bluuebunny/crossref_metadata_2025_split huggingface dataset:
Dramatic response to nivolumab in xeroderma pigmentosum skin tumor
Authors: Chambon Fanny , Osdoit Sophie , Bagny Kelly , Moro Anne , Nguyen Jacqueline , Réguerre Yves 
Published: October 2017

AbstractWe report the case of a 6‐year‐old female with xeroderma pigmentosum (XP) who developed a nonoperable scalp tumor, treated with anti‐programmed cell death protein 1 (anti‐PD‐1) therapy (nivolumab). She presented with a sarcomatoid carcinoma of the scalp with bone lysis as well as vascular and meningeal contact. Nivolumab was initiated because it has emerged as a promising immunotherapy. We observed a dramatic tumor response with excellent tolerance. However, while on nivolumab therapy she developed two large skin melanomas and several squamous cell carcinomas, which have been resected. These results demonstrate that cancer immunotherapy in patients with XP can be impressive but complex and warrants further investigation.
Comparison of two cytoreductive regimens for αβ‐T‐cell‐depleted haploidentical HSCT in pediatric malignancies: Improved engraftment and outcome with TBI‐based regimen
Authors: Jacoby Elad , Varda‐Bloom Nira , Goldstein Gal , Hutt Daphna , Churi Chaim , Vernitsky Helly , Toren Amos , Bielorai Bella 
Published: October 2017

AbstractBackgroundGraft manipulation using selective depletion of αβ‐T cells provides a source of haploidentical hematopoietic stem cell transplantation (haplo‐HSCT) enriched in effector cells. We report our experience implementing this haplo‐HSCT for high‐risk malignancies in pediatric patients focusing on the conditioning regimen.ProcedureWe performed a retrospective study of patients who underwent T‐cell receptor αβ‐depleted haplo‐HSCT for high‐risk pediatric malignancies.ResultsEighteen patients underwent haplo‐HSCT using this method. The initial reduced‐toxicity chemotherapy‐based conditioning regimen was given to eight patients, and resulted in a high rate of graft rejections (six of eight patients). Thus, total body irradiation (TBI) based regimen was introduced in the following 10 patients and resulted in engraftment in all patients. Neutrophil and platelet engraftment were rapid (median time to engraft, 10 days and 12 days, respectively). Significant treatment‐related complications for both cohorts were all due to graft failure in patients receiving chemotherapy‐based conditioning, with a treatment‐related mortality rate of 17%. None of the patients developed hepatic sinusoidal‐obstruction syndrome, and no grade III–IV acute graft versus host disease (GVHD) was observed. The majority of patients were free of immunosuppression in the first 100 days post‐HSCT, and only two patients developed chronic GVHD. The cumulative incidence of relapse was 39%. Compared to patients conditioned with chemotherapy, patients conditioned with TBI had superior actuarial overall survival (66% vs. 37%, P = 0.05) and event‐free survival (61% vs. 33%, P = 0.04).ConclusionsA TBI‐based conditioning for haplo‐HSCT using αβ‐T‐cell depletion for malignant diseases ensured engraftment and resulted in acceptable outcomes.
Triple therapy of vincristine, bleomycin and etoposide for children with Kaposi sarcoma: Results of a study in Malawian children
Authors: Macken Marita , Dale Helen , Moyo Dominic , Chakmata Eunice , Depani Sarita , Israels Trijn , Niyrenda Dalida , Bailey Simon , Chagaluka George , Molyneux Elizabeth M. 
Published: October 2017

AbstractBackgroundKaposi sarcoma (KS) is the most common paediatric cancer in human immunodeficiency virus (HIV) endemic countries of sub‐Saharan Africa, but there is little research on management and outcomes.MethodsChildren with KS at Queen Elizabeth Central Hospital, Blantyre, Malawi treated between August 2012 and March 2015 with six courses of vincristine, bleomycin and etoposide combination chemotherapy, including antiretroviral therapy (ART) if HIV infected, were studied and outcomes compared with previously reported results.FindingsFifty‐six children were included; 38 (68%) were male; and 48 (86%) were HIV positive, of whom 36 (77%) were on ART at diagnosis. Median age at diagnosis was 8 years (interquartile range [IQR] 3–12) and median follow‐up was 16.9 months (IQR 3.4–36.4). Quality of life improved in 45 (80%) children; the median Lansky Score increased from 80% pre‐treatment to 100% post‐treatment. Eighteen (32%) children had complete response to treatment. At 12 months, overall survival was 71% (95% confidence interval [CI] 56–82) and event‐free survival (event = death, loss to follow‐up or relapse) was 50% (95% CI 36–63). At 1 year, the risk of loss to follow‐up was 13.4%. In a previous, same‐site, randomized controlled study of vincristine monotherapy, vincristine and bleomycin, or oral etoposide, oral etoposide monotherapy had the best outcome with survival at 12 month of 66% (95% CI 46–80) and event‐free survival of 52% (95% CI 33–68); however, loss to follow‐up was not reported.ConclusionOverall survival, event‐free survival and quality of life appear to have improved with this three‐agent combination chemotherapy; however larger, randomized studies are needed to determine optimal management.
Cognitive outcomes among Latino survivors of childhood acute lymphoblastic leukemia and lymphoma: A cross‐sectional cohort study using culturally competent, performance‐based assessment
Authors: Bava Laura , Johns Alexis , Kayser Kimberly , Freyer David R. 
Published: October 2017

AbstractBackgroundThis study sought to characterize cognitive outcomes among Latino survivors of childhood acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LL).ProcedureIn this cross‐sectional cohort study, Latino survivors of ALL (n = 57) and LL (n = 5) aged 6–16 years were pooled and evaluated using validated measures of cognitive, academic, and behavioral function and English language proficiency. Performance was compared with norms using single‐sample t‐tests.ResultsIn this cohort (n = 62, 50% male), mean ages at diagnosis and testing were 4.5 and 10.8 years, respectively; mean time off treatment was 44.7 months. All participants spoke English and over half (57%) identified Spanish as the primary language in the home. Forty‐two families (68%) placed in the two lowest Hollingshead socioeconomic status categories. Participants were below average for working memory (P < 0.001). Overall, participants were in the average range, but significantly lower than published norms on domain‐specific measures of verbal comprehension (P < 0.001); perceptual reasoning (P = 0.033); processing speed (P = 0.003); visual memory (P < 0.001); visuomotor attention, scanning, and sequencing (P = 0.005); and reading comprehension (P = 0.001). Parents reported concerns with working memory (P < 0.001) and metacognition (P = 0.014).ConclusionsSimilar to other childhood ALL/LL survivors, overall cognitive function in this Latino sample was relatively preserved but selected deficits were observed. Routine cognitive screening is indicated in this population.
Synthesis and radical ring‐opening polymerization of vinylcyclopropanes derived from amino acids with hydrophobic moieties
Authors: Takahashi Naoya , Yamada Shuhei , Sudo Atsushi , Endo Takeshi 
Published: October 2017

ABSTRACTSix 1,1‐disubstituted vinylcyclopropanes (VCP) were synthesized from glycine and amino acids bearing hydrophobic moieties, l‐alanine, l‐valine, l‐leucine, l‐isoleucine, and l‐phenylalanine. These VCP derivatives efficiently underwent radical ring‐opening polymerization to afford the corresponding polymers bearing trans‐vinylene moiety in the main chains and the amino acid‐derived chiral moieties in the side chains. The polymers were film‐formable, and in the films of polymers bearing the glycine‐ and alanine‐derived side chains, presence of hydrogen bonding was confirmed by IR analysis. Thermogravimetric analysis of the polymers revealed that the temperatures of 5% weight loss were higher than 300 °C. Differential scanning calorimetry clarified that the polymers were amorphous ones showing glass transition temperatures in a range of 48–80 °C. © 2017 Wiley Periodicals, Inc. J. Polym. Sci., Part A: Polym. Chem. 2017, 55, 3996–4002
Synthesis and characterization of functional polyethylene with regularly distributed thioester pendants via ring‐opening metathesis polymerization
Authors: Song Shaofei , Zhang Zhen , Liu Xiaoyu , Fu Zhisheng , Xu Junting , Fan Zhiqiang 
Published: October 2017

ABSTRACTFour kinds of functional polyethylene carrying thioester pendants were synthesized via ring‐opening metathesis polymerization (ROMP) of alkyl cyclopent‐3‐enecarbothioate catalyzed by a ruthenium‐based commercial catalyst and subsequent hydrogenation of the ROMP products (alkyl = ethyl, n‐butyl, n‐octyl, or n‐dodecyl). In these polymers the pendant alkyl thioester groups are precisely distributed along the backbone on every five methylene carbons. Chain structure, molecular weight and molecular weight distribution of the polymers were characterized by 1H and 13C NMR, and GPC. The ROMP reactions all reached high monomer conversions, and hydrogenation of the ROMP products were exhaustive. Thermal transitions and side chain crystallization behaviors of the polymer were investigated and characterized by DSC and TGA. Glass transition temperature and melting temperature of these polymers were higher than the counterparts containing ester pendants. TGA analysis indicated that all the thioester‐containing polymers exhibited moderate thermal stability, and the sulfur‐containing polymers show slightly lower thermal stability than their counterparts without sulfur. The new family of functionalized polyethylenes could be used as models of ethylene‐thioacrylate copolymers, and find applications as novel functional materials. © 2017 Wiley Periodicals, Inc. J. Polym. Sci., Part A: Polym. Chem. 2017, 55, 4027–4036
Visible light‐emitting diode‐sensitive thioxanthone derivatives used in versatile photoinitiating systems for photopolymerizations
Authors: Wu Xingyu , Jin Ming , Malval Jean‐Pierre , Wan Decheng , Pu Hongting 
Published: October 2017

ABSTRACTNovel thioxanthone (TX) derivatives are used as versatile photoinitiators upon visible light‐emitting diode (LED; e.g., 405, 425, and 450 nm) exposure. The mechanisms for the photochemical generation of reactive species (i.e., cations and free radicals) produced from photoinitiating systems based on the photoinitiator and an iodonium salt, tris(trimethylsilyl)silane, or an amine, were studied by UV–vis spectroscopy, fluorescence, cyclic voltammetry, steady‐state photolysis, and electron spin resonance spin‐trapping techniques. The reactive species are particularly efficient for cationic, free radical, hybrid, and thiol‐ene photopolymerizations upon LED exposure. The optimized photoinitiating systems exhibit higher efficiency than those of reference systems (i.e., isopropyl TX‐based photoinitiating systems), especially in the visible range. According to their beneficial features, these photoinitiating systems have considerable potential in photocuring applications. © 2017 Wiley Periodicals, Inc. J. Polym. Sci., Part A: Polym. Chem. 2017, 55, 4037–4045
Cognitive profiles in childhood and adolescence differ between adult psychotic and affective symptoms: a prospective birth cohort study
Authors: Koike S. , Barnett J. , Jones P. B. , Richards M. 
Published: October 2017

BackgroundDifferences between verbal and non-verbal cognitive development from childhood to adulthood may differentiate between those with and without psychotic symptoms and affective symptoms in later life. However, there has been no study exploring this in a population-based cohort.MethodThe sample was drawn from the MRC National Survey of Health and Development, and consisted of 2384 study members with self-reported psychotic experiences and affective symptoms at the age of 53 years, and with complete cognitive data at the ages of 8 and 15 years. The association between verbal and non-verbal cognition at age 8 years and relative developmental lag from age 8 to 15 years, and both adult outcomes were tested with the covariates adjusted, and mutually adjusted for verbal and non-verbal cognition.ResultsThose with psychotic experiences [thought interference (n = 433), strange experience (n = 296), hallucination (n = 88)] had lower cognition at both the ages of 8 and 15 years in both verbal and non-verbal domains. After mutual adjustment, lower verbal cognition at age 8 years and greater verbal developmental lag were associated with higher likelihood of psychotic experiences within individuals, whereas there was no association between non-verbal cognition and any psychotic experience. In contrast, those with case-level affective symptoms (n = 453) had lower non-verbal cognition at age 15 years, and greater developmental lag in the non-verbal domain. After adjustment, lower non-verbal cognition at age 8 years and greater non-verbal developmental lag were associated with higher risk of case-level affective symptoms within individuals.ConclusionsThese results suggest that cognitive profiles in childhood and adolescence differentiate psychiatric disease spectra.
Development of the PHAST model: generating standard public health services data and evidence for decision-making
Authors: Bekemeier Betty , Park Seungeun 
Published: October 2017

Abstract

Objective
Standardized data regarding the distribution, quality, reach, and variation in public health services provided at the community level and in wide use across states and communities do not exist. This leaves a major gap in our nation’s understanding of the value of prevention activities and, in particular, the contributions of our government public health agencies charged with assuring community health promotion and protection. Public health and community leaders, therefore, are eager for accessible and comparable data regarding preventive services that can inform policy decisions about where to invest resources.

Methods
We used literature review and a practice-based approach, employing an iterative process to identify factors that facilitate data provision among public health practitioners.

Results
This paper describes the model, systematically developed by our research team and with input from practice partners, that guides our process toward maximizing the uptake and integration of these standardized measures into state and local data collection systems.

Discussion
The model we developed, using a dissemination and implementation science framework, is intended to foster greater interest in and accountability for data collection around local health department services and to facilitate spatial exploration and statistical analysis of local health department service distribution, change, and performance.

Conclusion
Our model is the first of its kind to thoroughly develop a means to guide research and practice in realizing the National Academy of Medicine’s recommendation for developing systems to measure and track state and local public health system contributions to population health.
Digital Goods Are Valued Less Than Physical Goods
Authors: Atasoy Ozgun , Morewedge Carey K 
Published: October 2017

Abstract
Digital goods are, in many cases, substantive innovations relative to their physical counterparts. Yet, in five experiments, people ascribed less value to digital than to physical versions of the same good. Research participants paid more for, were willing to pay more for, and were more likely to purchase physical goods than equivalent digital goods, including souvenir photographs, books (fiction and nonfiction), and films. Participants valued physical goods more than digital goods whether their value was elicited in an incentive compatible pay-what-you-want paradigm, with willingness to pay, or with purchase intention. Greater capacity for physical than digital goods to garner an association with the self (i.e., psychological ownership) underlies the greater value ascribed to physical goods. Differences in psychological ownership for physical and digital goods mediated the difference in their value. Experimentally manipulating antecedents and consequents of psychological ownership (i.e., expected ownership, identity relevance, perceived control) bounded this effect, and moderated the mediating role of psychological ownership. The findings show how features of objects influence their capacity to garner psychological ownership before they are acquired, and provide theoretical and practical insights for the marketing, psychology, and economics of digital and physical goods.
Biliary Strongyloides stercoralis With Cholecystitis and Extensive Portal Vein Thrombosis
Authors: Filkins Laura M , Gaston David C , Mathison Blaine , Smith Timothy , Couturier Marc Roger , Murphy Ryan D , Ciarkowski Claire , Hanson Kimberly E , Cariello Paloma F 
Published: October 2017

Abstract
We report the rare finding of Strongyloides stercoralis rhabditiform larvae in biliary fluid, here associated with cholecystitis and near total portal vein thrombosis. The role of S. stercoralis leading to atypical clinical presentations and difficulty diagnosing strongyloidiasis in such patients with appropriate geographic exposure is discussed.
Long-term Outcome of Antiretroviral Treatment in Patients With and Without Concomitant Tuberculosis Receiving Health Center–Based Care—Results From a Prospective Cohort Study
Authors: Reepalu Anton , Balcha Taye Tolera , Sturegård Erik , Medstrand Patrik , Björkman Per 
Published: October 2017

Abstract

Background
In order to increase treatment coverage, antiretroviral treatment (ART) is provided through primary health care in low-income high-burden countries, where tuberculosis (TB) co-infection is common. We investigated the long-term outcome of health center–based ART, with regard to concomitant TB.

Methods
ART-naïve adults were included in a prospective cohort at Ethiopian health centers and followed for up to 4 years after starting ART. All participants were investigated for active TB at inclusion. The primary study outcomes were the impact of concomitant TB on all-cause mortality, loss to follow-up (LTFU), and lack of virological suppression (VS). Kaplan-Meier survival estimates and Cox proportional hazards models with multivariate adjustments were used.

Results
In total, 141/729 (19%) subjects had concomitant TB, 85% with bacteriological confirmation (median CD4 count TB, 169 cells/mm3; IQR, 99–265; non-TB, 194 cells/mm3; IQR, 122–275). During follow-up (median, 2.5 years), 60 (8%) died and 58 (8%) were LTFU. After ≥6 months of ART, 131/630 (21%) had lack of VS. Concomitant TB did not influence the rates of death, LTFU, or VS. Male gender and malnutrition were associated with higher risk of adverse outcomes. Regardless of TB co-infection status, even after 3 years of ART, two-thirds of participants had CD4 counts below 500 cells/mm3.

Conclusions
Concomitant TB did not impact treatment outcomes in adults investigated for active TB before starting ART at Ethiopian health centers. However, one-third of patients had unsatisfactory long-term treatment outcomes and immunologic recovery was slow, illustrating the need for new interventions to optimize ART programs.
High Dynamic Range Bacterial Cell Detection in a Novel “Lab-In-A-Comb” for High-Throughput Antibiotic Susceptibility Testing of Clinical Urine Samples
Authors: Pivetal Jeremy , Woodward Martin J. , Reis Nuno M. , Edwards Alexander D. 
Published: October 2017

ABSTRACTAntibiotic resistance in urinary tract infection is a major global challenge, and improved cost-effective and rapid antibiotic susceptibility tests (AST) are urgently needed to inform correct antibiotic selection. Although microfluidic technology can miniaturise AST, the high dynamic range of pathogen density found in clinical urine samples makes direct testing of clinical samples – rather than testing colonies from overnight agar plates – extremely challenging. We evaluated for the first time how pathogen concentration in urine affects microfluidic AST using a novel microplate-compatible high-throughput microfluidic AST system termed “Lab-on-a-Comb”. When tested with clinicalE. coliisolates at standardised density, these devices gave identical antibiotic susceptibility profiles to standard disc diffusion and microtitre plate tests. Bacterial detection directly in synthetic urine spiked with clinicalE. coliUTI isolates was possible over a very large dynamic range of starting cell densities, from 103– 108CFU/mL which covers the range of pathogen cell densities found in patient urine. The lowest cell density where cell growth was reproducibly detected optically was 9.6x102CFU/mL, corresponding to one single CFU detected in a 1 μL microcapillary-an unprecedented level of sensitivity. Cell growth kinetics followed a simple Monod model with fast growth limited by the substrate availability and an estimated doubling time of 24.5 min, indicating optimalE. coligrowth conditions within these microfluidic devices. There was a trade-off between sensitivity and speed of detection, with 105CFU/mL detection possible within 2h, but 6h incubation required at 103CFU/mL.
Hey2restricts cardiac progenitor addition to the developing heart
Authors: Gibb Natalie , Lazic Savo , Deshwar Ashish R. , Yuan Xuefei , Wilson Michael D. , Scott Ian C. 
Published: October 2017

ABSTRACTA key event in vertebrate heart development is the timely addition of second heart field (SHF) progenitor cells to the poles of the heart tube. This accretion process must occur to the proper extent to prevent a spectrum of congenital heart defects (CHDs). However, the factors that regulate this critical process are poorly understood. Here we demonstrate that Hey2, a bHLH transcriptional repressor, restricts SHF progenitor accretion to the zebrafish heart.hey2expression demarcated a distinct domain within the cardiac progenitor population. In the absence of Hey2 function an increase in myocardial cell number and SHF progenitors was observed. We found that Hey2 limited proliferation of SHF-derived cardiomyocytes in a cell-autonomous manner, prior to heart tube formation, and further restricted the developmental window over which SHF progenitors were deployed to the heart. Taken together, our data suggests a role for Hey2 in controlling the proliferative capacity and cardiac contribution of late-differentiating cardiac progenitors.
Peripherally-induced regulatory T cells contribute to the control of autoimmune diabetes
Authors: Schuster Cornelia , Zhao Fangzhu , Kissler Stephan 
Published: October 2017

AbstractType 1 diabetes (T1D) results from the autoimmune destruction of pancreatic beta cells and is partly caused by deficiencies in the Foxp3+regulatory T cell (Treg) compartment. Conversely, therapies that increase Treg function can prevent autoimmune diabetes in animal models. The majority of Tregs develop in the thymus (tTregs), but a proportion of Foxp3+Tregs is generated in the periphery (pTregs) from Foxp3-CD4+T cell precursors. Whether pTregs play a distinct role in T1D has not yet been explored. We report here that pTregs are a key modifier of disease in the nonobesed diabetic (NOD) mouse model for T1D. We generated NOD mice deficient for theFoxp3enhancer CNS1 involved in pTreg induction. We show that CNS1 knockout decreased the frequency of pTregs and increased the risk of diabetes. Our results show that pTregs fulfill an important non-redundant function in the prevention of beta cell autoimmunity that causes T1D.