Miracle-dz/UniFAR-FTUs huggingface dataset:
Stem cell transplantation for the treatment of Fanconi anaemia using a fludarabine‐based cytoreductive regimen and T‐cell‐depleted related HLA‐mismatched peripheral blood stem cell grafts

We have employed a new cytoreductive regimen to transplant two patients with Fanconi anaemia (FA), using T cell‐depleted two HLA‐allele disparate related peripheral blood stem cell transplants (PBSCTs). Patient 1, a 5‐year‐old male with FA and aplastic anaemia, initially received an HLA two‐antigen mismatched unrelated cord blood transplant and failed to engraft. He received fludarabine (Flu) and cyclophosphamide (Cy), followed by a CD34+ E‐rosette− (CD34+E−), T cell‐depleted, granulocyte colony‐stimulating factor (G‐CSF)‐mobilized PBSCT from his HLA B‐DRB1 mismatched father. He received anti‐thymocyte globulin (ATG), steroids, FK506 and G‐CSF after transplant for rejection and graft‐versus‐host disease (GVHD) prophylaxis. The patient is now 23 months after SCT with no evidence of GVHD and with full haematopoietic and immune reconstitution. Patient 2, a 10‐year‐old boy with FA and myelodysplastic syndrome, received single‐dose total body irradiation (SDTBI), Flu and Cy followed by a CD34+E−, T‐cell‐depleted, G‐CSF‐mobilized PBSCT from his HLA B‐DRB1 mismatched sister. He also received ATG, steroids, FK506 and G‐CSF after transplant. The patient is now 12 months after SCT in complete remission with no evidence of GVHD. Absolute neutrophil counts (ANC) of > 1 × 109/l were achieved on day 11 and day 10 post transplant respectively. Both patients are fully engrafted. In summary, we report two successful T‐cell‐depleted stem cell transplants from mismatched related donors for the treatment of Fanconi anaemia, using a fludarabine‐based cytoreduction. Both patients experienced minimal toxicity, rapid engraftment and no GVHD.
Troubled Craft and Novice Teachers: An Ethnographic Account of Emerging Professional Identities of Novice Teachers in the English Lifelong Learning Sector

In adopting a qualitative, ethnographically informed approach this thesis explores the identity formation of novice teachers in the lifelong learning sector in England. The research is concerned with four areas for inquiry: how novice teachers perceive the relationship between their professional practices, experiences and emerging teacher identities; the usefulness of the concept of 'craft' in sociological writings to theorise the identities of novice teachers; the appropriateness of a digital ethnographic methodological approach enabling effective research into teachers lives in this sector; and, the applicability of online asynchronous blogging practices to support the development of the professional practices by novice teachers in the lifelong learning sector in England. The identity and pedagogic practices of these novice teachers are contextualised by the ‘turbulent times’ for both the workplace of this sector and the teacher education that supports entrants into this sector. The fieldwork for the research follows two cohorts of new entrants into first-time employment across an 18-month period. In developing an understanding of craft identities, blogging practices are developed as a methodological tool within a digital ethnographic approach, exploring the potential for this revised ethnography. The use of reflective practices through online tools to generate data is conceived herein as an ‘epistemology of doing’: a research practice that in turn supports in an ethical way the lives and social practices of those who participate. The findings of the thesis suggest (contrary to use of the term craft by neo-liberalism) that novice teachers’ craft practice and craft identity are a potentially stable basis for sustained practice in the otherwise turbulent lifelong learning sector. However, this ‘stable basis’ also provides contradictions, uneasy relations, compromises and insurmountable challenges when buttressed against the performativity cultures of the sector.
A low-voltage 3 mW 10-bit 4MS/s pipeline ADC in digital CMOS for sensor interfacing

A 1.5V 10-b 4MS/s pipeline ADC for sensor interfacing is described. Amplifiers are efficiently shared between stages and low-voltage techniques are used to reduce the power supply down to 1.4V. The selected resolution-per-stage greatly simplifies the implementation of a low-power design. Simulated results using a standard digital 0.18 /spl mu/m CMOS technology exhibit 9.5 effective bits at Nyquist-rate. The chip occupies 0.6 mm/sup 2/ and dissipates only 3 mW at maximum conversion-rate.
Electromagnetic characterization of automotive sunroofs for car-to-X applications

For Car-to-X communication at 5.9 GHz effects of electromagnetic wave propagation inside and along the sunroof of cars are investigated by means of modal analysis of a dielectric slab waveguide, numerical modeling by finite integration technique and measurements. Since the field is guided inside the glass as fundamental TMo mode and overlaid by free space radiation, interference effects reduce the range in forward direction, an important disturbing effect for car-to-car communication. The influences of roof curvature as well as window size and position on wave propagation and radiation are investigated. Deep nulls in the radiation pattern are found in direction of the forward corners of the glass roof. Metallized sunroofs are shown to improve forward radiation and nulls by at least 6 dB. Computational results are evaluated by measurements on a whole car in an open site test range.
Inferring chromatin-bound protein complexes from genome-wide binding assays

Genome-wide binding assays can determine where individual transcription factors bind in the genome. However, these factors rarely bind chromatin alone, but instead frequently bind to cis-regulatory elements (CREs) together with other factors thus forming protein complexes. Currently there are no integrative analytical approaches that can predict which complexes are formed on chromatin. Here, we describe a computational methodology to systematically capture protein complexes and infer their impact on gene expression. We applied our method to three human cell types, identified thousands of CREs, inferred known and undescribed complexes recruited to these CREs, and determined the role of the complexes as activators or repressors. Importantly, we found that the predicted complexes have a higher number of physical interactions between their members than expected by chance. Our work provides a mechanism for developing hypotheses about gene regulation via binding partners, and deciphering the interplay between combinatorial binding and gene expression.
Fibrinolysis status in the Budd–Chiari syndrome in China

Pathogenesis and clinical characteristics of the Budd–Chiari syndrome (BCS) in Asia are somewhat different from the ones observed in Western countries. Obstruction of the inferior vena cava (IVC) or of the hepatic veins is caused to a greater extent by membranous webs than by thrombosis. Impaired fibrinolysis has been found in European patients with BCS, but its status in Chinese patients with this condition is still unknown. To explore the characteristics of fibrinolysis in BCS patients in this country, we measured the euglobulin lysis time (ELT) for overall fibrinolysis and the plasma levels of five fibrinolytic components in 65 Chinese patients with BCS and 43 healthy controls. In patients, ELTs were slightly shorter than in controls (mean, 293 vs. 357 min, P < 0.02), tissue type plasminogen activator levels were higher than in controls (mean, 239 vs. 185 pg/ml, P < 0.01), and plasminogen activator inhibitor 1 levels were lower than in controls (mean, 1.43 vs. 1.73 ng/ml, P < 0.001). To explore BCS in more detail, we subgrouped the cases according to age, type of venous occlusion, Child-Pugh score, and thrombosis. As a result of this analysis, we found that young patients (age <30 years) had a longer ELT (mean, 440 min) than the older patient groups (30 ⩽ age ⩽44, 45 ⩽age ⩽54, age>54 years; mean ELT = 242, 198, and 289 min, respectively, all P < 0.05). The independent hepatic vein occlusion subgroup showed a longer ELT (mean, 367 min) than the combined hepatic vein and IVC or the independent IVC occlusion subgroup (mean ELT = 233 and 260 min, both P < 0.05). ELT did not show significant differences between Child-Pugh class A and B subgroups (mean, 267 vs. 333 min, P > 0.05). ELT in the subgroup without thrombosis was shorter than in controls (mean, 288 vs. 358 min, P < 0.05), and in the subgroup with thrombosis, it was also slightly shorter than in controls, without reaching statistical significance (mean, 306 vs. 358 min, P > 0.05). By and large, overall fibrinolytic potential was slightly increased in Chinese patients with BCS in this study, but fibrinolysis differed according to its baseline characteristics. Compared with the one seen in BCS patients from Western countries, BCS in China exhibits certain special changes in fibrinolysis and we were able to explain some of these changes.
Do movie contents influence box-office revenues?

ABSTRACT This article studies the determinants of box-office revenues in the motion picture industry. We first adopt an approach that takes into account quality signals (e.g. talent concentration, movie budget and Oscar awards, among others) to analyse the empirical relationship between category-specific parenthood ratings (R-ratings) and box-office revenues. Then, by matching movie contents with economic performance records, our original approach reveals that offensive contents like profanity or nudity may be a hindrance to achieve economic returns, while violent contents seems to enhance box-office revenues. Further research is needed to clarify the interaction in this regard between production budget and movie contents.
Characteristics of Elementary School Principals and Their Support for the Physical Education Program

The purpose was to describe the characteristics of elementary school principals who support elementary physical education programs. A statewide random sample of 500 elementary school principals and their respective elementary physical education teachers (n = 500) were mailed questionnaires. A total of 321 principals responded (64%) to a 20-item questionnaire which assessed their physical fitness and their perceptions of elementary physical education. A total of 340 physical education teachers (68%) responded to a 23-item survey assessing their perceptions of their elementary principals' support for the physical education program. The majority of teachers perceived their principals to be supportive of them and the physical education program. A series of l tests showed no significant differences in principals' age, gender, years of experience as a principal, or how important they thought health and physical education was compared to other subjects taught in the elementary school and how supportive of physical education the principals were perceived to be. Finally, a stepwise backward multiple regression analysis indicated that seven variables, including fitness of the principal and body mass index, did not explain more than 13% of the variance in teachers' perceived support by principals.
A compact two-dimensional phased array using grounded coplanar-waveguides butler matrices

A 2.45 GHz two-dimensional (2-D) 4×4 multi-beam phased array was designed, based on the Butler matrix beamforming technique. Sixteen beam states are produced by two stacks of 4-way Butler matrices, where one stack controls the vertical angle and the other one controls the horizontal angle. To achieve system compactness, the Butler matrix in grounded coplanar-waveguide (CPWG) configuration is newly designed, leading to 54% size reduction comparing with the microstrip line-based Butler matrix. The demonstration of beamforming capability of the integrated array is provided.
In vitro adhesion of Streptococcus sanguinis to dentine root surface after treatment with Er:YAG laser, ultrasonic system, or manual curette.

OBJECTIVE The purpose of this in vitro study was to evaluate the dentine root surface roughness and the adherence of Streptococcus sanguinis (ATCC 10556) after treatment with an ultrasonic system, Er:YAG laser, or manual curette. BACKGROUND DATA Bacterial adhesion and formation of dental biofilm after scaling and root planing may be a challenge to the long-term stability of periodontal therapy. MATERIALS AND METHODS Forty flattened bovine roots were randomly assigned to one of the following groups: ultrasonic system (n = 10); Er:YAG laser (n = 10); manual curette (n = 10); or control untreated roots (n = 10). The mean surface roughness (Ra, microm) of the specimens before and after exposure to each treatment was determined using a surface profilometer. In addition, S. sanguinis was grown on the treated and untreated specimens and the amounts of retained bacteria on the surfaces were measured by culture method. RESULTS All treatments increased the Ra; however, the roughest surface was produced by the curettes. In addition, the specimens treated with curettes showed the highest S. sanguinis adhesion. There was a significant positive correlation between roughness values and bacterial cells counts. CONCLUSION S. sanguinis adhesion was the highest on the curette-treated dentine root surfaces, which also presented the greatest surface roughness.
Survival of urinary leucocytes

The survival of white cells under 15 μ in diameter (95% polymorphonuclear leucocytes), obtained from the urine of patients with urinary tract infections, was examined in vitro under different conditions of osmolarity, pH, and temperature of incubation. The rate at which urinary white cells disintegrate was found to be accelerated by raising the pH, decreasing the osmolarity, and by increasing the temperature at which suspensions were kept. The most rapid disappearance of white cells occurred in hypotonic alkaline suspension at 37°C. There was little change in cell suspensions kept in acid (pH 3·0) or in hypertonic (1,200 mOsmoles/1.) conditions over a period of 24 hours. The findings indicate that in interpreting the significance of the number of leucocytes in urine it is necessary to take into account the condition of the urine and the time and temperature at which it has been kept before examination.
Analysis of hepatitis B virus genotypes and pre‐core region variability during interferon treatment of HBe antigen negative chronic hepatitis B

The clinical importance of hepatitis B virus (HBV) genome variability has been reported recently. One example is the occurrence of hepatitis B virus pre‐core mutants, which arise during spontaneous or interferon‐induced seroconversion from HBeAg to anti‐HBe and are thought to be selected by immune pressure. A survey of HBV pre‐core mutants and viral genotypes in 35 HBeAg negative patients during interferon therapy was carried out to understand viral pathogenesis in this form of chronic hepatitis B. Seventeen patients responded to interferon therapy as assessed by the sustained normalization of serum ALT levels and the significant decrease of viremia levels. The response rate to interferon was independent of both initial serum viral DNA level and interferon doses. During interferon therapy, a significant decrease of M0 (wild‐type pre‐core sequence at pos. 1887–1908), M1 (TGG to TAG at pos. 1896) or M2 (TGG to TAG at pos. 1896, and GGC to GAC at pos. 1899) positive viral genomes was found in 48%, 42%, and 33% of patients, respectively. A higher response rate to interferon therapy was observed in patients infected with HBV genotype A (70%) or M0 positive strains (75%) as compared to patients infected with genotype D/E (40%) or M1/M2 positive strains (44%). The data support the hypothesis that pre‐core defective HBV represent viral mutants with an increased capacity to resist exogenous alpha interferon. These findings emphasize that characterization of HBV genome variability prior to interferon therapy may help to predict antiviral response in HBeAg negative patients. © 1996 Wiley‐Liss, Inc.
Prevalence of Genes Encoding Pyrogenic Toxin Superantigens and Exfoliative Toxins among Strains of Staphylococcus aureus Isolated from Blood and Nasal Specimens

ABSTRACT A total of 429 different Staphylococcus aureus isolates encompassing 219 blood isolates and 210 isolates taken from anterior nares were systematically searched by two multiplex PCR-DNA enzyme immunoassays (PCR-DEIA) for exfoliative toxin (ET) genes eta and etb, as well as for the classical members of the pyrogenic toxin superantigen (PTSAg) gene family comprising the staphylococcal enterotoxin (SE) genes sea-see and the toxic shock syndrome toxin 1 gene tst. In addition, a third PCR-DEIA was established to investigate the possession of four recently described SE genes, viz. seg-sej. The most frequent PTSAg/ET genes amplified were seg and sei, which were found strictly in combination in 55.0% of the S. aureus isolates tested. Other frequently detected toxin genes were tst (20.3%), sea (15.9%), and sec (11.2%). Only five isolates harbored ET genes. Regarding the origin of the S. aureus isolates, a significant difference (P = 0.037) was found for the possession of the sed/sej gene combination (10.5% of blood isolates versus 3.3% of nasal strains). Overall, about half of S. aureus isolates tested harbored genes of the classical members of the PTSAg family and ETs (50.8%), whereas 73.0% of S. aureus isolates were toxin gene positive if the recently described SE genes were included. This notable higher prevalence indicates that the possession of PTSAg genes in particular seems to be a habitual feature of S. aureus. Moreover, mainly due to the fixed combinations of seg plus sei, as well as sed plus sej, the possession of multiple PTSAg genes (62.9%) is more frequent than assumed so far.
Linear imperative programming with Differential Evolution

Differential Evolution (DE) is an evolutionary approach for optimizing non-linear continuous space functions. This method is known to be robust and easy to use. DE manipulates vectors of floats that are improved over generations by mating with best and random individuals. Recently, DE was successfully applied to the automatic generation of programs by mapping real-valued vectors to full programs trees — Tree Based Differential Evolution (TreeDE). In this paper, we propose to use DE as a method to directly generate linear sequences of imperative instructions, which we call Linear Differential Evolutionary Programming (LDEP). Unlike TreeDE, LDEP incorporates constant management for regression problems and lessens the constraints on the architecture of solutions since the user is no more required to determine the tree depth of solutions. Comparisons with standard Genetic Programming and with the CMA-ES algorithm showed that DE-based approach are well suited to automatic programming, being notably more robust than CMA-ES in this particular context.
Multiple EBNA1-binding sites are required to form an EBNA1-dependent enhancer and to activate a minimal replicative origin within oriP of Epstein-Barr virus

EBNA1 activates the EBV plasmid maintenance sequence oriP by binding to its two essential regions. One region is a family of 30-base-pair (bp) repeats and is activated by EBNA1 to act as a transcriptional enhancer. The other region contains a 65-bp dyad symmetry and lacks enhancer function. To explore the functional differences between the two regions, we determined oriP activities as functions of the number of 30-bp repeats and compared them with activities determined when tandem copies of the dyad symmetry region were used to replace the 30-bp repeats. Three conclusions have been drawn. (i) Activation of the 30-bp repeats by EBNA1 to enhance transcription or to permit plasmid maintenance is a highly cooperative process involving at least six or seven 30-bp repeats for full activity. (ii) Tandem copies of the dyad symmetry region cooperatively enhance transcription but are less effective than 30-bp repeats providing a similar number of EBNA1-binding sites. (iii) Tandem copies of the dyad symmetry region alone cooperatively activate replication, suggesting that the region contains the actual origin of replication. We also report that while rodent-derived cell lines do not support replication of EBV-derived plasmids they do permit EBNA1-dependent enhancer activity. EBV plasmid replication thus requires the interaction of EBNA1 or oriP with a host factor that is not required for enhancement of transcription.
Comprehensive Genomic Profiling of Clinically Advanced Medullary Thyroid Carcinoma

Objective: The aim of this study was to determine the genomic alterations of cancer-related genes in advanced medullary thyroid carcinoma during the course of clinical care. Methods: Hybrid-capture-based comprehensive genomic profiling was performed on 34 consecutive medullary thyroid carcinoma cases to identify all four classes of genomic alterations, and outcome for an index patient was collected. Results:RET was mutated in 88% (30/34) of cases, with RET M918T being responsible for 70% (21/30) of the RET alterations. The other RET alterations were RET E632_L633del, C634R, C620R, C618G/R/S, V804M, and RET amplification. Two of the four RET wild-type patients harbored mutations in KRAS or HRAS (1/34 each). The next most frequent genomic alterations were amplifications of CCND1, FGF3, and FGF19 and alterations in CDKN2A (3/34 each). One case with a RET M918T mutation developed acquired resistance to progressively dose-escalated vandetanib. When the mTOR inhibitor everolimus was added to continued vandetanib treatment, the patient achieved a second 25% reduction of tumor volume (RECIST 1.1) for 8 months. Conclusions: Comprehensive genomic profiling identified the full breadth of RET alterations in metastatic medullary thyroid carcinoma and possible cooperating oncogenic driver alterations. This approach may refine the use of targeted therapy for these patients.
Millennium development goals and eye health

In September 2000, world leaders made a commitment to build a more equitable, prosperous and safer world by 2015 and launched the Millennium Development Goals (MDGs). In the previous year, the World Health Organization and the International Agency for the Prevention of Blindness in partnership launched the global initiative to eliminate avoidable blindness by the year 2020–VISION 2020 the Right to Sight. It has focused on the prevention of a disability-blindness and recognized a health issue–sight as a human right. Both global initiatives have made considerable progress with synergy especially on MDG 1–the reduction of poverty and the reduction in numbers of the blind. A review of the MDGs has identified the need to address disparities within and between countries, quality, and disability. Noncommunicable diseases are emerging as a challenge to the MDGs and Vision 2020:0 the Right to Sight. For the future, up to and beyond 2015, there will be need for both initiatives to continue to work in synergy to address present and emerging challenges.