jimnoneill/paper-to-field-training huggingface dataset:
<scp><i>HAX1</i></scp>‐related congenital neutropenia: Long‐term observation in paediatric and adult patients enrolled in the European branch of the Severe Chronic Neutropenia International Registry (<scp>SCNIR</scp>)

Summary HAX1 ‐related congenital neutropenia ( HAX1 ‐CN) is a rare autosomal recessive disorder caused by pathogenic variants in the HAX1 gene. HAX1 ‐CN patients suffer from bone marrow failure as assessed by a maturation arrest of the myelopoiesis revealing persistent severe neutropenia from birth. The disorder is strongly associated with severe bacterial infections and a high risk of developing myelodysplastic syndrome or acute myeloid leukaemia. This study aimed to describe the long‐term course of the disease, the treatment, outcome and quality of life in patients with homozygous HAX1 mutations reported to the European branch of the Severe Chronic Neutropenia International Registry. We have analysed a total of 72 patients with different types of homozygous ( n = 68), compound heterozygous ( n = 3), and digenic ( n = 1) HAX1 mutations. The cohort includes 56 paediatric (&lt;18 years) and 16 adult patients. All patients were initially treated with G‐CSF with a sufficient increase in absolute neutrophil counts. Twelve patients required haematopoietic stem cell transplantation for leukaemia ( n = 8) and non‐leukaemic indications ( n = 4). While previous genotype–phenotype reports documented a striking correlation between two main transcript variants and clinical neurological phenotypes, our current analysis reveals novel mutation subtypes and clinical overlaps between all genotypes including severe secondary manifestations, e.g., high incidence of secondary ovarian insufficiency.

Topic: Blood disorders and treatments
Subfield: Genetics
Field: Biochemistry, Genetics and Molecular Biology
Domain: Life Sciences
Introduction

Alexander Akin examines how the expansion of publishing in the late Ming dynasty prompted changes in the nature and circulation of cartographic materials in East Asia. Focusing on mass-produced printed maps, East Asian Cartographic Print Culture: The Late Ming Publishing Boom and its Trans-Regional Connections investigates a series of pathbreaking late sixteenth- and early seventeenth-century works in genres including geographical education, military affairs, and history, analysing how maps achieved unprecedented penetration among published materials, even in the absence of major theoretical or technological changes like those that transformed contemporary European cartography. By examining contemporaneous developments in neighboring Choson Korea and Japan, this book demonstrates the crucial importance of considering the East Asian sphere in this period as a network of communication and publication, rather than as discrete national units with separate cartographic histories. It also reexamines the Jesuit printing of maps on Ming soil within the broader context of the local cartographic publishing boom and its trans-regional repercussions.

Topic: Chinese history and philosophy
Subfield: Sociology and Political Science
Field: Social Sciences
Domain: Social Sciences
Making Tibetans in China

Tibet is required by China to have an education system that popularizes basic education, and also socializes Chinese citizens into a political discourse consonant with the state program to build a harmonious society (Wang & Lou, 2007; Wu, 1995, 1999). The extent to which this so-called civilizing mission occurs is reflected in enrolment rates, school curriculum, the medium of instruction, and preferential treatment policies. Yet, the success or failure of this major effort hinges to a great extent upon how much and how many Tibetan households become persuaded by the logic of modern state schooling and the value of schools for survival within an expansive market economy (Postiglione, Jiao, & Gyatso, 2006). New patterns of population mobility, increased flows of information from outside Tibet, and rapid economic change have had a profound effect on the transmission and replication of traditional culture. Views differ about the way this transmission process has been managed and directed (Dorjee & Giles, 2005; Sautman & Dreyer, 2005). Nevertheless, the expansion of educational opportunities has made Tibetans more like Chinese, though no less Tibetan, as ethnic identities are remodeled by state schooling and responded to by local communities. Regardless of the cultural transformations taking place in Tibet, the analysis of the education system, policies, programs, problems, and practices has to be based on the realities of students, households, schools, teachers, and communities. This chapter discusses several questions: How are Tibetans being educated? What and how do Tibetans learn in China's schools? How do nomadic Tibetan communities adapt to state schools? How do schools remake Tibetans? What are the views of elite students about their education? This chapter focuses on the Tibetan Autonomous Region (TAR), where Tibetans constitute virtually all of the rural and nomadic population. 1 It provides a brief background review of the policies that shape education in the TAR, and results of research on the neidi or dislocated secondary schools for Tibetans in urban Chinese cities. It argues that, while enrollment rates continue to rise, most schooling produces mixed results, in terms of providing a quality learning environment that can propel academic achievement to a level comparable with the national average, as well as foster a harmonious multiculturalism.

Topic: China's Ethnic Minorities and Relations
Subfield: Sociology and Political Science
Field: Social Sciences
Domain: Social Sciences
CCDC 1493000: Experimental Crystal Structure Determination

An entry from the Cambridge Structural Database, the world’s repository for small molecule crystal structures. The entry contains experimental data from a crystal diffraction study. The deposited dataset for this entry is freely available from the CCDC and typically includes 3D coordinates, cell parameters, space group, experimental conditions and quality measures.

Topic: Crystallization and Solubility Studies
Subfield: Materials Chemistry
Field: Materials Science
Domain: Physical Sciences
LB01 SIX WEEKS OF SOFOSBUVIR/LEDIPASVIR TREATMENT OF ACUTE HEPATITIS C VIRUS GENOTYPE 1 MONOINFECTION: FINAL RESULTS OF THE THE GERMAN HEPNET ACUTE HCV IV STUDY

Introduction Early treatment of acute hepatitis C virus (HCV) infection with interferon alfa monotherapy is highly effective but is associated with frequent unfavorable side effects. There is no fully published study yet exploring the safety, efficacy and required treatment duration of interferon-free treatment of acute hepatitis C virus monoinfection. Preliminary reports suggested that ledipasvir/sofosbuvir therapy is effective in acute hepatitis C but relapses were reported in HIV-coinfected patients after 6 weeks of treatment. Aims & Methods The German HepNet Acute HCV IV Study was designed as a single-arm, prospective multicenter pilot study to evaluate the efficacy and safety of treatment with sofosbuvir plus ledipasvir (SOF/LDV) for 6 weeks without ribavirin in patients with acute genotype 1 HCV monoinfection. We here report the final 24 weeks’ post-treatment results. Results Twenty patients were included by 10 centers (60% male, mean age 46 ± 12 years; 11 patients HCV genotype 1a, 9 patients genotype 1b). The main risk factors for HCV infection were sexual transmission (n = 11) and medical procedures/needle stick injuries (n = 5). Median alanine aminotransferase (ALT) and median bilirubin levels before start of antiviral treatment were 225 U/l (range 32–2716) and 13.6 µmol/l (range 5.13–111), respectively. ALT levels rapidly declined during therapy and values normalized already by treatment weeks 2 in nine patients and by week 4 in 17 patients. HCV RNA was undetectable by the Roche COBAS® AmpliPrep/COBAS® TaqMan® HCV Test v2.0 by weeks 2, 4 and 6 in eight, 13, and 20 patients, respectively. SVR-12 was 100% and 19 patients have completed FU-week 24 and all remained HCV-RNA negative. One patient was lost to follow-up at week 24 post treatment. Conclusion Treatment for 6 weeks with LDV/SOF was well tolerated and highly effective in HCV genotype 1 monoinfected patients with acute hepatitis C. Virological response was durable after therapy for at least 24 weeks. A rapid improvement in biochemical disease activity was observed during therapy. Short-duration treatment of acute hepatitis C could prevent the spread of HCV in high-risk populations and may be cost-saving as compared with treatment of chronic hepatitis C. Disclosure of Interest Katja Deterding: Lecturer fees and travel grants from Gilead, MSD/Merck and AbbVie

Topic: Hepatitis C virus research
Subfield: Hepatology
Field: Medicine
Domain: Health Sciences
Prescription Drug Coverage and Elderly Medicare Spending

The introduction of Medicare Part D has generated interest in the cost of providing drug coverage to the elderly.Of paramount importance --often unaccounted for in budget estimates --are the salutary effects that increased prescription drug use might have on other Medicare spending.This paper uses longitudinal data from the Medicare Current Beneficiary Survey (MCBS) to estimate how prescription drug benefits affect Medicare spending.We compare spending and service use for Medigap enrollees with and without drug coverage.Because of concerns about selection, we use variation in supply-side regulations of the individual insurance market --including guaranteed issue and community rating --as instruments for prescription drug coverage.We employ a discrete factor model to control for individual-level heterogeneity that might induce bias in the effects of drug coverage.Medigap prescription drug coverage increases drug spending by $170 or 22%, and reduces Medicare Part A spending by $350 or 13% (in 2000 dollars).Medigap prescription drug coverage reduces Medicare Part B spending, but the estimates are not statistically significant.Overall, a $1 increase in prescription drug spending is associated with a $2.06 reduction in Medicare spending.Furthermore, the substitution effect decreases as income rises, and thus provides support for the low-income assistance program of Medicare Part D.

Topic: Medication Adherence and Compliance
Subfield: Family Practice
Field: Medicine
Domain: Health Sciences
A Combination of Blood Lymphocytes and AST Levels Distinguish Patients with Small Hepatocellular Carcinomas from Non Cancer Patients.

<title>Abstract</title> <bold>Purpose</bold>HCC patients typically present at an advanced tumor stage, in which surgical therapies cannot be used. Screening ultrasound exams can increase the numbers of patients diagnosed with small tumors, but are often not used in patients at risk for HCC. We evaluated clinically-available and cheap potential blood tests as biomarkers for screening patients at risk for HCC.<bold>Methods</bold>A comparison was made of commonly used blood count and liver function parameters in a group of patients (n=101) with small HCCs (<underline>&lt;</underline>3cm) or without HCC (n=275), who presented for liver transplantation in our institute. <bold>Results</bold>Significant differences were found for blood lymphocytes and AST levels. This 2-parameter combination was found to be significantly different between patients with small HCCs versus no HCC. Using the combination of lymphocytes and AST levels to dichotomize the HCC patients, only blood levels of alpha-fetoprotein amongst the tumor characteristics, were found to be significantly different amongst the 2 HCC groups, as well as levels of blood total bilirubin, ALKP and PLR ratio. The results were confirmed using a separate smaller cohort of non-transplanted small size HCC patients.<bold>Conclusion</bold>The combination of elevated blood levels of lymphocyte counts and AST levels holds promise for screening of patients with chronic liver disease who are at risk for HCC.

Topic: Liver Disease Diagnosis and Treatment
Subfield: Epidemiology
Field: Medicine
Domain: Health Sciences
Aggressive Palliation in Extensive Stage Small Cell Lung Cancer, Practice Guidelines versus Clinical Practice: A Case Report and Review of the Literature

Small cell lung cancer (SCLC) constitutes approximately 16% of all primary lung cancers, with more than 35,000 new cases per year. Two-thirds of patients present with extensive stage disease (ES-SCLC) due to a tendency to metastasize early. Outcomes remain poor, with a median survival of approximately 10 months and a two-year overall survival of &lt;10%. Current recommendations call for combination chemotherapy alone in patients without localized symptoms. Thoracic radiation therapy following a good clinical response is controversial. We report on a patient with ES-SCLC that had an excellent response to chemotherapy and underwent whole brain radiotherapy for a known brain metastasis and consolidative radiotherapy to the thorax. His latest follow-up demonstrates only a stable residual pulmonary nodule and no evidence of active metastatic disease. ES-SCLC is a relatively common presentation with a variable burden of metastatic disease. In the absence of randomized trials demonstrating the efficacy of thoracic radiation therapy, the community radiation oncologist is placed in a difficult position when addressing these patients, particularly those with otherwise good performance status and a good response to initial systemic chemotherapy. More research in this area is sorely needed to help guide treatment recommendations.

Topic: Lung Cancer Research Studies
Subfield: Oncology
Field: Medicine
Domain: Health Sciences
STATIONARY DISTRIBUTION OF THE VOLUME AT THE BEST QUOTE IN A POISSON ORDER BOOK MODEL

We develop a Markovian model that deals with the volume offered at the best quote of an electronic order book. The volume of the first limit is a stochastic process whose paths are periodically interrupted and reset to a new value, either by a new limit order submitted inside the spread or by a market order that removes the first limit. Using applied probability results on killing and resurrecting Markov processes, we derive the stationary distribution of the volume offered at the best quote. All proposed models are empirically fitted and compared, stressing the importance of the proposed mechanisms.

Topic: Mathematical Dynamics and Fractals
Subfield: Mathematical Physics
Field: Mathematics
Domain: Physical Sciences
Assessing the quality of pharmacological treatments from administrative databases: the case of low‐molecular‐weight heparin after major orthopaedic surgery

Abstract Rationale, aims and objectives ‘Real world data’ are needed to assess the quality of pharmacological treatments in clinical practice. The aim of this study was to determine whether administrative databases can be used to assess the quality of prophylaxis with low‐molecular‐weight heparin after major orthopaedic surgery. Methods The study was performed in a Belgian university hospital. Patients undergoing total hip replacement (THR), total knee replacement (TKR) or hip fracture surgery (HFS) were selected retrospectively from the hospital's 2002 and 2003 administrative databases. Readmissions during the same year as the procedure were also analysed. Three quality indicators were assessed: incidence of venous thromboembolism (VTE), major bleeding and death; adherence to guidelines; and the costs of care. Results Although 70% of data were collected from administrative databases, patients' records also had to be examined. During the period studied, VTE and major bleeding events were rare. Patients undergoing HFS were at greater risk of having a pulmonary embolism [Exact odds ratio (OR) = 3.78; 95% confidence interval (CI) = 1.13–16.22; P = 0.03] or of death from any cause (Exact OR = 2.15; 95% CI = 1.52–infinity; P &lt; 0.01) than patients undergoing THR or TKR. The hospital's prophylaxis protocol was not always followed. Half the patients received higher prophylaxis doses than recommended and 11% received lower doses but no impact on adverse events was demonstrated. Conclusion Results show that administrative databases contain enough information to measure the frequency of adverse events but complementary data on patient weight and on non‐reimbursed drugs must be extracted from the patients' records to evaluate adherence to guidelines. Our findings stress the need for better integration of information systems.

Topic: Venous Thromboembolism Diagnosis and Management
Subfield: Internal Medicine
Field: Medicine
Domain: Health Sciences
Effects of Controlled Freezing Point Technology Combined with Modified Atmosphere Package on Preservation for Grape

The storage effects for Kyoho grape were studied in the three different preservation films package during controlled freezing point.The results showed that during the whole storage of 30 days change of gas concentration in the film of berry was the largest and change of gas concentration in the film of peach was larger than that in the film of polyethylene(PE).The contents of the total soluble solids,titratable acidity and reductive sugar were maintained effectively.Besides,the content of malondialdehyde(MDA) of the berry was accumulated slowly and the decending trend of the chlorophyll content of stem was effectively alleviated.At the end of the storage,the grape in the film of berry was best and the grape in the film of peach was better than that in the film of polyethylene(PE).

Topic: Food Quality and Safety Studies
Subfield: Food Science
Field: Agricultural and Biological Sciences
Domain: Life Sciences
What roles does accounting play in the fight against corruption? Insights from a systematic literature review

The fight against corruption has become a priority for both international organizations and national governments worldwide. To fight corruption, several strategies have been suggested which enlist accounting because of its natural orientation towards information disclosure, integrity systems, and efficiency. This article presents a comprehensive literature review regarding accounting vis-à-vis corruption in the public sector. The review combines a bibliometric and a qualitative analysis. The findings reveal a highly fragmented field of research, with no consolidated networks of scholars. The conceptual structure of the field consists of five main themes, corresponding to different strategies through which accounting can contribute towards fighting corruption. Accounting assemblages, however, may not fulfil expectations and were found to play a wide spectrum of roles, ranging from an effective prevention of corruption to active complicity with it. These roles are affected by individual, organizational, and external variables. The article concludes by outlining several avenues for future research.

Topic: Auditing, Earnings Management, Governance
Subfield: Accounting
Field: Business, Management and Accounting
Domain: Social Sciences
Analysis on the Results of Laboratory Identification of "Qiufeng×Baiyu" over the Years

The average performance of Qiufeng × Baiyu, taken as the control during the laboratory-joint identification of newly bred silkworm varieties in Zhejiang Province since 2001, was presented in this paper. And the results of major characters between spring and autumn rearing were compared; the changes of main characters over the years were analyzed so as to provide valuable information for improving and popularizing of Qiufeng × Baiyu.

Topic: Silkworms and Sericulture Research
Subfield: Insect Science
Field: Agricultural and Biological Sciences
Domain: Life Sciences
The art of groupwork practice with manualized curricula

There is a growing trend in social work toward the use of group-based manuals. Occasionally considered to be opposing approaches, practice based on manualized curricula and practice based on group processes are – in our view – complementary to each other. In this paper, we examine the advantages and disadvantages of manuals as a basis for practice. We offer a series of design and practice principles intended to assist designers and users of manuals. We illustrate the application of these principles with a manualized program, Making Choices, whose aim is to decrease aggression and improve peer relationships in elementary school children.

Topic: Social Work Education and Practice
Subfield: Public Administration
Field: Social Sciences
Domain: Social Sciences
Medication Errors in Critical Care Units in a Tertiary Hospital

Medication errors in critical care are frequent, serious, and predictable. Critically ill patients are prescribed, twice as many medications, as patients outside of the intensive care unit (ICU) and nearly all of them suffer, a potentially life-threatening error at some point during their hospitalization. Today, in the health care profession, all types of medication errors including missed dose, wrong dosage forms, wrong time interval, wrong route, etc., are a big deal, for quality patient care. Problems related to medications, are common in the healthcare profession, and are responsible for significant morbidity, mortality, and cost. Several recent studies have demonstrated that patients frequently have difficulty in reading and understanding medication labels. According to the Institute of Medicine report, “Preventing Medication Errors”, cited poor labeling as a top most cause of medication errors in the USA. Evidence suggests that specific content and format of prescription drug labels, facilitate a better communication to minimize apprehension by patients regarding medication errors.

Topic: Patient Safety and Medication Errors
Subfield: Emergency Medical Services
Field: Health Professions
Domain: Health Sciences
Notes on a New Protozoon,<i>Piridium sociabile</i>n.gen., n.sp., from the Foot of<i>Buccinum undatum</i>

The earliest stages found are groups of small bodies, 1·8 or 2·0μ in diameter. These groups are probably taken up by phagocytic cells and pass through the epithelium of the host into the connective tissue, where a cyst is formed by the host. Within the cyst the bodies grow. In many cysts there is a differentiation into two types: the larger, when mature, attain 40μ in size, are shaped like a pear, and contain at the broad end a chromatic rod. The small bodies reach 3Oμ in length, are also somewhat piriform, and contain two similar structures attached to their nuclei. In some cysts large bodies alone are found. Development is periodic, the youngest stages being found from January to March and the parasites not reaching maturity until September–November. Type species P. sociabile , with the features mentioned above: found near the ventral surface of the foot of Buccinum undatum .

Topic: Parasitic Infections and Diagnostics
Subfield: Parasitology
Field: Immunology and Microbiology
Domain: Life Sciences