analyses were performed in each case on 10 copies of a derivative chromosome and their normal homologs by glass‐needle microdissection, whole genome amplification (WGA), and microsatellite‐mediated haplotype analysis. With respect to the number of chromosomes involved in each case and in all cases together, the number of chromosomal segments in each case and in all cases together, and the number of breakpoints in each case and in all cases together, the conformity for paternal origin of all derivative chromosomes and maternal origin of their normal homologs makes formation in paternal germline more likely than a postzygotic formation with an accidental uniformity. In conclusion, our results confirm the preferential formation of de novo balanced CCRs in the paternal germline.
Evolution of reproductive effort in viscous populations: the importance of population dynamics
Authors: LION S. 
Published: March 2010

AbstractI present two ecological models for the evolution of reproductive effort in viscous populations with empty sites. In contrast with previous studies, I show that limited dispersal needs not have a positive effect on the evolutionarily stable allocation of resources to fecundity versus survival. Rather, depending on the feedback between the trait and the population dynamics, population viscosity may have no effect or even lead to a decrease in the evolutionarily stable reproductive effort when individuals can degrade their environment during their lifetime. I show that the different evolutionary outcomes can be explained by the asymmetry in the level of kin competition resulting from investing into juveniles or into adults.
Clinical management of short children with low serum immunoglobulin but no immunodeficiency features
Authors: Meazza Cristina , Cerutti Paola , Pagani Sara , Boncimino Agata , Marconi Massimo , Avanzini Antonia , Bozzola Mauro 
Published: March 2010

AbstractBackground:  In children of different ages investigated for failure to thrive, low (below the cut‐off for age) immunoglobulin (Ig) values can be detected, without any clinical evidence of humoral immunodeficiencies. To better characterize infants presenting with diminished immunoglobulin levels, we studied IgG subclasses, in vitro Ig production and B cell subpopulation.Methods:  We monitored 17 children (12 boys and five girls, age range 1–18 years) with low serum levels of one or more Ig isotypes but without any clinical or laboratory features of immunodeficiency.Results:  Low IgM levels were frequent (52.9%). During the follow up, six of 17 cases (35.3%) normalized their immunoglobulin levels. Frequently, in the observed patients, low levels of immunoglobulins were not limited to the period of infancy. In all patients, in vitro Ig production and B lymphocyte subpopulations were within normal ranges.Conclusions:  We suggest a quantification of serum Ig levels in children who fail to thrive in order to identify patients with low Ig levels. These children should be monitored until Ig levels normalize to exclude any immunodeficiency status. Early recognition of children with persistent hypogammaglobulinemia would allow prompt and appropriate clinical interventions.
A systematic review and meta‐analysis of tumor necrosis factor α‐308 polymorphism and Kawasaki disease
Authors: Arj‐Ong Sakda , Thakkinstian Ammarin , McEvoy Mark , Attia John 
Published: March 2010

AbstractBackground:  There have been genetic studies assessing the association between tumor necrosis factor (TNF)‐α‐308 and Kawasaki disease (KD) but the results have been conflicting due to lack of power. Therefore, a systematic review and meta‐analysis was conducted to increase the power for identifying the association between the TNF‐α‐308 polymorphism and KD.Method:  Studies were identified from MEDLINE and EMBASE databases and were included if the subjects were children and the frequencies between TNF‐α‐308 and KD were reported. Data were pooled using a random effect model if heterogeneity between studies was present.Results:  Thirteen studies were identified however only six studies were included. The pooled prevalence of minor A allele was 5.2% (95% confidence interval [CI]: 0.1%–9.5%). Gene effect was assessed using per‐allele and per‐genotype approaches. The pooled odds ratio of G versus A with the random effect model was 1.13 (95%CI: 0.34–3.27). The genotype effects for GG versus GA+AA was estimated and the pooled odds ratio was 1.08 (95%CI: 0.42–2.92).Conclusion:  This review suggests a trend of association between the TNF‐α‐308 G‐allele and KD. However, the gene effects are heterogeneous and assessing sources of heterogeneity are limited. An updated meta‐analysis is needed if more studies are published.
Impact of tension‐free vaginal tape procedure on dysfunctional voiding in women with stress urinary incontinence
Authors: Xu Dan‐Feng , Qu Chuang‐Yu , Ren Ji‐Zhong , Jiang Hai‐Hong , Yao Ya‐Cheng , Min Zhi‐Lian , Zhu You‐Hua , Chao Lv 
Published: March 2010

Objectives:  To determine the prevalence of dysfunctional voiding (DV) in female stress urinary incontinence (SUI) and its modification after tension‐free vaginal tape (TVT) procedure.Methods:  Three hundred and sixty women with SUI were enrolled and underwent urodynamics from 2002 to 2008. DV was determined when non‐neurogenic detrusor‐sphincter dyssynergia occurred during voluntary voiding. It was further quantitatively analyzed using the tense/loose value, a parameter derived from external anal sphincter electromyogram. The distribution of other urodynamic variables was also evaluated. One hundred and fifty patients underwent the TVT procedure and forty of them were studied with urodynamics after surgery during follow up.Results:  Overall, DV was diagnosed in ninety‐nine patients, with a prevalence of 27.5%. The functional profile length in SUI women with DV was significantly shorter than that in SUI women without DV (3.13 ± 0.76 vs 3.32 ± 0.65, P = 0.017). After the TVT procedure, the recovery of SUI between cases with and without DV showed no significant difference. The rate of DV state change after the surgery, namely from with to without DV or from without to with DV, significantly differed between the female patients with and without DV (66.7% vs 3.6%, P < 0.05) during follow up. The DV improved after the surgery in SUI women with DV.Conclusions:  DV might represent a coexistent finding in women with SUI. The main difference of women with SUI and DV, as compared with those without DV, is a shortened functional profile length. In such cases, TVT procedure can improve DV along with the treatment of SUI.