bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cancer complicating systemic lupus erythematosus – a dichotomy emerging from a nested case-control study
Authors: Dey D , Kenu E , Isenberg DA 
Published: July 2013

Objectives We determined whether any individual cancers are increased or decreased in a cohort of 595 patients with systemic lupus erythematosus (SLE) followed for up to 32 years at the University College London Hospitals Lupus Clinic, looking for any associated clinical or serological factors and the prognosis after cancer diagnosis. Methods We undertook a careful retrospective review of the medical records and identified all individuals diagnosed with cancer. For controls, we selected three other patients in the cohort who had not developed cancer, carefully matched for age, sex, ethnicity and disease duration, to determine if any obvious differences emerged in a nested case-control design. Results Thirty-three patients developed cancer after being diagnosed with SLE. There was a statistically insignificant small increase in overall cancer risk, standardized incidence ratios (SIRs) 1.05 (95% CI 0.52–1.58) and increased SIRs for cervical, prostate, anal and pancreatic cancers and reduction in breast cancer SIRs. Haematological and musculoskeletal manifestations, anticardiolipin and antithyroid globulin antibodies were found to be positively associated with cancer risk in multivariate analysis. There was no drug, dose or duration was associated with cancer risk. There was a reduction in survival with a cancer fatality rate of 84.2% ( p < 0.0001). Conclusion We found a very small but statistically insignificant increased cancer risk with reduction in survival. Whereas some cancers appear to be more common in SLE, notably prostate and cervical cancer, others, particularly breast cancer, are less frequent. Multiple clinical and serological factors are involved in the increased risk of malignancy in SLE. No drug dose or duration effect was identified.
Young foster children and their carers: An approach to assessing relationships
Authors: Mares Sarah , Torres Marianne 
Published: July 2013

Objective: This article outlines an approach to assessing the quality of relationships between young foster children and their carers. These children are at high risk of disorganised attachment relationships and of developmental psychopathology given their relational experiences prior to and in care. During a semi-structured play interaction the emphasis is on identifying behaviours of clinical interest. This can be complex given the likelihood of atypical or unexpected behaviours expressed within relationships. Method: The paper draws on literature on the clinical application of attachment theory to the assessment of relationships and on the authors’ experience of developing and delivering an assessment and intervention service for children aged 0 to 5-years-old within a mental health service for children in foster care. Clinical material is used to illustrate and develop the issues. Conclusion: The case for including a semi-structured observational procedure as part of a comprehensive assessment of foster children and their carers is outlined. This is argued to have more clinical utility than formal approaches to attachment classification. The benefits of including a semi-structured and relational approach to clinical assessment of foster children are outlined along with the need to be cautious in the use of attachment related terminology when formal assessments have not been undertaken.
Maternal visibility at the commodity frontier: Weaving love into birthday party consumption
Authors: Jennings Laura , Brace-Govan Jan 
Published: July 2013

Children's birthday parties are celebrations that offer a means to understand consumption values surrounding motherhood that can remain understated or even taken for granted. Mother's consumption activities and the involvement of their child can reveal their management of the commodity frontier. As the marketplace increasingly provides goods and services to support mothers and parenting, the extent to which this is acceptable is a source of anxiety for the enactment of ‘good’ mothering in some social groups. The maternal visibility that birthday parties demand adds further strain. Building from previous work that acknowledges mothers to be the organisers of family celebrations and work that identifies ideologies of motherhood as important in the enactment, or performance, of mothering, this study examined mother–child consumption and the management of the commodity frontier in the preparation and hosting of young children's birthday parties. The findings show that mothers could publicly demonstrate their intimate knowledge and care for their child by personalising the birthday party through the gift of their time and effort to create a ‘homemade’ event. Although overt commercialisation was managed, do-it-yourself understates the need to consume the constituent elements of the celebration. Instead mothers' abiding care was woven into the party to express something special for the child without material excess. Effortful work, time and emotional energy were all understated in the creation of a memorable event for the child. The underpinning rationalisation asserted that the fun, innocent simplicity of childhood was an imperative. The involvement of the child in consumption for the party allowed the mother to gauge the child's pleasure, teach values and manage the commodity frontier.
Endovascular Versus Medical Therapy for Uncomplicated Type B Aortic Dissection
Authors: Merola Jonathan , Garg Karan , Adelman Mark A. , Maldonado Thomas S. , Cayne Neal S. , Mussa Firas F. 
Published: July 2013

Background: Uncomplicated type B dissections have been traditionally managed with antihypertensive therapy. In the endovascular era, this dictum has been revisited. This review pooled the available studies to compare the outcomes of best medical therapy (BMT) to thoracic endovascular aortic repair (TEVAR) for uncomplicated type B dissections. Methods: A literature search was performed to identify studies on uncomplicated type B dissections managed with BMT with and without TEVAR. The primary outcome measures were mortality rates at 30 days and at 2 years following intervention. Results: A total of 6 studies included 123 patients who underwent TEVAR/BMT, and 566 patients who had BMT alone. The mortality rates at 30 days (6.5% TEVAR/BMT vs 4.8% BMT, P = .21) and at 2 years (9.7% vs 11.9%, P = .32) were similar. Renal failure was greater in TEVAR/BMT (15.4% vs 2.1%, P < .01). Rates of surgical reintervention/intervention were similar (17.6% vs 20.1%, P = .31). Conclusion: The TEVAR with BMT does not provide survival benefit compared to BMT alone, 2 years following uncomplicated type B aortic dissection.
Popliteal Artery Entrapment Syndrome
Authors: Altintas Ümit , Helgstrand Ulf V. J. , Hansen Marc A. , Stentzer Kim F. , Schroeder Torben V. , Eiberg Jonas P. 
Published: July 2013

Objectives: The purpose of this study was to report our experience with popliteal artery entrapment syndrome (PAES) with special emphasis on the applicability of duplex ultrasound scanning (DUS) when diagnosing PAES. In addition to examining the correlation between DUS and intraoperative findings in symptomatic limbs, the ultrasonic effect of plantar flexion in healthy volunteers were also evaluated. Methods: During a 12-month period, 11 symptomatic limbs in 8 patients with a mean age of 29 years were referred with suspected PAES and enrolled consecutively. The popliteal artery was studied preoperatively with DUS in rest and during active plantar flexion. The popliteal artery was explored in all symptomatic limbs, and the intraoperative findings served as gold standard. Additionally, the popliteal arteries in 11 healthy volunteers (22 limbs) were evaluated with DUS during rest and plantar flexion. Results: Intraoperative findings confirmed PAES in all 11 symptomatic limbs in accordance with the preoperative DUS examination. Surgical release of the popliteal artery was performed in 11 limbs. At a median follow-up of 15 months, all 11 limbs were free of ischemic symptoms and regained normalized popliteal flow on DUS. In the 22 symptom-free limbs, DUS showed normal popliteal flow during both rest and plantar flexion. Conclusions: In this series of patients with surgically confirmed PAES, we found preoperative DUS to have perfect agreement with the intraoperative findings in diagnosing PAES. The applicability of the method seems to be emphasized by the restoration of popliteal flow and relief of arterial insufficiency after surgical release in all patients, and by the fact, that none of the healthy volunteers were able to compress the popliteal artery during plantar flexion.
Reflexive design of the recursive space of virtual worlds
Authors: Smith David Harris 
Published: July 2013

This article considers the intersection of urbanism and digital culture to theorize a model of mediated urbanism and its application to a designed virtual world for artistic and scientific collaboration. Historical precedents informing the current research include artist networks of the late 1960s and early 1970s that sought to bridge and democratize scientific, technological, and artistic endeavors. The author proposes that mediated urbanism in the reflexive recursive space of virtual worlds references and reworks embodied forms of urbanism and fosters the development of new urbanite forums. The awareness of the nature and history of this articulation may be productively used to inform the design of these emerging virtual forums of knowledge production and artistic creation.
The effect of rituximab on vaccine responses in patients with immune thrombocytopenia
Authors: Nazi Ishac , Kelton John G. , Larché Mark , Snider Denis P. , Heddle Nancy M. , Crowther Mark A. , Cook Richard J. , Tinmouth Alan T. , Mangel Joy , Arnold Donald M. 
Published: July 2013

Key PointsAfter treatment with rituximab, immunological responses to both polysaccharide and conjugated vaccines are impaired in patients with ITP. Splenectomized patients who received rituximab may be at increased risk of infection because of compromised immune responses to vaccines.
Immune gene expression profiling of Proliferative Kidney Disease in rainbow trout Oncorhynchus mykiss reveals a dominance of anti-inflammatory, antibody and T helper cell-like activities
Authors: Gorgoglione Bartolomeo , Wang Tiehui , Secombes Christopher J , Holland Jason W 
Published: July 2013

AbstractThe myxozoanTetracapsuloides bryosalmonaeis the causative agent of Proliferative Kidney Disease (PKD) targeting primarily the kidney of infected fish where it causes a chronic lymphoid immunopathology. Although known to be associated with suppression of some cellular aspects of innate immunity and a prominent lymphocytic hyperplasia, there remains a considerable knowledge gap in our understanding of the underlying immune mechanisms driving PKD pathogenesis. To provide further insights, the expression profiles of a panel of innate / inflammatory and adaptive immune molecules were examined in rainbow troutOncorhynchus mykissfollowing a natural exposure to the parasite. Relative to controls, fish with early to advanced stages of kidney pathology exhibited up-regulation of the inflammatory cytokines interleukin (IL)-6 and IL-11, although remaining refractory towards genes indicative of macrophage activity. Antimicrobial peptides (AMPs) and anti-inflammatory markers, including cathelicidin (CATH) and IL-10 were markedly up-regulated during clinical disease. Up-regulation of adaptive immune molecules, including cell markers and antibody genes reflect the lymphocytic dominance of this disease and the likely importance of lymphocyte subsets in PKD pathogenesis. Up-regulation of T helper (TH) cell-like response genes and transcription factors implies thatT.bryosalmonaemay elicit a complex interplay between THcell subsets. This work, for the first time in the study of fish-myxozoan interactions, suggests that PKD pathogenesis is shaped by an anti-inflammatory phenotype, a profound B cell / antibody response and dysregulated THcell-like activities. A better understanding of the functional roles of fish immune cells and molecules in PKD pathogenesis may facilitate future development of control measures against this disease.
MicroRNAome profiling in benign and malignant neurofibromatosis type 1-associated nerve sheath tumors: evidences of PTEN pathway alterations in early NF1 tumorigenesis
Authors: Masliah-Planchon Julien , Pasmant Eric , Luscan Armelle , Laurendeau Ingrid , Ortonne Nicolas , Hivelin Mikael , Varin Jennifer , Valeyrie-Allanore Laurence , Dumaine Valérie , Lantieri Laurent , Leroy Karen , Parfait Béatrice , Wolkenstein Pierre , Vidaud Michel , Vidaud Dominique , Bièche Ivan 
Published: July 2013

Abstract

Background
Neurofibromatosis type 1 (NF1) is a common dominant tumor predisposition syndrome affecting 1 in 3,500 individuals. The hallmarks of NF1 are the development of peripheral nerve sheath tumors either benign (dermal and plexiform neurofibromas) or malignant (MPNSTs).

Results
To comprehensively characterize the role of microRNAs in NF1 tumorigenesis, we analyzed 377 miRNAs expression in a large panel of dermal and plexiform neurofibromas, and MPNSTs. The most significantly upregulated miRNA in plexiform neurofibromas was miR-486-3p that targets the major tumor suppressor gene, PTEN. We confirmed PTEN downregulation at mRNA level. In plexiform neurofibromas, we also report aberrant expression of four miRNAs involved in the RAS-MAPK pathway (miR-370, miR-143, miR-181a, and miR-145). In MPNSTs, significant deregulated miRNAs were involved in PTEN repression (miR-301a, miR-19a, and miR-106b), RAS-MAPK pathway regulation (Let-7b, miR-195, and miR-10b), mesenchymal transition (miR-200c, let-7b, miR-135a, miR-135b, and miR-9), HOX genes expression (miR-210, miR-196b, miR-10a, miR-10b, and miR-9), and cell cycle progression (miR-195, let-7b, miR-20a, miR-210, miR-129-3p, miR-449a, and miR-106b).

Conclusion
We confirmed the implication of PTEN in genesis of plexiform neurofibromas and MPNSTs in NF1. Markedly deregulated miRNAs might have potential diagnostic or prognostic value and could represent novel strategies for effective pharmacological therapies of NF1 tumors.
Natural variation in floral nectar proteins of two Nicotiana attenuata accessions
Authors: Seo Pil Joon , Wielsch Natalie , Kessler Danny , Svatos Ales , Park Chung-Mo , Baldwin Ian T , Kim Sang-Gyu 
Published: July 2013

AbstractBackgroundFloral nectar (FN) contains not only energy-rich compounds to attract pollinators, but also defense chemicals and several proteins. However, proteomic analysis of FN has been hampered by the lack of publically available sequence information from nectar-producing plants. Here we used next-generation sequencing and advanced proteomics to profile FN proteins in the opportunistic outcrossing wild tobacco,Nicotiana attenuata.ResultsWe constructed a transcriptome database ofN. attenuataand characterized its nectar proteome using LC-MS/MS. The FN proteins ofN. attenuataincluded nectarins, sugar-cleaving enzymes (glucosidase, galactosidase, and xylosidase), RNases, pathogen-related proteins, and lipid transfer proteins. Natural variation in FN proteins of elevenN. attenuataaccessions revealed a negative relationship between the accumulation of two abundant proteins, nectarin1b and nectarin5. In addition, microarray analysis of nectary tissues revealed that protein accumulation in FN is not simply correlated with the accumulation of transcripts encoding FN proteins and identified a group of genes that were specifically expressed in the nectary.ConclusionsNatural variation of identified FN proteins in the ecological model plantN. attenuatasuggests that nectar chemistry may have a complex function in plant-pollinator-microbe interactions.
Elongator subunit 3 positively regulates plant immunity through its histone acetyltransferase and radical S-adenosylmethionine domains
Authors: DeFraia Christopher T , Wang Yongsheng , Yao Jiqiang , Mou Zhonglin 
Published: July 2013

AbstractBackgroundPathogen infection triggers a large-scale transcriptional reprogramming in plants, and the speed of this reprogramming affects the outcome of the infection. Our understanding of this process has significantly benefited from mutants that display either delayed or accelerated defense gene induction. In our previous work we demonstrated that the Arabidopsis Elongator complex subunit 2 (AtELP2) plays an important role in both basal immunity and effector-triggered immunity (ETI), and more recently showed that AtELP2 is involved in dynamic changes in histone acetylation and DNA methylation at several defense genes. However, the function of other Elongator subunits in plant immunity has not been characterized.ResultsIn the same genetic screen used to identifyAtelp2, we found another Elongator mutant,Atelp3-10, which mimicsAtelp2in that it exhibits a delay in defense gene induction following salicylic acid treatment or pathogen infection. Similarly to AtELP2, AtELP3 is required for basal immunity and ETI, but not for systemic acquired resistance (SAR). Furthermore, we demonstrate that both the histone acetyltransferase and radicalS-adenosylmethionine domains of AtELP3 are essential for its function in plant immunity.ConclusionOur results indicate that the entire Elongator complex is involved in basal immunity and ETI, but not in SAR, and support that Elongator may play a role in facilitating the transcriptional induction of defense genes through alterations to their chromatin.
Dystonia, facial dysmorphism, intellectual disability and breast cancer associated with a chromosome 13q34 duplication and overexpression of TFDP1: case report
Authors: Moscovich Mariana , LeDoux Mark S , Xiao Jianfeng , Rampon Garrett L , Vemula Satya R , Rodriguez Ramon L , Foote Kelly D , Okun Michael S 
Published: July 2013

Abstract

Background
Dystonia is a movement disorder characterized by involuntary sustained muscle contractions causing twisting and repetitive movements or abnormal postures. Some cases of primary and neurodegenerative dystonia have been associated with mutations in individual genes critical to the G1-S checkpoint pathway (THAP1, ATM, CIZ1 and TAF1). Secondary dystonia is also a relatively common clinical sign in many neurogenetic disorders. However, the contribution of structural variation in the genome to the etiopathogenesis of dystonia remains largely unexplored.

Case presentation
Cytogenetic analyses with the Affymetrix Genome-Wide Human SNP Array 6.0 identified a chromosome 13q34 duplication in a 36 year-old female with global developmental delay, facial dysmorphism, tall stature, breast cancer and dystonia, and her neurologically-normal father. Dystonia improved with bilateral globus pallidus interna (GPi) deep brain stimulation (DBS). Genomic breakpoint analysis, quantitative PCR (qPCR) and leukocyte gene expression were used to characterize the structural variant. The 218,345 bp duplication was found to include ADPRHL1, DCUN1D2, and TMCO3, and a 69 bp fragment from a long terminal repeat (LTR) located within Intron 3 of TFDP1. The 3' breakpoint was located within Exon 1 of a TFDP1 long non-coding RNA (NR_026580.1). In the affected subject and her father, gene expression was higher for all three genes located within the duplication. However, in comparison to her father, mother and neurologically-normal controls, the affected subject also showed marked overexpression (2×) of the transcription factor TFDP1 (NM_007111.4). Whole-exome sequencing identified an SGCE variant (c.1295G > A, p.Ser432His) that could possibly have contributed to the development of dystonia in the proband. No pathogenic mutations were identified in BRCA1 or BRCA2.

Conclusion
Overexpression of TFDP1 has been associated with breast cancer and may also be linked to the tall stature, dysmorphism and dystonia seen in our patient.
Brain natriuretic peptide is not predictive of dilated cardiomyopathy in Becker and Duchenne muscular dystrophy patients and carriers
Authors: Schade van Westrum Steven , Dekker Lukas , de Haan Rob , Endert Erik , Ginjaar Ieke , de Visser Marianne , van der Kooi Anneke 
Published: July 2013

Abstract

Background
Cardiomyopathy is reported in Duchenne and Becker muscle dystrophy patients and female carriers. Brain Natriuretic peptide (BNP) is a hormone produced mainly by ventricular cardiomyocytes and its production is up regulated in reaction to increased wall stretching. N-terminal-proBNP (NT-proBNP) has been shown to be a robust laboratory parameter to diagnose and monitor cardiac failure, and it may be helpful to screen for asymptomatic left ventricular dysfunction. Therefore we tested whether NT-proBNP can distinguish patients with Duchenne or Becker muscular dystrophy patients and carriers of a dystrophin mutation with a dilated cardiomyopathy from those without.

Methods
In a cohort of Duchenne and Becker muscle dystrophy patients (n = 143) and carriers (n = 219) NT-proBNP was measured, and echocardiography was performed to diagnose dilated cardiomyopathy (DCM).

Results
In total sixty-one patients (17%) fulfilled the criteria for DCM, whereas 283 patients (78%) had an elevated NT-pro BNP. The sensitivity of NT-proBNP for DCM in patients or carriers was 85%, the specificity 23%, area under the ROC-curve = 0.56. In the specified subgroups there was also no association.

Conclusion
Measurement of NT-pro BNP in patients suffering from Duchenne or Becker muscular dystrophy and carriers does not distinguish between those with and without dilated cardiomyopathy.
Improvement of quality of life in methadone treatment patients in northern Taiwan: a follow-up study
Authors: Chou Ying-Chun , Shih Shu-Fang , Tsai Wei-Der , Li Chiang-shan R , Xu Ke , Lee Tony Szu-Hsien 
Published: July 2013

Abstract

Background
This study examined long-term improvement of quality of life amongst heroin users enrolled in methadone maintenance treatment (MMT).

Methods
The sample contained 553 heroin-dependent individuals from 4 hospitals in northern Taiwan who enrolled in MMT for an average of 184 days. Each patient signed a consent form and was assessed prospectively 3 times semi-annually. Quality of life was measured using the WHOQOL-BREF questionnaire, 26 items of which were scored by the participants. The WHOQOL-BREF consists of four domains: physical, psychological, social, and environmental. 285 and 155 participants completed 6-month and 12-month follow-ups respectively.

Results
After controlling for demographic and clinical characteristics, there were statistically significant improvements in the psychological and environmental domains between baseline and 6 months. Significant improvements were found in psychological and social domains between baseline and 12 months.

Conclusions
It is concluded that methadone maintenance treatment improves heroin users’ long-term quality of life in the psychological and social relationship domains.