bluuebunny/crossref_metadata_2025_split huggingface dataset:
An international survey of patients with tetrahydrobiopterin deficiencies presenting with hyperphenylalaninaemia
Authors: Opladen Thomas , Hoffmann Georg F. , Blau Nenad 
Published: June 2012

AbstractObjectivesThe present study summarizes clinical and biochemical findings, current treatment strategies and follow‐up in patients with tetrahydrobiopterin (BH4) deficiencies.MethodsWe analyzed the clinical, biochemical and treatment data of 626 patients with BH4 deficiencies [355 with 6‐pyruvoyl‐tetrahydropterin synthase (PTPS), 217 with dihydropteridine reductase (DHPR), 31 with autosomal recessive GTP cyclohydrolase I (GTPCH), and 23 with pterin‐4a‐carbinolamine dehydratase (PCD) deficiencies] from the BIODEF Database. Patients with autosomal dominant GTPCH and SR deficiencies will not be discussed in detail.ResultsUp to 57 % of neonates with BH4 deficiencies are already clinically symptomatic. During infancy and childhood, the predominant symptoms are muscular hypotonia, mental retardation and age‐dependent movement disorders, including dystonia. The laboratory diagnosis of BH4 deficiency is based on a positive newborn screening (NBS) for phenylketonuria (PKU), characteristic profiles of urinary or dried blood spot pterins (biopterin, neopterin, and primapterin), and the measurement of DHPR activity in blood. Some patients with autosomal recessive GTPCH deficiency and all with sepiapterin reductase deficiency may be diagnosed late due to normal blood phenylalanine in NBS. L‐dopa, 5‐hydroxytryptophan, and BH4 are supplemented in PTPS and GTPCH‐deficient patients, whereas L‐dopa, 5‐hydroxytryptophan, folinic acid and diet are used in DHPR‐deficient patients. Medication doses vary widely among patients, and our understanding of the effects of dopamine agonists and monoamine catabolism inhibitors are limited.ConclusionsBH4 deficiencies are a group of treatable pediatric neurotransmitter disorders that are characterized by motor dysfunction, mental retardation, impaired muscle tone, movement disorders and epileptic seizures. Although the outcomes of BH4 deficiencies are highly variable, early diagnosis and treatment result in improved outcomes.
A comparison of quality of life following spontaneous conception and assisted reproduction
Authors: De Pascalis Leonardo , Agostini Francesca , Monti Fiorella , Paterlini Marcella , Fagandini Piergiuseppina , La Sala Giovanni B. 
Published: June 2012

AbstractObjectiveTo compare the levels of and changes in quality of life (QoL) during pregnancy between couples who conceived spontaneously and couples who underwent successful treatment by assisted reproductive technology (ART).MethodsIn a survey at the Santa Maria Nuova Hospital, Reggio Emilia, Italy, between September 11, 2009, and May 25, 2011, 230 individuals (57 ART couples and 58 couples who conceived spontaneously) completed Short‐Form 36 on QoL at 22 and 32 gestational weeks.ResultsCompared with men, women had lower scores in most dimensions of QoL regardless of the method of conception; women also showed a decrease in physical and social QoL during pregnancy that was not evident among men. Compared with non‐ART couples, ART couples had lower physical (especially women) and social QoL; ART couples also showed a decrease in social QoL from the second to the third trimester that was not observed among non‐ART couples.ConclusionThe results underline the higher burden that pregnancy represents for women on a physical and social level. ART treatment seems to increase the negative impact of pregnancy on QoL in the couple as a whole; early support in adjusting to the realities and demands of pregnancy might prove beneficial for these future parents.
Strokes of upward illumination occurring within a few milliseconds after typical lightning return strokes
Authors: Stolzenburg Maribeth , Marshall Thomas C. , Karunarathne Sumedhe , Karunarathna Nadeeka , Warner Tom A. , Orville Richard E. , Betz Hans‐Dieter 
Published: June 2012

Correlated data for natural cloud‐to‐ground lightning flashes were obtained with a high‐speed video camera (54000 frames per second), five stations of electric field change antennas, and a seven‐station VLF/LF LIghtning NETwork (LINET), along with operational lightning and electric field networks at Kennedy Space Center, Florida. Four observed flashes at 25–55 km distance had return‐stroke‐like upward illumination (UI) from the ground just after (within 2 ms of) a return stroke (RS), but in a separate channel connection to ground (0.7 to 2.5 km apart). The UI events were visible for short duration (0.5 ms or less) and short length (to 1.7 km altitude or less), and they had slow propagation speed (1.6–2.6 × 107 m s−1) and no apparent connection to the RS channel. In one case, the RS was no longer visible to the ground when the UI began 1.30 ms later, although mid‐level channel was still distinct. In another case, the main RS had dimmed over the 0.52 ms interval before the UI but was still visible. The remaining two cases had the entire length of the prior RS channel no longer visible (for 40 and 240 μs) before the UI began (1.32 and 1.87 ms later). Electric field change data for the UI events resemble those of weak return strokes. These events were not located by the operational ground‐strike and VHF detection systems; all four UI events were detected by LINET as negative ground strokes with small (less than 7 kA) peak current magnitude.
Systemwide Evaluation of Avian Predation on Juvenile Salmonids from the Columbia River Based on Recoveries of Passive Integrated Transponder Tags
Authors: Evans Allen F. , Hostetter Nathan J. , Roby Daniel D. , Collis Ken , Lyons Donald E. , Sandford Benjamin P. , Ledgerwood Richard D. , Sebring Scott 
Published: June 2012

AbstractWe recovered passive integrated transponder (PIT) tags from nine piscivorous waterbird colonies in the Columbia River basin to evaluate avian predation on Endangered Species Act (ESA)‐listed salmonid Oncorhynchus spp. populations during 2007–2010. Avian predation rates were calculated based on the percentage of PIT‐tagged juvenile salmonids that were detected as passing hydroelectric dams and subsequently were consumed and deposited by birds on their nesting colonies. Caspian terns Hydroprogne caspia (hereafter, “terns”) and double‐crested cormorants Phalacrocorax auritus (hereafter, “cormorants”) nesting on East Sand Island in the Columbia River estuary consumed the highest proportions of available PIT‐tagged salmonids, with minimum predation rates ranging from 2.5% for Willamette River spring Chinook salmon O. tshawytscha to 16.0% for Snake River steelhead O. mykiss. Estimated predation rates by terns, cormorants, gulls of two species (California gull Larus californicus and ring‐billed gull L. delawarensis), and American white pelicans Pelecanus erythrorhynchos nesting near the confluence of the Snake and Columbia rivers were also substantial; minimum predation rates ranged from 1.4% for Snake River fall Chinook salmon to 13.2% for upper Columbia River steelhead. Predation on ESA‐listed salmonids by gulls and American white pelicans were minor (<2.0% per ESA‐listed salmonid population) relative to predation by terns and cormorants. Cumulative impacts were greater for Snake River and upper Columbia River salmonids than for salmonids originating closer to the estuary because upriver salmonids must migrate past more bird colonies to reach the ocean. Predation rates adjusted for colony size (per capita rates) were significantly higher for terns and cormorants nesting at inland colonies (upstream of Bonneville Dam) than for those nesting in the estuary, suggesting that inland colonies have a greater reliance on salmonids as a food source. Management actions to increase salmonid survival by reducing avian predation in the estuary could be offset if birds that disperse from the estuary relocate to inland nesting sites on or near the Columbia River.
Counsellors' perceptions of client progression when working with clients who intentionally self‐harm and the impact such work has on the therapist
Authors: Fleet Doreen , Mintz Rita 
Published: June 2012

AbstractIntroduction: This paper describes a qualitative study focusing on counsellors' perceptions of client progression when working with clients who intentionally self‐harm and the impact this has on the therapist. Method: Semi‐structured interviews were employed and the constant comparative method utilised to analyse the data. Findings: Findings indicate that counsellors experience intense emotions in response to self‐harm including: shock; sadness; anxiety; anger; and frustration. Counsellors also struggled to manage the tension between multiple dualities, corresponding to professional opinions of a seemingly polar opposite nature. Although participants indicated a requirement to work in a client‐led way, they all had either an explicit or implicit agenda for change. These two conflicting states create a tension for a counsellor that is difficult to resolve. In addition there were various views of client progression, yet all participants agreed that progression was not simply about stopping self‐harm. Conclusion: There is a significant impact on the counsellor when working with clients who intentionally self‐harm and the complexity of the phenomenon appears to exacerbate their anxiety. Implications: Further research within the counselling field focusing on the ambiguous nature of the subject may increase understanding, which in turn may contribute to greater therapist efficacy when working with this client group.
Angle-resolved photoemission study of Fe3O4(0 0 1) films across Verwey transition
Authors: Ran Fan-Yong , Tsunemaru Yasushi , Hasegawa Takahide , Takeichi Yasuo , Harasawa Ayumi , Yaji Koichiro , Kim Sunghun , Kakizaki Akito 
Published: June 2012

Abstract
The valence band structure of well-ordered Fe3O4(0 0 1) films was investigated in situ by angle-resolved photoemission spectroscopy (ARPES) at room temperature (RT) and 90 K. A band gap of about 70 meV below the Fermi level (E
F) can be observed at 90 K, suggesting a ‘metal–insulator’ transition. With temperature reduced from RT to 90 K, O 2p-derived features shown in ARPES spectra change substantially. Meanwhile, the feature near E
F of the Fe3O4(0 0 1) film at 90 K becomes about 0.1 eV deeper than that at RT. The results are compared with recent theoretical studies.
Diameter-speed relation of sprite streamers
Authors: Kanmae T , Stenbaek-Nielsen H C , McHarg M G , Haaland R K 
Published: June 2012

Abstract
Propagation and splitting of sprite streamers has been observed at high temporal and spatial resolution using two intensified high-speed CMOS cameras recording at 10 000 and 16 000 frames per second. Concurrent video recordings from a remote site provided data for triangulation allowing us to determine accurate altitude scales for the sprites. Diameters and speeds of the sprite streamers were measured from the high-speed images, and the diameters were scaled to the reduced diameters based on the triangulated locations. The sprite streamers with larger reduced diameter move faster than those with smaller diameter; the relation between the reduced diameter and speed is roughly linear. The reduced diameters at ≈65–70 km altitude are larger than streamer diameters measured at ground pressure in laboratory discharges indicating a deviation from the similarity law possibly due to the effects of the photoionization and an expansion of the streamer head along its propagation over a long distance. The reduced diameter and speed of the sprite streamers agree well with the diameter–velocity relation proposed by Naidis (2009 Phys. Rev. E 79 057401), and the peak electric field of the sprite streamers is estimated to be approximately 3–5 times the breakdown threshold field.
Electric field measurement in a DBD in helium and helium–hydrogen mixture
Authors: Ivković S S , Obradović B M , Kuraica M M 
Published: June 2012

Abstract
Time–space-resolved optical emission spectroscopy was used for the investigation of a diffuse dielectric barrier discharge (DBD) in helium and in 95%He + 5%H2 gas mixture. The DBD in helium was diffuse in the whole range of the applied voltage, while in the gas mixture the discharge operated in diffuse mode only in the regime with more than three current peaks. In the helium discharge, the excitation of hydrogen H
β
is delayed compared with the excitation of the helium atomic line, while in the gas mixture both lines are excited simultaneously. Using the Stark polarization spectroscopy technique based on the He I 492.19 nm line and its forbidden counterpart, the electric field distribution (EFD) was measured in the cathode region of the DBD in helium and in the helium–hydrogen mixture. Electric field space distributions in both gas discharges, obtained at 20 kPa pressure, showed linear decreasing in the cathode region, which is a typical characteristic of the glow discharge. Linear fits of the EFDs showed that the length of the cathode fall region is smaller in the gas mixture than in pure helium. In the multiple current peak mode of the DBD, EFD was measured during each of the three current peaks and no noticeable difference between them is observed.
ITO electrode/photoactive layer interface engineering for efficient inverted polymer solar cells based on P3HT and PCBM using a solution-processed titanium chelate
Authors: Zhang Wenqing , Tan Zhan'ao , Qian Deping , Li Liangjie , Xu Qi , Li Shusheng , Zheng Hua , Li Yongfang 
Published: June 2012

Abstract
We report efficient inverted polymer solar cells (PSCs) based on poly(3-hexylthiophene) (P3HT) and [6,6]-phenyl-C61-butyric acid methyl ester (PCBM) using alcohol-soluble titanium (diisopropoxide) bis (2,4-pentanedionate) (TIPD) as an electron selective layer between the indium tin oxide (ITO) electrode and the photoactive layer. The thermally annealed TIPD layer is highly transparent in the visible range and shows effective electron collection ability. By optimizing the electron-collecting layer, the photoactive layer and the hole-collecting layer, the power conversion efficiency (PCE) of the inverted device with the structure ITO/TIPD/P3HT : PCBM/MoO3/Ag reaches 4.10% under the illumination of AM1.5G, 100 mW cm−2, which is among the highest values for inverted PSCs based on P3HT : PCBM. The PCE of the inverted device is improved in comparison with the conventional device (3.77%) under the same experimental conditions.
Surface effect on the buckling of piezoelectric nanofilms
Authors: Zhang Jin , Wang Chengyuan , Adhikari Sondipon 
Published: June 2012

Abstract
A sandwich-plate model is developed to account for the effect of surface layers on the buckling of piezoelectric nanofilms (PNFs) due to an electrical voltage. The physical mechanisms of the surface effects are investigated and the contributions to the resultant effect are evaluated for surface piezoelectricity, surface stress and surface elasticity. It is found that the surface effect originates primarily from the residual surface stresses and enhanced piezoelectric coefficient due to the surface piezoelectricity. Its influence on the critical buckling voltage of a PNF depends sensitively on the thickness, the length-to-thickness ratio and the nature of residual surface stress. In addition, the intrinsic buckling may occur for a thin PNF where the relatively strong residual surface compression is achieved.
Enhanced formation of Ge nanocrystals in Ge : SiO2 layers by swift heavy ions
Authors: Antonova I V , Skuratov V A , Volodin V A , Smagulova S A , Marin D M , Janse van Vuuren A , Neethling J , Jedrzejewski J , Balberg I 
Published: June 2012

Abstract
In this paper we report the ability of swift heavy Xe ions with an energy of 480 MeV and a fluence of 1012 cm−2 to enhance the formation of Ge nanocrystals within SiO2 layers with variable Ge contents. These Ge-SiO2 films were fabricated by the co-sputtering of Ge and quartz sources which followed various annealing procedures. In particular, we found that the irradiation of the Ge : SiO2 films with subsequent annealing at 500 °C leads to the formation of a high concentration of nanocrystals (NCs) with a size of 2–5 nm, whereas without irradiation only amorphous inclusions were observed. This effect, as evidenced by Raman spectra, is enhanced by pre-irradiation at 550 °C and post-irradiation annealing at 600 °C, which also leads to the observation of room temperature visible photoluminescence.
Acoustic cloak/anti-cloak device with realizable passive/active metamaterials
Authors: Shen Huijie , Païdoussis Michael P. , Wen Jihong , Yu Dianlong , Cai Li , Wen Xisen 
Published: June 2012

Abstract
Utilizing the coordinate transformation method, together with exchange of variables between Maxwell's equations and the acoustic equations with axial-invariance in cylindrical coordinates, the acoustic parameters (anisotropic density and scalar bulk modulus) for an ideal cloak and an ideal anti-cloak are obtained. An anti-cloak allows the inside object to ‘see’ outside, but to be invisible from outside; whereas a cloak is invisible from outside, but ‘blind’ from inside. Utilizing a scattering algorithm developed in this paper, the pressure field calculation of the cloak/anti-cloak is performed and the concepts and characteristics of the acoustic cloak/anti-cloak are revisited. To be more easily achievable experimentally, a multilayered cloak/anti-cloak model with homogeneous isotropic materials is introduced, and its corresponding pressure distributions are calculated. Also, the total scattering cross-section curves for the multilayered cloak and anti-cloak over a certain frequency range are presented and compared. Finally, an active acoustic metamaterial made up of piezo-diaphragm cavity arrays is designed for the cloak/anti-cloak. Taking into account the coupling between adjacent cavity cells, a multi-control strategy for piezo-diaphragm cavity arrays is exploited, rendering possible wide ranges of effective densities and effective bulk moduli (or acoustic speeds), or even double-negative transformation medium (i.e. both density and bulk modulus parameters are negative). With such sets of active acoustic metamaterials, the cloak and anti-cloak may become both theoretically and experimentally realizable.
A case–control study of determinants for the occurrence of gouty arthritis in heart failure patients
Authors: Hueskes Berdine A.A. , Willems Frank F. , Leen Aukelien C. , Ninaber Patricia A. , Westra Richard , Mantel‐Teeuwisse Aukje K. , Janssens Hein J.E.M. , van de Lisdonk Eloy H. , Roovers Elisabeth A. , Janssen Matthijs 
Published: June 2012

AimsGouty arthritis is a frequent and disabling complication in heart failure patients. This study aimed to investigate which factors are associated with the occurrence of gouty arthritis in these patients.Methods and resultsA case–control study was performed in heart failure patients (February 2007 to October 2009). Cases were defined as patients with gouty arthritis. Factors that are possibly associated with gouty arthritis and/or heart failure were evaluated. Echocardiographic dimensions and laboratory values including glomerular filtration rate (GFR) and fractional excretion of uric acid (FEUA) were measured. Logistic regression analysis was used to determine crude and adjusted odds ratios (ORs) and 95% confidence intervals (CIs) for possibly associated factors. In total, 188 patients (59 with gouty arthritis) were included. Spironolactone use was associated with a decreased occurrence of gouty arthritis (OR 0.32, 95% CI 0.13–0.77). Independently associated with an increased occurrence of gouty arthritis were hypertensive heart failure (OR 3.6, 95% CI 1.6–8.2), New York Heart Association (NYHA) class III/IV (OR 4.5, 95% CI 1.8–11.0), lower GFR (P < 0.001), and FEUA <4% (OR 3.3, 95% CI 1.4–7.9). Among the four age/gender groups, the strongest association with gouty arthritis was found in men <65 years.ConclusionOur identification of factors that are associated with the occurrence of gouty arthritis makes it possible to develop strategies to improve further the quality of life in heart failure patients. The possible decreased occurrence of gouty arthritis in spironolactone users has to be confirmed in prospective studies.
Affordability of alcohol as a key driver of alcohol demand in New Zealand: a co‐integration analysis
Authors: Wall Martin , Casswell Sally 
Published: June 2012

AbstractAimsTo investigate whether affordability of alcohol is an important determinant of alcohol consumption along with price. This will inform effective tax policy to influence consumption.DesignCo‐integration analysis was used to analyse relationship between real price, affordability and consumption. Changes in retail availability of wine in 1990 and beer in 1999 were also included in the models. The econometric approach taken allows identification of short‐ and long‐term responses. Separate analyses were performed for wine, beer, spirits and ready‐to‐drinks (spirits based pre‐mixed drinks).SettingNew Zealand 1988–2011.MeasurementsQuarterly data on price and alcohol available for consumption for wine, beer, spirits and ready‐to‐drinks. Price data were analysed as: real price (own price of alcohol relative to the price of other goods) and affordability (average earnings relative to own price).FindingsThere was strong evidence for co‐integration between wine and beer consumption and affordability. There was weaker evidence for co‐integration between consumption and real price.ConclusionsThe affordability of alcohol is more important than real price in determining consumption of alcohol. This suggests that affordability needs to be considered by policy makers when determining tax and pricing policies to reduce alcohol‐related harm.
Impaired processing of emotion in music, faces and voices supports a generalized emotional decoding deficit in alcoholism
Authors: Kornreich Charles , Brevers Damien , Canivet Delphine , Ermer Elsa , Naranjo Cecilia , Constant Eric , Verbanck Paul , Campanella Salvatore , Noël Xavier 
Published: June 2012

AbstractAimTo test the generalized emotional decoding impairment hypothesis in alcoholism.DesignCross‐sectional behavioural study comparing emotion recognition conveyed by faces, voices and musical excerpts.SettingAlcohol detoxification unit of Brugmann University Hospital.ParticipantsTwenty‐five recently detoxified alcohol‐dependent patients were compared to 25 normal controls matched for sex, age and educational level.MeasurementsFrom faces, voices and musical excerpts, participants were instructed to rate the intensity of several emotions on a scale from 0 for ‘absent’ to 9 for ‘highly present’. Depression, anxiety and sustained/selective attention capacities were controlled for.FindingsAlcohol‐dependent patients were less accurate than controls in identifying the target emotion in faces (P < 0.001), voices (P < 0.001) and musical excerpts (P < 0.001).ConclusionsAlcohol‐dependent patients who are completing detoxification are impaired in recognizing emotions conveyed by faces, voices and music; these results suggest a generalized emotional decoding impairment. Hypothetically, deficits in the fronto‐parietal mirror neurone system could link all these disturbances together.