bluuebunny/crossref_metadata_2025_split huggingface dataset:
Speech characteristics in the congenital and childhood‐onset forms of myotonic dystrophy type 1
Authors: Sjögreen Lotta , Mårtensson Åsa , Ekström Anne‐Berit 
Published: January 2018

AbstractBackgroundMyotonic dystrophy type 1 (DM1) is a slowly progressive multi‐systemic disease with an autosomal‐dominant inheritance caused by a mutation on chromosome 19 (19q13.3).AimsTo explore speech characteristics in a group of individuals with the congenital and childhood‐onset forms of DM1 in terms of intelligibility, speech‐sound production, nasality and compensatory articulation. A further aim was to analyse whether speech characteristics were correlated to subforms of DM1 and if speech outcome could be related to muscle strength.Methods & ProceduresFifty native Swedish speakers (7–29 years old) with the congenital and childhood‐onset forms of DM1 and 13 healthy controls participated in the study. The intelligibility of spontaneous speech, speech‐sound production — single‐word and sentence repetition — including percentage consonants correct (PCC) and compensatory articulation, were evaluated by speech–language pathologists from video recordings. A nasometer and lip‐force meter were used for objective evaluations of nasality and orofacial strength.Outcomes & ResultsIn severe (n = 9) and mild congenital DM1 (n = 13), all participants had impaired intelligibility to some degree, while this applied to 79% of those with childhood DM1 (n = 28). PCC for bilabials were 53.9% in severe congenital DM1, 57.4% in mild congenital DM1 and 85.3% in childhood DM1; the corresponding results for dentals were 69.3%, 59.2% and 87.3%. Bilabials were most often compensated for with interdental or labiodental articulation. Dentals were substituted with interdental articulation. Velars were seldom affected. The mean nasalance score was high in the study group compared with controls and with normative data and the majority had weak lips. Maximum lip force, as well as the mean nasalance score, correlated significantly with the intelligibility score.Conclusions & ImplicationsThe deviant production of bilabial consonants, interdental articulation and hypernasal speech are characteristic features of dysarthria in congenital and childhood DM1. Dysarthria is more frequent and more severe in congenital DM1 compared with childhood DM1. Most individuals with congenital DM1 and childhood‐onset DM1 will need speech therapy from a young age. For some children with incomprehensible speech or severe neurodevelopmental disorders, alternative and augmentative ways of communication will be part of the treatment.
Systematic review with meta‐analysis: effectiveness and tolerability of interferon‐free direct‐acting antiviral regimens for chronic hepatitis C genotype 1 in routine clinical practice in Asia
Authors: Ji F. , Wei B. , Yeo Y. H. , Ogawa E. , Zou B. , Stave C. D. , Li Z. , Dang S. , Furusyo N. , Cheung R. C. , Nguyen M. H. 
Published: January 2018

SummaryBackgroundDirect‐acting antiviral (DAA) regimens have shown high efficacy and tolerability for patients with HCV genotype 1/1b (GT1/1b) in clinical trials. However, robust real‐world evidence of interferon (IFN)‐free DAA treatment for HCV GT1‐infected patients in Asia is still lacking.AimTo systematically review and meta‐analyse the effectiveness and tolerability of IFN‐free DAA therapy for HCV GT1 infection in Asia.MethodsWe included studies that enrolled adult patients with HCV GT1 infection in routine clinical practice in Asia, using IFN‐free DAA regimens, and reported sustained virological response (SVR) after 12/24 weeks end‐of‐treatment by 31 May 2017. The pooled SVR rates were computed with a random‐effects model. Subgroup analysis and meta‐regression as previously registered in PROSPERO were performed to determine how pre‐planned variables might have affected the pooled estimates.ResultsWe included 41 studies from eight countries and regions, comprising of 8574 individuals. The pooled SVR rates for GT1 were 89.9% (95% CI 88.6‐91.1, I2 = 55.1%) with daclatasvir/asunaprevir (DCV/ASV) and 98.1% (95% CI 97.0‐99.0, I2 = 41.0%) with ledipasvir/sofosbuvir ± ribavirin (LDV/SOF ± RBV). Baseline cirrhosis but not prior treatment history and age, attenuated the effectiveness of both regimens. Baseline resistance associated substitutions (RASs) severely attenuated SVR of DCV/ASV (65.4% vs 94.3%, P < 0.001) and only minimally with LDV/SOF ± RBV (94.5% vs 99.2%, P = 0.003). Patients with renal dysfunction treated with DCV/ASV showed a higher SVR rate (93.9% vs 89.8%, P = 0.046). Patients with hepatocellular carcinoma (HCC) LDV/SOF ± RBV achieved a lower SVR than those without HCC (94.1% vs 98.7%, P = 0.001).ConclusionAll oral DAA treatment of HCV GT1 resulted in high cure rates in Asian patients in routine clinical practice setting including elderly patients and those with end‐stage renal disease.
The Economic Benefits of Irrigation Districts under Prior Appropriation Doctrine: An Econometric Analysis of Agricultural Land‐Allocation Decisions
Authors: Ji Xinde , Cobourn Kelly M. 
Published: January 2018

AbstractThe economic literature has established that prior appropriation doctrine induces heterogeneity in risk among water users, which leads to an inefficient allocation of resources. In this study, we show that irrigation districts alleviate that risk by deviating from the strict application of prior appropriation doctrine. As a result, farmers inside irrigation districts are able to plant more water‐intensive crops than farmers outside irrigation districts, which increases average profitability. We empirically examine this hypothesis by leveraging a georeferenced panel data set at the spatial scale of the individual water right and spanning 2007–14 in Idaho's Eastern Snake River Plain. Our results indicate that on average, irrigation districts allocate larger portions of their land to drought‐sensitive, high‐value crops such as sugar beets and potatoes. As a result of differences in planting decisions, members of irrigation districts earn on average $16.20 per acre, or 6.0% more per year than those outside of irrigation districts.
P‐wave and interatrial block: New predictor for atrial high rate episodes in patients with cardiac implantable electronic devices
Authors: Rubio Campal José M. , Benezet‐Mazuecos Juan , Iglesias Bravo José Antonio , Sánchez Borque Pepa , Miracle Blanco Ángel , de la Vieja Alarcón Juan José , Martínez Mariscal Jaime , Baranchuk Adrian M. , Farré Muncharaz Jerónimo 
Published: January 2018

AbstractBackgroundThe presence of interatrial block (IAB) is associated with the development of atrial fibrillation (AF). The aim of this study was to determine whether P‐wave duration and presence of IAB before the implantation of a cardiac implantable electronic device (CIED) are associated with the presence of atrial high rate episodes (AHRE), during long‐term follow‐up.Methods380 patients (57% men; 75 ± 10 years) were included. IAB was defined according to the International Consensus Criteria. AHRE was defined as an episode of atrial rate ≥225 beats/min with a minimum duration of 5 minutes.ResultsDocumented paroxysmal AF before the implantation was present in 24% of the patients; 80% had hypertension and 32% structural heart disease. Mean P‐wave duration was 123 ± 23 ms, and 39% of the patients had IAB (32% partial, 7% advanced). After a mean follow‐up of 18 ± 12 months, 33% of the patients presented AHRE. Patients with AHRE had a P‐wave duration significantly longer (130 ± 24 ms vs 119 ± 21 ms; P < 0.001) and a greater prevalence of IAB (53% vs 32%; P < 0.001). In a multivariate analysis, predictors of AHRE were: IAB (odds ratio [OR] 2.1; 95% confidence interval [CI] [1.3–3.4], P < 0.001) and previous paroxysmal AF (OR 2.6; 95% CI [1.5–4.3], P < 0.001). In patients without previous AF, the presence of IAB was also a significant predictor of AHRE (OR 3.1; 95% CI [1.8–5.5], P < 0.001).ConclusionsIAB is a strong predictor of AHRE in patients with CIED. This finding is independent of the presence of prior paroxysmal AF.
Long‐term effects of intralymphatic immunotherapy (ILIT) on canine atopic dermatitis
Authors: Timm Katrin , Mueller Ralf S. , Nett‐Mettler Claudia S. 
Published: January 2018

BackgroundSubcutaneous allergen immunotherapy (SCIT) is an established and efficacious therapy for canine atopic dermatitis (AD). In humans, intralymphatic immunotherapy (ILIT) was reported to be associated with fewer and less severe adverse effects than subcutaneous allergen immunotherapy and to be efficacious for several years after three intralymphatic injections.ObjectiveTo evaluate safety and effects ofILITin a case series of dogs with (AD).AnimalsFifty one privately owned dogs withAD.MethodsDogs received injections of 0.2 mL alum‐precipitated allergen extract into the popliteal lymph nodes at monthly intervals for 3–5 months. Lesion scores, pruritus and medication scores were determined before and at three and 12 months after beginning immunotherapy, and compared in a per protocol analysis (PP) and an intention‐to‐treat analysis (ITT).ResultsTwenty two dogs completed the study and 29 dogs did not fulfil study completion criteria due to lack of a final study visit (21 of 29) or due to insufficient improvement (14 of 29). All scores improved during the study with both analyses. For pruritus and Quality of Life scores this improvement was significant with both analyses; for Canine Atopic Dermatitis Extent and Severity Index (CADESI)‐03 values and medication scores only withPP. The only rare adverse effects observed included mild swelling of the lymph node post‐injection and increased pruritus.Conclusion and clinical relevanceILITis safe and feasible, and provides long‐lasting relief in some atopic dogs with a limited number of injections.
Origins of tobacco harm reduction in the UK: the ‘Product Modification Programme' (1972–1991)
Authors: Elias Jesse , Ling Pamela M 
Published: January 2018

ObjectiveTo better understand the current embrace of long-term nicotine maintenance by British governmental agencies and tobacco harm reduction by several leading British public health organisations, describe the context and deliberations of the UK’s first formal tobacco risk reduction programme: ‘Product Modification’.MethodsAnalysis of previously secret tobacco industry documents, news archives and Parliamentary debate records.ResultsFrom 1972 to 1991, the British government sought to investigate safer smoking through the ‘product modification programme'. The Independent Scientific Committee on Smoking and Health (ISCSH) advised the British government on these efforts and collaborated with the tobacco industry, with which government then negotiated to determine policy. The ISCSH operated from four industry-backed premises, which contributed to the ISCSH’s support of safer smoking: (1) reduced toxicity indicates reduced risk; (2) collaboration with the tobacco industry will not undermine tobacco control; (3) nicotine addiction is unavoidable; (4) to curtail cigarette use, solutions must be consumer-approved (ie, profitable). These premises often undermined tobacco control efforts and placed the ISCSH at odds with broader currents in public health. The product modification programme was abandoned in 1991 as the European Community began requiring members to adopt upper tar limits, rendering the ISCSH redundant.Policy implicationsEndorsements of reduced harm tobacco products share the same four premises that supported the product modification programme. Current tobacco harm reduction premises and policies supported by the British government and leading British public health organisations may reflect the historical influence of the tobacco industry.
Patient predictors of response to cognitive behaviour therapy and schema therapy for depression
Authors: Carter Janet D , McIntosh Virginia VW , Jordan Jennifer , Porter Richard J , Douglas Katie , Frampton Christopher M , Joyce Peter R 
Published: January 2018

Objective: Few studies have examined differential predictors of response to psychotherapy for depression. Greater understanding about the factors associated with therapeutic response may better enable therapists to optimise response by targeting therapy for the individual. The aim of the current exploratory study was to examine patient characteristics associated with response to cognitive behaviour therapy and schema therapy for depression. Methods: Participants were 100 outpatients in a clinical trial randomised to either cognitive behaviour therapy or schema therapy. Potential predictors of response examined included demographic, clinical, functioning, cognitive, personality and neuropsychological variables. Results: Individuals with chronic depression and increased levels of pre-treatment negative automatic thoughts had a poorer response to both cognitive behaviour therapy and schema therapy. A treatment type interaction was found for verbal learning and memory. Lower levels of verbal learning and memory impairment markedly impacted on response to schema therapy. This was not the case for cognitive behaviour therapy, which was more impacted if verbal learning and memory was in the moderate range. Conclusion: Study findings are consistent with the Capitalisation Model suggesting that therapy that focuses on the person’s strengths is more likely to contribute to a better outcome. Limitations were that participants were outpatients in a randomised controlled trial and may not be representative of other depressed samples. Examination of a variety of potential predictors was exploratory and requires replication.
Legibility of Text and Pictograms in Variable Message Signs: Can Single-Word Messages Outperform Pictograms?
Authors: Roca Javier , Insa Beatriz , Tejero Pilar 
Published: January 2018

Objective: The current research shows the advantage of single-word messages in the particular case of variable message signs (VMSs) with a high aspect ratio. Background: Early studies on traffic sign design proposed that pictorial information would advantage equivalent text messages in static signs. Method: We used a driving simulator to present individually 36 VMSs, showing six words (e.g., “congestion”) and six danger signs (e.g., congestion traffic sign). In Experiment 1, 18 drivers read aloud the text or orally identified the pictograms as soon as they could correctly do it. In Experiment 2, a different sample of 18 drivers gave a motor response, according to the meaning of the message. We analyzed the legibility distance and accuracy, driving performance (speed variability), and glance behavior. Results: Our results show that single-word messages were associated with better performance (farther reading distances) and required less visual demands (fewer glances and less glancing times) than pictograms. Conclusion: As typical configurations of VMSs usually have a high aspect ratio, and thus allow large character heights, single-word messages can outperform the legibility of pictograms. However, the final advantage of text or pictorial messages would depend on several factors, such as the driver’s knowledge of the language and the pictogram set, the use of single or multiple words, the particular design and size of critical details in letters and pictograms, environmental factors, and driver age. Application: Potential applications include the design of VMSs and other devices aimed at displaying text and/or pictograms with a high aspect ratio.
User satisfaction and energy use behavior in offices in Qatar
Authors: Indraganti Madhavi , Boussaa Djamel , Assadi Somayeh , Mostavi Ehsan 
Published: January 2018

The occupant behavior significantly contributes to the total energy use in buildings and its understanding is greatly needed in energy analysis/simulation studies. Uncertainties about the occupant behavior adversely affect the building performance predictability of the simulation models. As field data in Qatar on occupant energy use and behavior was unavailable, we conducted an environmental satisfaction and energy use survey in the city of Doha, Qatar in seven office buildings. Building occupants were highly satisfied with their work environments with respect to most of the environmental parameters excepting acoustics. Access to operable controls such as windows, thermostats improved their satisfaction. Subjects in private offices were more satisfied with their work environments. We noted the self-declared productivity of respondents to be high in general. It was significantly higher in offices with better occupant access to temperature controls. Noise level dissatisfaction was the highest in high partitioned cubicle offices and satisfaction was high in open plan offices. These findings provide vital design direction for new offices and environmental systems design and for managerial motivational campaigns. Practical application: Using field study data, we analyzed the occupant behavior and environmental satisfaction in offices in Doha, Qatar. The Middle East in general and Qatar in particular are known to consume enormous energy for buildings leaving a large ecological footprint. The finding of this study informs the designers of air-conditioning systems about the user behavioral patterns. Further, these findings help the building managers in designing targeted energy awareness/saving campaigns, as user motivation is vital in energy saving.
Clinical Evaluation of Internal Hemodiafiltration (iHDF): A Diffusive-Convective Technique Performed with Internal Filtration Enhanced High-Flux Dialyzers
Authors: Lucchi L. , Fiore G.B. , Guadagni G. , Perrone S. , Malaguti V. , Caruso F. , Fumero R. , Albertazzi A. 
Published: January 2018

Aim Efficiency in removing middle molecules such as ß2-microglobulin (ß2-MG) is one of the main purposes of modern dialytic therapy. In order to achieve this, techniques requiring complex machines and substitution fluid have been developed over recent years. Alternatively, the internal filtration / back filtration phenomenon can be used. The recent development of a so-called “internal filtration enhanced dialyser” prompted us to compare the removal of ß2-MG together with other small molecules when the dialyser was used either in standard hemodiafiltration (HDF) or internal hemodiafiltration (iHDF). Methods Ten stable, anuric, hemodialysis (HD) patients treated by thrice weekly standard bicarbonate HD using low-flux synthetic membrane entered the study. A new high-flux polysulfone dialyser designed with the specific aim of enhancing internal filtration (BS-1.6 UL, 1.6 m2, Toray Industries) was used. Post dilution HDF (2.5 l/hour of substitution fluid, dialysate flow 500 ml/min) was compared with iHDF (dialysate flow 750 ml/min), with blood flow at 300 ml/min. Samples were obtained at the start and at the end of the session in order to measure the % removal of urea, creatinine, uric acid, phosphate and ß2-MG (corrected for total protein concentration). In addition, after 20 min of dialysis the clearances of the same molecules were measured. A mathematical model has been developed for the description of the hydrodynamic phenomena taking place within the dialyser and of fluid filtration across the membrane. Results No significant differences have been observed in removal rate switching from HDF to iHDF except for ß2-MG removal, which was slightly higher in HDF than in iHDF. Phosphate clearance is significantly higher than those obtained with creatinine in both HDF (p<0.005) and iHDF (p<0.01) modalities. The total convection calculated with the model is reduced with respect to HDF only by 24% (4100 ml/h vs. 5400 ml/h on the average). Conclusions iHDF is a high flux dialysis method, which, if performed with a dialyser designed to enhance internal filtration, obtains a much higher removal rate in comparison with dialysers in traditional high flux dialysis, as previously reported in the literature. Provided that the dialyser is used on a dialysis machine working with ultra pure dialysate and UF control, this dialyser line can perform reliable internal HDF without the need for replacement solution. Considering the narrow difference in performance observed between iHDF and HDF, and the increasing number (and age) of patients leading to higher dialysis costs, iHDF represents a cost-effective alternative to other diffusive-convective techniques.
Bilirubin-Adsorption in 23 Critically Ill Patients with Liver Failure
Authors: Senf R. , Klingel R. , Kurz S. , Tullius S. , Sauer I. , Frei U. , Schindler R. 
Published: January 2018

Purpose Previous studies suggest that high levels of bilirubin exert cytotoxic, neurotoxic and encephalopathic effects that themselves may lead to further deterioration of liver function and multiorgan failure. Although extracorporeal BA is not a causal therapy, there are case reports of clinical benefits of BA. The present retrospective study investigated the clinical utility and effectiveness of BA in 23 patients with liver failure. Methods Twenty-three patients (61±11 years) with excessive hyperbilirubinemia (>25 mg/dL) after liver transplantation (n=7), partial liver resection (n=12) and others (n=4) were treated with BA (3.6 liters plasma per BA, BR350, Asahi) and followed for 45±8 days. Results A mean of 6.6 treatments (3–16) were performed per patient. On average, a single BA treatment reduced bilirubin-levels from 31±12 to 23.7±9 mg/dL (p<0.001). Levels of bile acid were reduced from 41.8±6 to 33.5±5 mg/dL. The 30-day mortality was 50%. BA was able to halt and stabilize the progressive increase in bilirubin levels in all patients. In contrast to survivors, non-survivors were characterized by a repeated rapid rise in bilirubin levels after cessation of BA treatment. Conclusions BA is able to stabilize or decrease bilirubin levels in patients with liver failure. Our experience suggests that BA is a safe and promising short-term treatment option for patients with acute deterioration of hepatic function.
Cardiac Calcium Evaluation in Hemodialysis Patients with Multisection Spiral Computed Tomography
Authors: Splendiani G. , Morosetti M. , Manni M. , Jankovic L. , Naticchia A. , Sturniolo A. , Tullio T , Balducci A. , Coen G. 
Published: January 2018

Aim The aim of this study is cardiac calcium content evaluation in hemodialysis patients by a new technique, based on ultrafast multisection CT (MTC). Methods The study was carried out on 30 HD patients, 14F and 16 M, average age 57.7±13.9 years, average HD age 57.3±47.4 months. The intact PTH levels were 625.4±571 pg/mL. Serum calcium, phosphate and CaxP product were 9.75±0.84 mg/mL, 6.21±1.01 mg/dL and 60.2±10.7 mg2/dL2, respectively. Results The values obtained with the MTC technique were reported in terms of Agatson scores. Score values frankly in the pathologic range (>100) were found in 24 patients (80%). Correlation analysis has shown positive and significant correlation coefficients of the score with patients’ age (p=0.003), serum calcium (p=0.012), CaxP (p=0.015), iPTH (=0.049), and borderline, to HD age (p=0.06). Conclusion Risk factors for cardiac calcification are mainly age, degree of hyperparathyroidism, increased CaxP and serum calcium levels. A control of calcium phosphate parameters in hemodialysis patients seems to be mandatory to avoid increased severity of coronary artery disease.