bluuebunny/crossref_metadata_2025_split huggingface dataset:
The effects of joint disease, inhibitors and other complications on health‐related quality of life among males with severe haemophilia A in the United States
Authors: Soucie J. M. , Grosse S. D. , Siddiqi A.‐E.‐A. , Byams V. , Thierry J. , Zack M. M. , Shapiro A. , Duncan N. ,   the Hemophilia Treatment Centers Network
Published: June 2017

IntroductionHealth‐related quality of life (HRQoL) is reduced among persons with haemophilia. Little is known about how HRQoL varies with complications of haemophilia such as inhibitors and joint disease. Estimates of preference‐based HRQoL measures are needed to model the cost‐effectiveness of prevention strategies.AimWe examined the characteristics of a national sample of persons with severe haemophilia A for associations with two preference‐based measures of HRQoL.MethodsWe analysed utility weights converted from EuroQol 5 Dimensions (EQ‐5D) and the Short Form 6 Dimensions (SF‐6D) scores from 1859 males aged ≥14 years with severe haemophilia A treated at 135 US haemophilia treatment centres in 2005‐2011. Bivariate and regression analyses examined age‐group‐specific associations of HRQoL with inhibitor status, overweight/obesity, number of bleeds, viral infections, indicators of liver and joint disease, and severe bleeding at the time of the first HRQoL measurement.ResultsOverall mean HRQoL utility weight values were 0.71 using the SF‐6D and 0.78 using the EQ‐5D. All studied patient characteristics except for overweight/obesity were significantly associated with HRQoL in bivariate analyses. In a multivariate analysis, only joint disease was significantly associated with utility weights from both HRQoL measures and across all age groups. After adjustment for joint disease and other variables, the presence of an inhibitor was not significantly associated with HRQoL scores from either of the standardized assessment tools.ConclusionClinically significant complications of haemophilia, especially joint disease, are strongly associated with HRQoL and should be accounted for in studies of preference‐based health utilities for people with haemophilia.
PNPLA3 as a liver steatosis risk factor following living‐donor liver transplantation for hepatitis C
Authors: Miyaaki Hisamitsu , Miuma Satoshi , Taura Naota , Shibata Hidetaka , Soyama Akihiko , Hidaka Masaaki , Takatsuki Mitsuhisa , Eguchi Susumu , Nakao Kazuhiko 
Published: June 2017

AimLiver steatosis frequently occurs following liver transplantation (LT) and can affect patient outcome. Here, we aimed to clarify the steatosis and steatohepatitis risk factors that apply after living‐donor LT for chronic hepatitis C.MethodsWe retrospectively examined 43 transplant recipients and donors, and tested for single nucleotide polymorphisms in the PNPLA3 gene. Liver biopsies taken 1 year after transplantation and yearly thereafter, or when abnormal liver enzyme levels were detected, were examined by histopathology.ResultsLiver steatosis (>5% steatotic hepatocytes) was evident in 13 of 43 cases (30%), and steatohepatitis in 3 (7.0%). The average time to steatosis after LT was 2.74 ± 1.55 years. The PNPLA3 rs738409 GG genotype, a steatosis risk factor, was identified in 13 recipients and 10 donors. Steatosis prevalence did not differ according to recipient genotype. However, this condition was significantly more common among patients who received tissue from donors carrying the rs738409 GG genotype compared to those with grafts from donors of the CC or CG genotype (60, 7, and 26%, respectively; P < 0.05). All 3 steatohepatitis cases were associated with the GG donor genotype.ConclusionThe PNPLA3 rs738409 GG donor genotype affects liver steatosis and steatohepatitis risk following living‐donor LT.
Weathering of ancient and medieval glasses—potential proxy for nuclear fuel waste glasses. A perennial challenge revisited
Authors: Heimann Robert B. 
Published: June 2017

AbstractAncient and medieval glasses that have survived the deleterious attack of the environment for millennia have long since proposed as proxy to estimate and predict the corrosion mechanism of nuclear waste glasses. However, because both composition and environmental burial conditions vastly differ between hydrolytically less stable ancient glasses and modern advanced nuclear waste glasses, only semiquantitative conclusions can be drawn about the likely performance of the latter as long‐term stable immobilization matrices for high‐level radioactive nuclear waste. In this contribution, special emphasis has been devoted to the behavior of manganese, present as both iron decolorant and coloring ions in ancient Roman and medieval glasses. Study of the behavior of manganese in ancient glasses during weathering may provide some limited clues to the behavior of long‐lived radioactive technetium‐99. Knowledge of the corrosion kinetics of ancient glasses will allow, eventually, a reasonable prediction of the long‐term performance of glassy nuclear waste forms as function of their composition and environmental parameters, i.e. groundwater composition, flow rate, pH, solution volume, and surface area.
Understanding the experience of women admitted to a psychiatric hospital in Sydney with psychosis or mania following childbirth after World War II (1945–1955)
Authors: Jefferies Diana , Duff Margaret , Nicholls Daniel 
Published: June 2017

AbstractIn the present study, we investigated a unique set of historical health‐care records of women admitted to a psychiatric hospital in Sydney, Australia with a diagnosis of psychosis or mania after childbirth in the post‐World War II (WWII) period, from 1945 to 1955. This research is part of a larger project examining how the descriptions of these women documented in the health‐care records from 1885 to 1975 affected their treatment and the outcome of their admission. In the present paper, we report on the findings from an intensive examination of the post WWII documents. Eighteen health‐care records from a psychiatric facility (Gladesville Hospital) were identified from admission registers housed in the State Records Office of New South Wales in 2014. Although seven records had been destroyed, 11 were transcribed verbatim. The records contain demographic information; descriptions of the women's signs and symptoms on admission; and information about the women before, during, and after their admission found in letters from relatives or medical staff. A content analysis of admission information showed how the women were described by health‐care professionals, but a textual analysis of the records revealed that there were other factors that could have contributed to the women's condition, which might not have been taken into consideration when treatment and care were devised. The present study demonstrates the value of investigating historical health‐care records to understand how prevailing attitudes and practices might affect diagnosis and treatment.
Large barocaloric effect and pressure‐mediated electrocaloric effect in Pb0.99Nb0.02(Zr0.95Ti0.05)0.08O3 ceramics
Authors: Patel Satyanarayan , Chauhan Aditya , Vaish Rahul , Lynch Christopher S. 
Published: June 2017

AbstractFerroelectric materials are being actively explored for next‐generation solid‐state cooling technology. Even though bulk materials possess an advantage in terms of overall heat extraction capacity, their performance is limited due to low adiabatic temperature change. In this regard, the present article explores enhanced cooling capacity of bulk polycrystalline Pb0.99Nb0.02(Zr0.95Ti0.05)0.08O3 (PNZT) through external‐field mediation and coupled caloric effects. Barocaloric (BC) and electrocaloric (EC) effects were indirectly estimated using polarization versus electric field (P‐E) loops (under varying pressure and temperature). It was observed that under applied pressure of 325 MPa, ΔTEC could be improved from 1 K to 4.5 K. Similarly, a peak unbiased ΔTBC of 1.5 K could be enhanced to 5.3 K under an electric field of 5 MV·m−1. These figures correspond to an improvement of ~400% over the unbiased values. The results are indicative of the multicaloric cooling capacity of bulk ferroelectric materials.
Prevalence of sun‐protective behaviour and intentional sun tanning in German adolescents and adults: results of a nationwide telephone survey
Authors: Görig T. , Diehl K. , Greinert R. , Breitbart E.W. , Schneider S. 
Published: June 2017

AbstractBackgroundThe incidence rate of melanoma in the Caucasian population is rising worldwide. One of the major environmental risk factors for melanoma is the exposure to ultraviolet (UV) radiation. To prevent skin damage caused by UV exposure, several organizations recommend wearing protective clothing, staying in the shade, avoiding the outdoors during midday and using sunscreen.ObjectiveTo provide representative data on factors associated with sun‐protective behaviours and intentional sun exposure during summertime in the German population.MethodsA population‐based sample of 3000 German residents aged 14–45 years (response: 32.1%) was interviewed via telephone from October to December 2015. Survey participants provided data on the use of recommended sun‐protective measures on a sunny summer day and their intentional sun exposure during summertime. Data were weighted by age, sex, educational level and federal state to ensure the national representativeness of the sample.ResultsWearing long‐sleeved clothes was the most frequently reported protective measure (53.9%), while wearing headgear was the least common (17.9%). Significantly lower frequencies of almost all recommended sun‐protective measures were identified for males, participants between 14 and 25 years, those with an immigrant background and those with low levels of education. Using sun protection was positively associated with female gender (OR = 1.62), higher levels of education (ORhigh = 1.27), sunburn during childhood (OR = 1.33) and paying attention to healthy nutrition (OR = 1.64; all P < 0.05). Sun tanning behaviour was associated with female gender (OR = 1.42), younger age, lower level of education, sunbed use (OR = 5.24) and smoking status (OR = 1.50; all P < 0.001).ConclusionAs individual sun‐protective measures are easy to implement and provide effective protection against high‐risk UV exposure, campaigns on skin cancer prevention in Germany should put a stronger focus on young adults, men, less educated individuals and those with immigration background.
Characterization of a PRISTANE‐induced lupus‐associated model in the non‐human primate cynomolgus monkey
Authors: Wang Jing , Shen Hui , Zhu Yuqiang , Zhu Ying , Cai Lei , Wang Zhiyao , Shi Qin , Qiu Yuhua 
Published: June 2017

AbstractBackgroundLupus is an autoimmune disease with complex syndrome. Rodent models have limitations for recapitulating the spectrum of the disease. A more powerful translational model is desirable.MethodLupus‐associated model in cynomolgus monkeys was induced by two intraperitoneal injections of 2, 6, 10, 14‐tetramethylpentadecane (PRISTANE). Lupus‐specific biomarkers and manifestations over a 246‐day period were observed at multilevel. To visualize and quantify kidney function in real time, contrast‐enhanced ultrasound was used.ResultsThe indicative biomarkers and manifestations fulfilled major diagnosis criteria according to the “Criteria of Lupus” of the American College of Rheumatology. Significant changes in time‐intensity curve parameters were observed, indicating impaired renal function and the method as a feasible, non‐invasive diagnostic method in primate model.ConclusionsWe successfully induced lupus‐associated model with systemic lupus syndrome. This primate model can be a valuable translational model for further pathogenesis and symptomology studies and for exploring therapeutic candidates.
Creating a placental inflammatory composite index that has a high prognostic relevance to child morbidity
Authors: Chen Yan , Zou Lile , Zhao Yanjun , Wu Ting , Ye Jiangfeng , Zhang Huijuan , Zhang Jun 
Published: May 2017

AbstractAimSelecting pathologic measures of placental inflammation that affect pregnancy and childhood health is largely empirical. We aimed to systematically select several core inflammation‐related placental measures to construct a novel placental inflammatory evaluation criterion with a high prognostic relevance to child morbidity.MethodsWe used data from the US Collaborative Perinatal Project (1959–1976), a longitudinal birth cohort study that recruited women during pregnancy and followed the children until 7 years of age. Bootstrap resampling, least absolute shrinkage and selection operator, and receiver–operator curve were used to select placental pathologic measures that were closely related to child morbidity to form a placental inflammatory composite index.ResultsTwenty‐six candidate placental inflammation‐related measures were ranked based on their close association with adverse neonatal outcomes. The top five placental measures were: (i) neutrophilic infiltration in umbilical artery; (ii) placental weight–birthweight ratio; (iii) necrosis in decidua capsularis; (iv) bacterial colony in epithelium of amnion; and (v) opacity of membranes and fetal surface. Several composite indexes were constructed. A five‐measure composite index that had the highest prognostic relevance was chosen. Compared with subjects without any of the five abnormal measures, those with any lesion ranging from 1 to 5 had a 1.2‐ to 4.6‐fold risk of adverse child outcomes, respectively.ConclusionOur composite index is simple, evidence‐based, and has predictive value for child morbidity. It may be used as a novel placental inflammatory evaluation criterion.
Prenatal diagnosis of Duchenne and Becker muscular dystrophies: Underestimated problem of the secondary prevention of monogenetic disorders
Authors: Massalska Diana , Zimowski Janusz Grzegorz , Roszkowski Tomasz , Bijok Julia , Pawelec Magdalena , Bednarska‐Makaruk Małgorzata 
Published: May 2017

AbstractAimThe aim of this study was to analyze the influence of effective preconceptional testing for carrier status in women at risk for Duchenne and Becker muscular dystrophies (D/BMD) on the prenatal diagnosis.MethodsA retrospective analysis of 201 prenatal tests was performed in 169 Polish women at risk, in regard to time of testing for carrier status (prior to conception or during pregnancy) and carrier status of tested women, including confirmed D/BMD carriers (n = 78; 46.2%), D/BMD non‐carriers – tested for germline mosaicism risk (n = 23; 13.6%), and women at risk with uncertain carrier status (n = 68; 40.2%).ResultsOnly 52.7% of women were tested for D/BMD carrier status prior to conception and in these women prenatal diagnosis was carried out more frequently in the first trimester of pregnancy (64.7% vs 47.8%; P = 0.035). The results of prenatal testing in male fetuses in pregnancies of confirmed D/BMD carriers and D/BMD non‐carriers – tested for germline mosaicism risk were conclusive in all cases, whereas in women with uncertain carrier status, only 60.0% of results were conclusive. Eighty‐five of 103 female fetuses (82.5%) were tested prenatally and in 31.8% of them fetal carrier status was confirmed.ConclusionCarrier status testing in women prior to conception has a positive impact on the frequency of first‐trimester prenatal diagnosis and known D/BMD carrier status on the effectiveness of prenatal diagnosis. Due to the low percentage of women tested effectively prior to conception, carrier status testing in the families at risk should be propagated (including possibility of prenatal diagnosis of female fetuses).
Noninvasive beat‐to‐beat finger arterial pressure monitoring during orthostasis: a comprehensive review of normal and abnormal responses at different ages
Authors: van Wijnen V. K. , Finucane C. , Harms M. P. M. , Nolan H. , Freeman R. L. , Westerhof B. E. , Kenny R. A. , ter Maaten J. C. , Wieling W. 
Published: May 2017

AbstractOver the past 30 years, noninvasive beat‐to‐beat blood pressure (BP) monitoring has provided great insight into cardiovascular autonomic regulation during standing. Although traditional sphygmomanometric measurement ofBPmay be sufficient for detection of sustained orthostatic hypotension, it fails to capture the complexity of the underlying dynamicBPand heart rate responses. With the emerging use of noninvasive beat‐to‐beatBPmonitoring for the assessment of orthostaticBPcontrol in clinical and population studies, various definitions for abnormal orthostaticBPpatterns have been used. Here, age‐related changes in cardiovascular control in healthy subjects will be reviewed to define the spectrum of the most important abnormal orthostaticBPpatterns within the first 180 s of standing. Abnormal orthostaticBPresponses can be defined as initial orthostatic hypotension (a transient systolicBPfall of >40 mmHg within 15 s of standing), delayedBPrecovery (an inability of systolicBPto recover to a value of >20 mmHg below baseline at 30 s after standing) and sustained orthostatic hypotension (a sustained decline in systolicBPof ≥20 mmHg occurring 60–180 s after standing). In the evaluation of patients with light‐headedness, pre(syncope), (unexplained) falls or suspected autonomic dysfunction, it is essential to distinguish between normal cardiovascular autonomic regulation and these abnormal orthostaticBPresponses. The prevalence, clinical relevance and underlying pathophysiological mechanisms of these patterns differ significantly across the lifespan. Initial orthostatic hypotension is important for identifying causes of syncope in younger adults, whereas delayedBPrecovery and sustained orthostatic hypotension are essential for evaluating the risk of falls in older adults.
Tax competition, tax coordination, and e‐commerce
Authors: Bacache Beauvallet Maya 
Published: June 2017

AbstractThis paper contributes to the political debate on commodity tax coordination in Europe. It examines, within Nielsen's setting, the impact of online shopping on taxes and tax competition and contrasts destination‐based taxation with origin‐based taxation. Heterogeneous consumers surf the Internet at a cost. It is assumed that fiscal leakage occurs during transactions on the Internet either because of tax evasion or of the existence of a tax liability threshold. Whereas cross‐border shopping effectively results in taxes being levied at origin, this model by adding e‐commerce shows that taxing online trade based on the origin principle softens tax competition and allows sustaining the destination principle on brick‐and‐mortar shops. Tax competition is reduced if tax is origin‐based, because the Internet helps the small country expand its tax base, which induces tax convergence.
Method development for the determination of elements in Hypericum perforatum L. (St John's wort) herb and preparations using inductively coupled plasma–optical emission spectroscopy and microwave digestion
Authors: Owen Jade D , Evans Sara J , Guirguis Amira , Kellett Kathryn , Stair Jacqueline L 
Published: May 2017

Abstract

Objectives
A method was developed to analyze St John's wort (Hypericum perforatum L.) herb and preparations using inductively coupled plasma–optical emission spectroscopy (ICP-OES) to determine the quantity of 11 elements (Al, B, Ba, Ca, Cu, Fe, Mg, Mn, Ni, Sr and Zn).

Methods
This study includes the evaluation of digestion acids and calibration methods, as well as instrumental parameters such as choice of nebulizer and emission wavelength.

Key findings
Two nebulizers (Conikal and SeaSpray) performed similarly for most elements, and two optimum wavelengths were determined for each element. Five acids were evaluated for the digestion of the Polish Certified Reference Material Tea Leaves (INCT-TL-1), while three were taken forward to use for the different St John's wort formulations (i.e. herb, capsule and tablet). A simple protocol using 5 ml HNO3 was sufficient in most cases; however, variability was observed for elements often bound in silicates (e.g. Al, Fe and Zn). An external weighted calibration was also found to be preferential over unweighted, and the use of standard addition affected some concentration values up to 20%.

Conclusions
Therefore, this paper presents the development and optimized method parameters to be used with ICP-OES that will allow the analysis of 11 key elements present in St John's wort herb and preparations.
The relationship between sleep and glucagon‐like peptide 1 in patients with abnormal glucose tolerance
Authors: Reutrakul Sirimon , Sumritsopak Rungtip , Saetung Sunee , Chanprasertyothin Suwannee , Anothaisintawee Thunyarat 
Published: May 2017

SummaryGlucagon‐like peptide 1 plays a role in glucose regulation. Sleep disturbances (obstructive sleep apnea, insufficient or poor sleep quality) have been shown to adversely affect glucose metabolism. This study aimed to explore the relationship between sleep and glucagon‐like peptide 1 regulation in patients with abnormal glucose tolerance. Seventy‐one adults with haemoglobin A1c levels between 5.7% and < 6.5% and no history of diabetes participated. Habitual sleep duration and efficiency were obtained from 7‐day actigraphy recordings. Obstructive sleep apnea was assessed using an overnight home monitor. Glucagon‐like peptide 1 levels were measured during a 75‐g glucose tolerance. The area under the curve of glucagon‐like peptide 1 was calculated. The mean age (SD) was 55.1 (8.3) years and median (interquartile range) haemoglobin A1c was 5.97% (5.86, 6.23). There was no relationship between sleep duration or efficiency and fasting or area under the curve glucagon‐like peptide 1. Glucagon‐like peptide 1 levels did not differ among those sleeping ≤ 5.75, > 5.75–< 6.5 or ≥ 6.5 h per night. Increasing apnea–hypopnea index, an indicator of obstructive sleep apnea severity, correlated with lower area under the curve glucagon‐like peptide 1 (B −0.242, P = 0.045), but not with fasting glucagon‐like peptide 1 (B −0.213, P = 0.079). After adjusting for sex, haemoglobin A1c and body mass index, increasing apnea–hypopnea index was negatively associated with having area under the curve glucagon‐like peptide 1 in the highest quartile (odds ratio 0.581, P = 0.028, 95% CI 0.359, 0.942). This study demonstrated that increasing obstructive sleep apnea severity was associated with lower glucagon‐like peptide 1 response to glucose challenge. This could possibly be an additional mechanism by which obstructive sleep apnea affects glucose metabolism. Whether raising glucagon‐like peptide 1 levels in patients with abnormal glucose tolerance with more severe obstructive sleep apnea will be beneficial should be explored.