Miracle-dz/UniFAR-FTUs huggingface dataset:
ABCC6 Gene Analysis in 20 Japanese Patients with Angioid Streaks Revealing Four Frequent and Two Novel Variants and Pseudodominant Inheritance

Purpose To report the spectrum of ABCC6 variants in Japanese patients with angioid streaks (AS). Patients and Methods This was a single-center cohort study. The medical records of 20 patients with AS from 18 unrelated Japanese families were retrospectively reviewed. Screening of the ABCC6 gene (exons 1 to 31) was performed using PCR-based Sanger sequencing. Results Eight ABCC6 variants were identified as candidate disease-causing variants. These eight variants included five known variants (p.Q378X, p.R419Q, p.V848CfsX83, p.R1114C, and p.R1357W), one previously reported variant (p.N428S) of unknown significance, and two novel variants (c.1939C>T [p.H647Y] and c.3374C>T [p.S1125F]); the three latter variants were determined to be variants of significance. The following four variants were frequently identified: p.V848CfsX83 (14/40 alleles, 35.0%), p.Q378X (7/40 alleles, 17.5%), p.R1357W (6/40 alleles, 15.0%), and p.R419Q (4/40 alleles, 10.0%). The ABCC6 variants were identified in compound heterozygous or homozygous states in 13 of 18 probands. Two families showed a pseudodominant inheritance pattern. Pseudoxanthoma elasticum was seen in 15 of 17 patients (88.2%) who underwent dermatological examination. Conclusions We identified disease-causing ABCC6 variants that were in homozygous or compound heterozygous states in 13 of 18 families (72.2%). Our results indicated that ABCC6 variants play a significant role in patients with AS in the Japanese population.
ACTION POTENTIAL PARAMETERS IN DIFFERENT HUMAN MUSCLES

Clianges i i i duration, :iinl)litude, and shape of muscle action poteutials are used as signs of the degree and the localisation of involvement in neuroinuscular diseases. Normative values of these parameters are necessary. Previous papers dealing with the influence of soine physical and physiological factors on the action potential parameters concerned the brachial biceps only (Buchthal et al. 1954, b and c) . There are, however, differences in different muscles (PefersCn and Iiugelberg 1949 ; Jasper and Hallein 1949 ; Buchtlial and Pinelli 1951 : Feinstein et nl. 1954, a and h ) , and i t is the aim of the present study to provide data on different muscles for comparison with findings i n clinical electromyography.
Isolation and immunisation studies of a canine parco-like virus from dogs with haemorrhagic enteritis

A newly recognised canine parvo like virus was isolated from faeces of dogs with haemorrhagic enteritis. Cell cultures from several species were susceptible to it. Virus infected cells could be demonstrated by staining with fluorescent antibody reagents (prepared against canine virus or feline panleucopenia virus) or by haemagglutination with pig or rhesus monkey red blood cells. Inhibition of haemagglutination by specific antiserum prepared in specific-pathogen-free beagles provided a convenient method for viral identification. Experimental inoculation of specific-pathogen-free beagles resulted in elevated body temperatures and caused lymphopenia lasting one to three days. Feline panleucopenia virus vaccines protected dogs against challenge with virulent canine parvo-like virus.
Home and community-based service and other senior service use: Prevalence and characteristics in a national sample

ABSTRACT We report on the use of home and community-based services (HCBS) and other senior services and factors affecting utilization of both among Americans over age 60 in the Health and Retirement Study (HRS). Those using HCBS were more likely to be older, single, Black, lower income, receiving Medicaid, and in worse health. Past use of less traditional senior services, such as exercise classes and help with tax preparation, were found to be associated with current use of HCBS. These findings suggest use of less traditional senior services may serve as a “gateway” to HCBS that can help keep older adults living in the community.
Draft Genome Sequence and Comparative Analysis of the Superb Aromatic-Hydrocarbon Degrader Rhodococcus sp. Strain DK17

ABSTRACT Rhodococcus sp. strain DK17 is capable of utilizing various derivatives of benzene and bicyclics containing both aromatic and alicyclic moieties as sole carbon and energy sources. Here, we present the 9,107,362-bp draft genome sequence of DK17 and its genomic analysis in comparison with other members of the genus Rhodococcus.
Nutrition status of junior elite Canadian female soccer athletes.

CONTEXT Adolescent female team-sport athletes are faced with the challenge of meeting nutrition requirements for growth and development, as well as sport performance. There is a paucity of evidence describing the dietary adequacy of this population in respect to these physiological demands. Therefore, the aim of this study was to comprehensively evaluate the nutrition status of junior elite female soccer athletes. METHOD A total of 33 athletes (15.7 ± 0.7 yr) completed anthropometric assessment, 4-day food records analyzed for macro- and micronutrient intake, and hematological analysis. Energy expenditure was estimated using predictive equations. RESULTS Mean sum of 7 skinfolds was 103.1 ± 35.2 mm, and body-mass index was 22.7 ± 2.7. Mean energy intake was 2,079 ± 460 kcal/day, and estimated energy expenditure was 2,546 ± 190 kcal/day. Of the athletes, 51.5% consumed <5g/kg carbohydrate, 27.3% consumed <1.2g/kg protein, and 21.2% consumed <25% of energy intake from fat. A large proportion of athletes did not meet Dietary Reference Intakes for pantothenic acid (54.5%), vitamin D (100%), folate (69.7%), vitamin E (100%), and calcium (66.7%). Compared with recommendations for athletes, 89.3% and 50.0% of participants had depleted iron and 25-hydroxyvitamin D, respectively. CONCLUSION A high proportion of players were not in energy balance, failed to meet carbohydrate and micronutrient recommendations, and presented with depleted iron and vitamin D status. Suboptimal nutrition status may affect soccer performance and physiological growth and development. More research is needed to understand the unique nutrition needs of this population and inform sport nutrition practice and research.
Exploring the surgeon volume outcome relationship among women with breast cancer.

BACKGROUND A relationship between higher surgeon volume and lower mortality has been described for breast cancer, but selection bias has not been rigorously evaluated. We studied potential bias in the surgeon volume-outcome relationship by comparing the relationship of surgeon volume to breast cancer mortality and to mortality from other causes of death. METHODS We conducted an observational cohort study from tumor registry and Medicare claims data on 12 216 women, 66 years or older, with stage I or II breast cancer, who were operated on by 1856 surgeons. Breast cancer mortality and other-cause mortality were determined from death certificate sources and surgeon volume from Medicare claims. RESULTS Treatment by a high-volume surgeon was associated with younger patient age, white race, less comorbidity, and residence in a more affluent zip code. Patients treated by low-, medium-, and high-volume surgeons had small differences in breast cancer mortality (17.4, 15.7, and 13.0 deaths per 1000 person-years, respectively; P = .03) but larger differences in non-breast cancer mortality (46.0, 36.8, and 31.7 deaths per 1000 person-years, respectively; P < .001). After adjustment for multiple patient and disease factors, women treated by high-volume surgeons, compared with those treated by low-volume surgeons, were not less likely to die of breast cancer (relative risk, 0.94; 95% confidence interval, 0.76-1.16) but were significantly less likely to die of other causes (relative risk, 0.86; 95% confidence interval, 0.75-0.98). CONCLUSIONS The surgeon volume-outcome relationship for these patients with breast cancer was attributable not to mortality from breast cancer but to other causes of death. The lack of specificity of this relationship raises the possibility of selection bias as an explanatory factor.
The Economic Costs and Benefits of Self-Managed Teams Among Skilled Technicians

This paper estimates the economic costs and benefits of implementing teams among highly-skilled technicians in a large regional telecommunications company. It matches individual survey and objective performance data for 230 employees in matched pairs of traditionally-supervised and self-managed groups. Multivariate regressions with appropriate controls show that teams do the work of supervisors in 60-70% less time, reducing indirect labor costs by 75 percent per team. Objective measures of quality and labor productivity are unaffected. Team members receive additional overtime pay that represents a 4-5 percent annual wage premium, which may be viewed alternatively as a share in the productivity gains associated with innovation or as a premium for learning skills.
Cross‐national environmentalism differentials: Empirical evidence from core and noncore nations

This study explores cross‐national variation in measures of environmentalism in 24 developed and less‐developed countries. The commonly held Western stereotypes of Third World people as relatively less concerned about environmental issues, less aware of environmental problems, and less active in pro‐environmental activities are challenged. Using the Health of the Planet Survey (HOPS), analysis and findings suggest that the citizens of noncore nations demonstrate a higher degree of environmental consciousness, are actually more concerned about the environment, and perceive in‐country environmental health issues to be more serious relative to their counterparts in core nations. These findings suggest that postmaterialism is not necessarily the prime root of environmentalism as suggested in the literature. While the former is a cultural value reorientation evolving in postindustrial societies, the latter appears as a universal phenomenon with multifarious roots. The theoretical and policy implications of the.
[Study of serum albumin and BMI as nutritional markers in hemodialysis patients].

BACKGROUND Protein calorie malnutrition as well as systemic inflammation and metabolic disorders are common among patients with chronic renal failure undergoing renal replacement therapy (haemodialysis), which contributes to its morbidity and mortality. AIMS The aims of this work was to evaluate the nutritional status of patients in a hemodialysis treatment through the assessment of biochemical parameters nutritional as albumin, and anthropometric parameters of body mass index during ten years of follow up. METHODS In this work has been followed 90 patients of both sexes with chronic kidney disease who were treated with hemodialysis regularly on our unit for ten years. All patients were conducted quarterly measurements of plasma albumin (Alb), and other biochemical determinations, and anthropometric measurements of height, weight and body mass index calculated by the formula weight/height², grouped n BMI < 23 kg/m² and albumin levels <3.8 g/dl according to the consensus of the panel of experts of the International Society for renal Nutrition and metabolism. RESULTS During the 10 years all patients showed a significant decline in the biochemical parameters and the albumin, change in BMI does not presented significant changes in relation to malnutrition. CONCLUSIONS Malnutrition in patients on dialysis is a fact patent, BMI does not correspond with the biochemical parameters were observed, for what nutritional impairment in these patients is mainly expressed by serum albumin.
Increase in insulin-like growth factor 1 (IGF-1) and insulin-like growth factor binding protein 1 after supplementation with selenium and coenzyme Q10. A prospective randomized double-blind placebo-controlled trial among elderly Swedish citizens

Background Insulin-like growth factor-1(IGF-1) has a multitude of effects besides cell growth and metabolism. Reports also indicate anti-inflammatory and antioxidative effects. The concentrations of IGF-1 decrease with age and during inflammation. As selenium and coenzyme Q10 are involved in both the antioxidative defense and the inflammatory response, the present study aimed to examine the effects of supplementation with selenium and coenzyme Q10 on concentrations of IGF-1 and its binding protein IGFBP-1 in a population showing reduced cardiovascular mortality following such supplementation. Methods 215 elderly individuals were included and given the intervention for four years. A clinical examination was performed and blood samples were taken at the start and after 48 months. Evaluations of IGF-1, the age adjusted IGF-1 SD score and IGFBP-1 were performed using group mean values, and repeated measures of variance. Findings After supplementation with selenium and coenzyme Q10, applying group mean evaluations, significantly higher IGF-1 and IGF-1 SD scores could be seen in the active treatment group, whereas a decrease in concentration could be seen of the same biomarkers in the placebo group. Applying the repeated measures of variance evaluations, the same significant increase in concentrations of IGF-1 (F = 68; P>0.0001), IGF-1 SD score (F = 29; P<0.0001) and of IGFBP-1 (F = 6.88; P = 0.009) could be seen, indicating the effect of selenium and coenzyme Q10 also on the expression of IGF-1 as one of the mechanistic effects of the intervention. Conclusion Supplementation with selenium and coenzyme Q10 over four years resulted in increased levels of IGF-1 and the postprandial IGFBP-1, and an increase in the age-corrected IGF-1 SD score, compared with placebo. The effects could be part of the mechanistic explanation behind the surprisingly positive clinical effects on cardiovascular morbidity and mortality reported earlier. However, as the effects of IGF-1 are complex, more research on the result of intervention with selenium and coenzyme Q10 is needed.
The contours of civic and ethnic national identification in Ukraine

IN ALL NATION-STATES national identity has both a quantitative and a qualitative component. Quantitatively, one may speak of the strength of national identification. This refers to the degree to which people in a nation-state identify themselves as members of the national community and feel positively toward it. Qualitatively, one can analyse the reasons why people in a nation-state feel they form a community. In this sense the content of national identity refers to the traits that simultaneously unify people into a nation and distinguish them from other nations. On the basis of these traits, boundaries between the national in-group and out-groups are defined, thereby creating criteria for national membership. Scholars of ethnic politics and nationalism have long identified two basic forms of this qualitative component of national identity-civic and ethnic. With civic nationalism, people in a nation-state think that what can, does or should unite and distinguish all or most members of the nation are such features as living on a common territory, belief in common political principles, possession of state citizenship, representation by a common set of political institutions and desire or consent to be part of the nation. With ethnic nationalism, the people think that what can, does or should unite and distinguish them are such features as common ancestry, culture, language, religion, traditions and race. All scholars recognise that national identities will be based on some combination of civic and ethnic elements, but they assert that the relative strength of the civic and ethnic components can vary from nation to nation. One widespread argument is that ethnic nationalism is dominant in Eastern Europe and the former Soviet Union, while civic nationalism is dominant in Western Europe and the United States.
Poverty, adolescent well‐being and outcomes later in life

This paper investigates the impact of various factors in childhood (such as poverty, parental guidance, educational orientation, self-esteem and delinquent behaviour) on outcomes later in life. Using data from the British Household Panel Survey which incorporates a survey of children aged 11–15 years (also known as the British Youth Panel (BYP)) the technique of Structural Equation Modelling is employed to show the relative impact of these factors on educational attainment and employment status when the respondents were in their late 20s. Copyright (C) 2010 John Wiley & Sons, Ltd.
Political Campaign Communication: Inside and Out

I. AN OVERVIEW OF POLITICAL COMMUNICATION ISSUES. 1. Political Communication: An Introduction. 2. Political Socialization: The Development of Political Attitudes. 3. Campaign Strategies. 4. The Image of the Political Candidate. 5. Media Theory and Political Communication. II. THE CAMPAIGN TEAM. 6. Campaign Organization. 7. Campaign Communications in the Mass Media. 8. Campaign Communications: Direct Voter Contact. 9. Political Speeches. 10. Political Polling. III. CAMPAIGN CONCERNS. 11. Press Coverage and Media Relations. 12. The Role of Political Money. 13. The Role of Interpersonal Influence. 14. Critical Events Analysis. 15. Ethical Questions in Political Communication. References. Index.
Friends from Afar: The Taiping Rebellion, Cultural Proximity and Primary Schooling in the Lower Yangzi, 1850-1949

This paper tests the hypothesis that the cultural distance between migrants and natives impedes the provision of public goods. The Taiping Rebellion was a shock that caused groups without a history of shared governance to be relocated to the same region. We use a unique historical dataset of surnames in the Lower Yangzi of China to construct a measure of the cultural distance between migrants and natives (MNCD). We find that a one-standard-deviation increase in the MNCD is associated with a decrease of over 0.19 public primary schools per 10,000 persons in the early twentieth century. The results survive various robustness checks and an instrumental variable analysis that exploits the pre-existing cultural distances between the native and the nearby population. Evidence from the timing of when the MNCD takes effect suggests that the primary mechanism runs from migrant-native cultural distance through quality of collective decision-making to modern primary education.
Journal of Agricultural & Food Industrial Organization Picking Apples : Can Multi-Attribute Ecolabels Compete ?

Global food markets in Europe, the U.S. and elsewhere, are experiencing a rapid growth in the number of private party and government environmental labeling programs. Most current ecolabels are defined by standards related to multiple environmental practices. This study presents an analysis of consumers’ choice of food products, in this case apples with or without ecolabels, where the ecolabels present varying combinations of farm practices with implications for environmental quality. These practices include: whether or not standards are met specific to on-farm pest management; presence of stream or groundwater quality protection; presence of on-farm wildlife habitat provision; and which certifier provides the guarantee. Factors influencing consumer preferences for ecolabel attributes are evaluated as a choice-based conjoint analysis. To empirically test the effect of heterogeneity of consumers on preferences for ecolabel attributes, surveys were conducted in a stratified sample in three regions (Portland, Oregon; Minneapolis, Minnesota; Rhode Island) with a focus on sampling across shoppers at different types of markets including conventional supermarkets, farmers markets, natural food stores and food co-ops. Results show that preferences for ecolabels are most strongly driven by type of pesticide usage, in particular for non-synthetic pesticides which were identified with organic production. With an appropriate price premium, ecolabels with an alternative pest management practice and other environmental practices were preferred to conventionally produced apples. These results varied according to age and gender of respondents, and type of store at which respondents shopped.
Persistent pulmonary hypertension of the newborn infant. Comparison of conventional versus extracorporeal membrane oxygenation in neonates fulfilling Bartlett's criteria.

Persistent pulmonary hypertension of the newborn (PPHN) is a challenge for the neonatologist and a common indication for treatment with extracorporeal membrane oxygenation (ECMO) when medical management fails. We observed 132 neonates born between January 1985 and December 1988 with the diagnosis of persistent pulmonary hypertension of the newborn: 73 (55%) met the Bartlett criteria for treatment with ECMO with 80% predicted mortality; 21 (29%) deteriorated despite conventional medical treatment, were thought to be dying, and were sent for ECMO. Among the 52 patients who were medically treated 40 (77%) survived, a marked difference compared with a predicted 20% survival. All ECMO-treated neonates survived. Although conventionally treated infants showed a trend toward less dependence on supplemental oxygen at > 28 days of life, this study failed to detect a significant difference between those two groups. We conclude that mortality was lower for ECMO-treated infants than for those who were medically treated (0 of 21 vs 12 of 52, p < 0.05); mortality for infants with persistent pulmonary hypertension of the newborn who met Bartlett's criteria and were medically treated was lower than published data; and there was no significant difference in oxygen dependence at > 28 days between the survivors who received ECMO and those who received medical therapy.
An Optimized Least-Squares Technique for Improving Ambiguity Resolution and Computational Efficiency

Donghyun Kim is a post-doctoral fellow in the Department of Geodesy and Geomatics Engineering at the University of New Brunswick (UNB), where he has been developing a new on-the-fly (OTF) ambiguity resolution technique for long baselines. He received a B.Sc., M.S. and Ph.D. from Seoul National University. He has been involved in GPS research since 1991 and is a member of the IAG Special Study Group “Wide Area Modeling for Precise Satellite Positioning”.
Detection of immune system cells in paraffin wax-embedded ovine tissues.

This report describes a method (fixation, paraffin wax-embedding and immunolabelling) for the demonstration of several immune system cell epitopes (CD1, CD2, CD4, CD8, CD14, CD21, CD45R, WC-1, 28 kDa surface antigen, immunoglobulins and MHC II antigens) in ovine lymph nodes collected at necropsy. Cell surface epitopes considered to be sensitive to processing methods were successfully demonstrated by a procedure that included the use of a non-aldehyde-containing, zinc salts-based fixative, coupled with a sensitive system of immunolabelling. This novel method had the advantage of avoiding antigen-retrieval steps and of providing consistently good morphological definition.
Why peg? The role of capital mobility and financial intermediation

Many economists argue that the growth of international capital mobility has made the maintenance of pegged exchange rates more costly, forcing developing states to choose alternative arrangements. But some states do not simply abandon pegged exchange rates as their exposure to capital mobility rises. Some states abandon pegs long before a crisis can erupt, while others maintain pegs until the speculative pressures became unbearable. Why, in an environment of growing capital mobility, do some states maintain pegs longer than others do? One reason is that the more that bank lending dominates investment in a country, the more likely that state is to hold on to a pegged exchange rate. When banks have accumulated significant amounts of foreign debt they lobby for exchange rate stability. In a bank‐dominated financial system, a concentrated banking sector can organize easily and use its crucial role in the economy to exert influence over economic policy. This article presents new evidence from statistical tests on 61 developing countries that confirm that states with deeper banking systems are more likely to peg their exchange rates, in spite of growing capital mobility.