Miracle-dz/UniFAR-FTUs huggingface dataset:
Substantial improvement in a Meige's syndrome patient with levetiracetam treatment

We report on a woman with idiopathic Meige's syndrome whose dystonia improved with the use of levetiracetam (LEV, Keppra, UCB Pharma, Smyrna, GA). This report and data from an animal model of paroxysmal dystonia suggest that LEV might be helpful in the treatment of dystonia. © 2004 Movement Disorder Society
Kidney cancer in Sweden: A decrease in incidence and tumour stage, 1979 – 2001

Abstract Objective. In the Western world the incidence of renal cell carcinoma (RCC) has been increasing for several decades. In Sweden the incidence has decreased since 1980. This may reflect better health of the population. Another possible explanation could be a decrease in incidentally diagnosed RCC. Since these tumours are smaller, relatively more advanced tumours would then enter the cancer registry. The aim of this study was to compare methods of detection of RCC, tumour characteristics and survival from three periods over a timespan of more than 20 years. Material and methods. Adult patients (n = 515) with RCC were identified in a well-defined population-based area with the same incidence of RCC as the rest of Sweden. Patient data from three periods, 1979 – 1981 (A), 1989 – 1991 (B) and 1999 – 2001 (C), were collected for gender, age, tumour side, method of detection, tumour size, tumour type, metastasis, T stage and Fuhrman grade at the time of diagnosis. Using the Swedish Cause-of-Death Register, cause-specific survival was calculated. When available, tissue was reanalysed according to modern standards by an experienced pathologist. Results.The frequency of ultrasound and computed tomography increased and autopsy and intravenous pyelography decreased with time as the first detection method. There was a significant change towards smaller tumours and less severe stages and grades in more recent periods. Metastatic disease was most common in the first period. The distribution between the different histological tumour types did not change over time. Five-year cause-specific survival increased significantly from 41% to 63%. Subgroup analysis found significantly increased survival for patients with no metastases or with low-grade tumours. Conclusion. The data support a true decrease in the incidence of RCC over time in Sweden with a migration towards lower tumour stages but no change in distribution between the different histological subtypes over time.
Structure and functional properties of resistant starch from butyrylated arenga starches

Butyrylated arenga starches (BAS) with different degree of substitution (DS 0.074, DS 0.151, DS 0.226) were synthesized by the reaction of arenga starch with butyric anhydride under varying pH and butyric anhydride concentration. The products were characterized for resistant starch (RS), chemical structure of the RS by nuclear magnetic resonance (NMR) spectroscopy and functional properties including water and oil holding capacity (WHC and OHC), bile acid/salts and cholesterol binding. The results indicated that RS increased with increase in the DS of BAS. Analysis of 1H NMR spectra of RS from BAS revealed the presence of methylene (CH2) and methyl (CH3) protons, while the 13C NMR spectra gave the signals of methylene, methyl and carbonyl carbon of butyryl groups. Compared to the 13C NMR spectrum of the native starch, the modified starches gave additional signals broadening of the C1 and C6 signals. Those results further suggested that the butyryl groups were substituted at the C2 and C6 positions. The WHC and OHC of RS from BAS increased with increase in the DS, indicating that butyrylation increased both hydrophilicity and hydrophobicity of the starches. The bile acid/salts of RS from BAS increased with the increase of DS and the total cholesterol decreased with the increasing DS. Key words: Resistant starch, butyrylated arenga starch, degree of substitution.
Evaluation of MRI T1-based treatment monitoring during laser-induced thermotherapy of liver metastases for necrotic size prediction

Abstract Purpose: The aim of this study was to evaluate the accuracy of real-time magnetic resonance imaging (MRI) T1-based treatment monitoring for predicting volume of lesions induced by laser-induced thermotherapy (LITT) of liver metastases. Materials and methods: This prospective study was approved by the institutional review board and informed consent from all included patients was obtained. In 151 patients, 237 liver metastases were ablated during 372 LITT procedures. 1.5T MRI treatment monitoring was performed based on longitudinal relaxation time (T1) using fast low-angle shot (FLASH) sequences. Patients underwent additional contrast-enhanced MRI directly after LITT, 24 h after the procedure and during follow-up at 3, 6 and 12 months. The amount of energy necessary to induce a defined necrotic area was investigated within the various liver segments. Results: The total amount of energy applied during LITT varied from 6.12–225.32 kJ (mean 48.96 kJ). Ablation in liver segments 5 (2.12 kJ/cm3) and 8 (2.16 kJ/cm3) required the highest energy. The overall pre-ablative metastasis volume ranged from 0.5–51.94 cm3 (mean 1.99 cm3, SD 25.49 cm3) while the volume measured in the last available T1 image varied from 0.78–120 cm3 (mean 26.25 cm3, SD 25.66 cm3). Volumes measured via MRI T1-based treatment monitoring showed a stronger correlation with necrosis 24 h after LITT (r = 0.933, p < 0.001) than contrast-enhanced MRI directly after the procedure (r = 0.888, p < 0.001). Conclusions: Real-time MRI T1-based treatment monitoring during LITT of liver metastases allows for precise estimation of the resulting lesion volume and improves control of the energy necessary during ablation.
Seroprevalence of HBsAg , Anti-HCV and Anti-HIV in Hemophilia ‘ A ’ and ‘ B ’ Patients

The aim of the study was to find out seroprevalence of HBsAg, anti-HCV and anti-HIV in hemophilia “A” AND “B” patients. To find out the association of hepatitis B, hepatitis C and HIV infection with various factors like age, sex, family history, number of transfusions of blood products and factor concentrates. It was a hospital based cross sectional descriptive study. Hemophilia affected children were selected and evaluated in detail with regards to the age of onset of symptoms, mode of presentation, family history of bleeding/ hemophilia, severity of bleeding episodes and their relation to trauma , number of bleeding episodes with respect to time, most common site of bleeding, most common joint involved, therapies taken for bleeding episodes including blood products and / or factor concentrates, number of times therapy being taken, response to therapy, age of using blood product / factor for the first time, prophylactic or episodic (on demand) therapy. This study revealed that infection rates were higher with higher number of blood products transfusion. Hemophiliacs who received only factor concentrates were less prone for infection with above studied viral infection as compared to the ones who received blood products. Hence, we recommend the use of factor concentrates over blood products in hemophilia patients. Also, routine screening, for transfusion related infections like HCV is advisable for better outcome.
Two phases of increased cell death in the inner retina following early elimination of the ganglion cell population

Neurons in the inner nuclear layer (INL) of the vertebrate retina undergo considerable programmed cell death during development, but the determinants of this cell death remain largely unknown. The present study examines the role of retinal ganglion cells in support of INL neurons in the developing ferret retina. The retinal ganglion cell population was eliminated by optic nerve transection at postnatal day (P) 2, and the incidence of cell death was examined using terminal deoxytransferase dUTP nick‐end labelling (TUNEL) at various ages during the first 3 postnatal weeks. Significant increases in TUNEL‐positive cells were observed in the neuroblast layer (NBL) as early as P3, prior to synapse formation within the inner plexiform layer (IPL), and again in the INL at P22, the normal peak of naturally occurring cell death within the ferret's INL. A decrease in TUNEL‐positive cells was found in the NBL at P8. These results show three phases of response to the loss of retinal ganglion cells and suggest that cells in the NBL/INL are normally dependent on retinal ganglion cells for their survival. Recent studies have shown that certain populations of retinal neurons are reduced in adult animals that had lost the population of ganglion cells during early development, so the present study also examined when this reduction could first be detected. The number of parvalbumin‐immunoreactive amacrine cells was decreased significantly in the NBL of the manipulated eye as early as P8, when we could first label this population, and this difference persisted through adulthood. The fact that cell death in the NBL has already increased within 24 hours of ganglion cell elimination, coupled with the specificity of this effect on the adult complement of INL cell types, shows that cell–cell interactions controlling survival are already highly specific for particular types of retinal neuron early in development J. Comp. Neurol. 439:440–449, 2001. © 2001 Wiley‐Liss, Inc.
11p15 imprinting center region 1 loss of methylation is a common and specific cause of typical Russell-Silver syndrome: clinical scoring system and epigenetic-phenotypic correlations.

CONTEXT Russell-Silver syndrome (RSS), characterized by intrauterine and postnatal growth retardation, dysmorphic features, and frequent body asymmetry, spares cranial growth. Maternal uniparental disomy for chromosome 7 (mUPD7) is found in 5-10% of cases. We identified loss of methylation (LOM) of 11p15 Imprinting Center Region 1 (ICR1) domain (including IGF-II) as a mechanism leading to RSS. OBJECTIVE The aim was to screen for 11p15 epimutation and mUPD7 in RSS and non-RSS small-for-gestational-age (SGA) patients and identify epigenetic-phenotypic correlations. STUDIED POPULATION AND METHODS: A total of 127 SGA patients were analyzed. Clinical diagnosis of RSS was established when the criterion of being SGA was associated with at least three of five criteria: postnatal growth retardation, relative macrocephaly, prominent forehead, body asymmetry, and feeding difficulties. Serum IGF-II was evaluated for 82 patients. RESULTS Of the 127 SGA patients, 58 were diagnosed with RSS; 37 of these (63.8%) displayed partial LOM of the 11p15 ICR1 domain, and three (5.2%) had mUPD7. No molecular abnormalities were found in the non-RSS SGA group (n = 69). Birth weight, birth length, and postnatal body mass index (BMI) were lower in the abnormal 11p15 RSS group (ab-ICR1-RSS) than in the normal 11p15 RSS group [-3.4 vs.-2.6 SD score (SDS), -4.4 vs.-3.4 SDS, and -2.5 vs.-1.6 SDS, respectively; P < 0.05]. Among RSS patients, prominent forehead, relative macrocephaly, body asymmetry, and low BMI were significantly associated with ICR1 LOM. All ab-ICR1-RSS patients had at least four of five criteria of the scoring system. Postnatal IGF-II levels were within normal values. CONCLUSION The 11p15 ICR1 epimutation is a major, specific cause of RSS exhibiting failure to thrive. We propose a clinical scoring system (including a BMI < -2 SDS), highly predictive of 11p15 ICR1 LOM, for the diagnosis of RSS.
What are the risk factors and settings for non‐alcoholic fatty liver disease in Asia–Pacific?

The risk factors and settings for non‐alcoholic fatty liver disease (NAFLD) in Asians are reviewed comprehensively. Based particularly on large community‐based studies using ultrasonography, case–control series and prospective longitudinal studies, the prevalence of NAFLD in Asia is between 12% and 24%, depending on age, gender, locality and ethnicity. Further, the prevalence in China and Japan has nearly doubled in the last 10–15 years. A detailed analysis of these data shows that NAFLD risk factors for Asians resemble those in the West for age at presentation, prevalence of type 2 diabetes mellitus (T2DM) and hyperlipidemia. The apparent differences in prevalence of central obesity and overall obesity are related to criteria used to define waist circumference and body mass index (BMI), respectively. The strongest associations are with components of the metabolic syndrome, particularly the combined presence of central obesity and obesity. Non‐alcoholic fatty liver disease appears to be associated with long‐standing insulin resistance and likely represents the hepatic manifestation of metabolic syndrome. Not surprisingly therefore, Asians with NAFLD are at high risk of developing diabetes and cardiovascular disease. Conversely, metabolic syndrome may precede the diagnosis of NAFLD. The increasing prevalence of obesity, coupled with T2DM, dyslipidemia, hypertension and ultimately metabolic syndrome puts more than half the world's population at risk of developing NAFLD/non‐alcoholic steatohepatitis/cirrhosis in the coming decades. Public health initiatives are clearly imperative to halt or reverse the global ‘diabesity’ pandemic, the underlying basis of NAFLD and metabolic syndrome. In addition, a perspective of NAFLD beyond its hepatic consequences is now warranted; this needs to be considered in relation to management guidelines for affected individuals.
Implementation of the digital control algorithm for the maximum power point tracking DC module

The distributed maximum power point tracking (DMPPT) has become one of the most attractive structures of photovoltaic (PV) generation system recently. In this improved structure, the maximum power point tracking (MPPT) DC module is one of the most important components, which provides not only the better utilization of PV sources, but also many supplementary features on the reliability and safety. As more and more functions are put together into this MPPT DC module, the digital controller design gives the flexibility to reduce the complexity. Therefore, this paper discusses the implementing issues about the digital control algorithm for the MPPT DC module from the computational viewpoint. With the improved modifications, the computation time of the overall algorithm is reduced, and the discussed algorithm can thus be implemented in a tailor-made digital control core as a result.
Transferring Agent Behaviors from Videos via Motion GANs

A major bottleneck for developing general reinforcement learning agents is determining rewards that will yield desirable behaviors under various circumstances. We introduce a general mechanism for automatically specifying meaningful behaviors from raw pixels. In particular, we train a generative adversarial network to produce short sub-goals represented through motion templates. We demonstrate that this approach generates visually meaningful behaviors in unknown environments with novel agents and describe how these motions can be used to train reinforcement learning agents.
The presence of aberrant DNA methylation in noncancerous esophageal mucosae in association with smoking history

Esophageal squamous cell carcinomas (ESCCs) tend to have multiple primary lesions, and it is believed that they arise from background mucosae with accumulation of genetic/epigenetic alterations. In this study, the objective was to elucidate the effects of smoking and drinking on the accumulation of epigenetic alterations in background mucosae.
Understanding capabilities through everyday practice: The case of a weather information system for farmers in West Bengal

In this paper we seek to employ the Capability Approach to evaluate the development impact of a weather information system in West Bengal, India. Responding to the challenges of operationalising the CA, we have adopted a focus on social practice as a basis for identifying capability enhancement among the participants in the system. We find that practice as an epistemological lens can be an appropriate way to fill in the analytical gap between ICTs as "goods and services" and their role in enabling valued "beings and doings".
The development of motor projection patterns in the chick hind limb.

1. Retrograde transport of horseradish peroxidase was used to map the initial projection patterns of lumbosacral motoneurones to the embryonic chick hind limb. 2. The stage 28 segmental projection pattern to each of the four primary muscle masses was characteristic and indistinguishable from the stage 36 projection pattern to the sum of the muscles derived from that mass. In addition, the adductor motoneurone pool was found to be similar in position (both rostro‐caudal and mediolateral) at stages 29, 30, 32, 33 1/2 and 36. 3. Therefore axons from lumbosacral motoneurones project for the most part only to appropriate regions from early times shortly after they grow into the limb bud. Furthermore, the attainment of the segmental projection pattern occurs prior to the normal time of, and therefore without the aid of, cell death. This conclusion was supported by electrophysiological recordings made from muscle nerves. 4. A regionalization of the projection patterns within a single muscle mass could be shown both anatomically and physiologically prior to the cleavage of the mass into individual muscles and the projections were in a general way appropriate for the muscles derived from those regions. 5. Therefore the process of muscle cleavage does not in itself create the specific projection patterns observed, and motoneurone axons appear to grow to and to ramify and make synapses only within regions which correspond to their adult muscles. 6. Finally, the termination site of each motoneurone axon in the early limb was found to be tightly correlated in a somatotopic fashion with the position occupied by its soma in the cord. This suggests that some feature of the motoneurone related to its position may be of importance in achieving the specific projection patterns observed.
Alprostadil infusion in patients with dry age related macular degeneration: a randomized controlled clinical trial

Background: Age-related macular degeneration is the leading cause of blindness among elderly individuals in industrialized countries. New drugs and advanced concepts for the treatment of dry AMD (dAMD) are needed. A new approach is the application of intravenous infusions of prostaglandin E1. Objective: The aim of this study was to assess efficacy and safety of intravenous alprostadil infusion in patients with dAMD. Methods: This was a prospective, randomized, multi-center study. Patients were treated with intravenous infusion of either 60 µg alprostadil or placebo over 3 weeks. Main efficacy outcomes were mean differences in best corrected visual acuity (BCVA) from baseline assessed in early treatment diabetic retinopathy study (ETDRS) lines immediately, 3 months and 6 months after treatment. Results: In the full analysis set (FAS) a mean difference of 0.89 ± 0.537 ETDRS lines according to analysis of variance-covariance (ANCOVA) resulted in the alprostadil group (n = 16) and a mean difference of -0.05 ± 0.578 in the placebo group (n = 17) 3 months after end of treatment. Thus, effectiveness of alprostadil infusion was numerically superior to placebo treatment by a mean of 0.94 lines after 3 months (1.51 lines after 6 months). These findings were more pronounced in the per protocol set (PPS). Safety results were in line with the good safety profile of alprostadil. Conclusion: A numerical treatment effect in favor of alprostadil was visible, which lasted until the end of follow up. These results provide further evidence that alprostadil probably has a therapeutic effect in the treatment of dAMD and justify further clinical studies.
Adaptive control for cable driven parallel robots

CDPR(Cable Driven Parallel Robot) is flexible robot system that can be applied to various environment and energy effective system. Because the rigid actuators of the conventional robots are changed into cables. Cable can generate only tensile force and has nonlinear features, such as nonlinear stiffness, hysteresis, creep, and so on, so it occurs unexpected vibration. In this paper adaptive controller is used to track the desired trajectory and estimated coefficient of cable model. This methods show that tracking error is reduced even though estimated parameter value has 40% error rate. The algorithm is implied to 2-D planar CDPR simulator and confirm performance of it.
T-tilt: a modified tilt model for F0 analysis and synthesis in tonal languages

This paper proposes a modified Tilt model, called T-Tilt , for analyzing and synthesizing F0 contours in tonal languages. The Tilt model successfully designed for intonation modeling is extended to cover syllable-based F0 realization influenced strongly by the tonal context. Two modification approaches include adding a parameter indicating a F0 curve pattern and separating duration and amplitude controls inherent in the Tilt parameter for sake of flexibility. Evaluations are conducted by both an objective RMSE measure and a subjective MOS test on intelligibility and naturalness aspects. Applying to Thai and Mandarin Chinese continuous speech, the proposed model is proved to be very effective for F0 contour analysis. It rather requires extensive work on parameter synthesis although the synthesizing performance is comparable to those produced by other proposed models.
Innovación y desarrollo local: actores estratégicos en el apoyo sistémico a la creación del start up

Estudios han confirmado que los spin-off academicos configurados en mayoria como nuevas empresas “technology based”, resultan ser mas innovadoras respecto a la media de las empresas; de esta forma, estas empresas contribuyen directamente a la creacion de conocimiento, y representan la fuente de nuevos trabajos cualificados. Este Articulo, a partir del analisis de 15 casos de economias locales exitosos en terminos de creacion sistematica de start-up innovadoras, provee la construccion de diferentes hipotesis. Este articulo examina el papel desempenado por un cierto tipo de actor llamado “meta-organizacion”, que desempena un papel en la implementacion de redes de innovacion y la revision de las politicas de desarrollo de los sistemas locales. Con este fin, hemos desarrollado un modelo en tres pasos siguiendo el modelo del ciclo de vida de las redes presentadas por Larson y Starr para destacar las principales caracteristicas y funciones de la “meta-organizacion”
Comprehensive analysis of cooperative gene mutations between class I and class II in de novo acute myeloid leukemia

Acute myeloid leukemia (AML) has been thought to be the consequence of two broad complementation classes of mutations: class I and class II. However, overlap‐mutations between them or within the same class and the position of TP53 mutation are not fully analyzed. We comprehensively analyzed the FLT3, cKIT, N‐RAS, C/EBPA, AML1, MLL, NPM1, and TP53 mutations in 144 newly diagnosed de novo AML. We found 103 of 165 identified mutations were overlapped with other mutations, and most overlap‐mutations consisted of class I and class II mutations. Although overlap‐mutations within the same class were found in seven patients, five of them additionally had the other class mutation. These results suggest that most overlap‐mutations within the same class might be the consequence of acquiring an additional mutation after the completion both of class I and class II mutations. However, mutated genes overlapped with the same class were limited in N‐RAS, TP53, MLL‐PTD, and NPM1, suggesting the possibility that these irregular overlap‐mutations might cooperatively participate in the development of AML. Notably, TP53 mutation was overlapped with both class I and class II mutations, and associated with morphologic multilineage dysplasia and complex karyotype. The genotype consisting of complex karyotype and TP53 mutation was an unfavorable prognostic factor in entire AML patients, indicating this genotype generates a disease entity in de novo AML. These results collectively suggest that TP53 mutation might be a functionally distinguishable class of mutation.