Miracle-dz/UniFAR-FTUs huggingface dataset:
Role of low plasma volume treatment on clinical efficacy of plasmapheresis in neuromyelitis optica

Background: Neuromyelitis optica (NMO) is an autoimmune demyelinating disease preferentially targeting the optic nerves and spinal cord. Plasmapheresis (PP) is an effective adjunct therapy in severe NMO attacks. The recommended minimum plasma volume to be treated per session of PP is equivalent to total plasma volume (TPV) of the patient. Aim: To study the effect of lower plasma volume treated in patients with NMO on clinical efficacy of plasmapheresis in comparison to minimum recommended volume. Methods: This retrospective study was done on acute NMO patients who were managed with PP at our center. Patients who had 5 sessions of PP, spread over 10 days, were included. Clinical outcome was defined as per predefined criteria. Results: 24 patients who underwent PP for acute NMO met our inclusion criteria. Females (age; mean (SD) 33.7 (11.2) years) were more common (n = 18). The minimum recommended plasma volume (PV) that was supposed to be treated per patient during entire acute therapeutic period was 195.5 (14.6) mL per kilogram-body-weight (kg-bw). We treated lower plasma volume (mean (SD) 112.7 (17.0) mL per kg-bw); the difference was significant (P < 0.05). The volume treated was same across the gender (P > 0.05). Significant clinical improvement was observed in 79% of patients (n = 19) after 6 months. There were no significant differences in volume of plasma treated, between patients who had moderate and marked improvement; also, who did, and did not have significant clinical improvement (P > 0.05; for both). Conclusions: Plasmapheresis is a safe and efficient add-on therapy in NMO, especially in steroid-resistant cases. Although the volumes of plasma treated during acute plasmapheresis were less than recommended minimum volumes, majority of patients had significant clinical improvement.
Admission Variables as Predictors of PANCE Scores in Physician Assistant Programs: A Comparison Study Across Universities

Purpose: The purpose of this study was to create a model of cognitive and noncognitive measures that could estimate the probability of achieving a given level of performance on the Physician Assistant National Certifying Examination (PANCE). Methods: A retrospective records review of admissions information used by six universities was conducted to discover which factor had the most impact on the dependent variable of the PANCE score. Multiple predictors were measured: undergraduate grade point average (uGPA), graduate GPA, prerequisite grades, Graduate Record Exam (GRE)‐verbal, GRE‐quantitative, GRE combined, interview scores, years of health care experience, age, gender, and first‐year scores on the Physician Assistant Clinical Knowledge Rating and Assessment Tool (PACKRAT). While PACKRAT scores are not applicable to admission selection, they are a strong midpoint predictor of PANCE performance. Multiple regression analysis was used to develop prediction equations. Expectancy tables were developed to provide estimation of PANCE performance, given the various score ranges on each of the predictor variables. Results: Four predictors made a significant contribution to the final regression equation: GPA, GREverbal, GRE‐quantitative, and PACKRAT scores. The PACKRAT scores were consistently the best predictors of performance on the PANCE. Each of these four predictors can be plugged into predictability tables to estimate the probability of achieving various score intervals on the PANCE. Conclusion: A model of equations and predictors can be used to project how successful a physician assistant (PA) graduate will be on PANCE performance. Years of health care experience, grades on prerequisites, and demographics were not significant predictors across programs but did have significance in certain individual institutions. Future research should examine which specific noncognitive traits measured in interviews can add value to predictability.
Interest Group Influence in the U. S. Congress

This essay critically reviews research published in the last decade that explores how lobbyists and interest groups influence the actions of the U.S. Congress. The research is extensive and contains a rich, but often conflicting, set of findings about the influence of interest groups. The purpose of this essay is to inventory, review, and critique this research in ways that convey as clearly as possible the status of our current understanding of the nature and level of interest group influence in the U.S. Congress.
Magnetic Resonance for T-staging of nasopharyngeal carcinoma--the most informative pair of sequences.

OBJECTIVE To evaluate the most informative pair of sequences in magnetic resonance (MR) for T-staging of nasopharyngeal carcinoma (NPC). METHODS The MR images of 134 patients with newly diagnosed NPC, from 1996 to 2002, were retrospectively reviewed. All the patients were scanned using 1.5 Tesla MR systems. The images of the nasopharynx were reviewed by two qualified radiologists to determine the positive findings and the T-stage by UICC (6th edition) System, using each sequence separately. The T-stage derived from a single MR sequence was then compared with the T-stage based on the five selected sequences to assess the number and percentage of patients who were being understaged. Therefore, the overall percentage accuracy of each single sequence could be determined. A pair of sequences providing information to achieve almost 100% diagnostic accuracy was then derived. RESULTS The overall percentage accuracy of five individual sequences of the nasopharynx is as follows: contrast-enhanced (CE) fat suppression (FS) axial T1 (94.8%), CE FS coronal T1 (88.1%), FS axial T2 (85.8%), non-contrast enhanced (NE) axial T1 (78.4%) and non-contrast enhanced (NE) coronal T1 (77.6%). CE FS axial T1 has the best accuracy. All the structures that are missed in CE FS axial T1, which lead to apparent understaging, are appreciated in NE axial T1-weighted images. CONCLUSION Individual sequences supplement each other in the NPC staging. CE FS axial T1 is the most informative individual sequence. Combination of CE FS axial T1 and NE axial T1 of the nasopharynx provides sufficient information to achieve almost 100% diagnostic accuracy in T-staging; therefore, both should be included in the MR-staging protocol.
Molecular nature of colon tumors in hereditary nonpolyposis colon cancer, familial polyposis, and sporadic colon cancer.

BACKGROUND & AIMS Microsatellite instability (replication error [RER]) is a characteristic of tumors in hereditary nonpolyposis colon cancer (HNPCC), but the mechanism of HNPCC carcinogenesis is not yet understood. To clarify the nature of HNPCC tumors, RER and genetic changes were compared between HNPCC and non-HNPCC tumors. METHODS RER and genetic changes were analyzed in 21 HNPCC, 389 familial adenomatous polyposis, and 206 sporadic tumors using polymerase chain reaction, single-strand conformation polymorphism, sequencing, and Southern hybridization. RESULTS. in HNPCC, 95% tumors at all stages showed RER positivity (altered loci, 4.3 of 5). In familial adenomatous polyposis and sporadic tumors, RER positivity (1.7 of 5) was 3% in adenoma and intramucosal carcinoma, 13%-24% in invasive carcinoma, and 35% in carcinoma metastasized to liver. Fifty percent of RER-positive HNPCC tumors had both germline and somatic mutations of hMSH2 or hMLH1 gene, whereas 6% of RER-positive non-HNPCC had somatic mutation. APC, p53, and K-ras-2 mutations and loss of heterozygosity of tumor-suppressor genes were significantly less frequent (P = 0.03 to 0.0006) but transforming growth factor beta type II receptor mutation was significantly more frequent (P = 0.000001) in HNPCC than in non-HNPCC. CONCLUSIONS RER positivity occurs from an early stage of carcinogenesis in HNPCC but in later stages in non-HNPCC. Most HNPCC tumors may develop through different genetic changes from those in the adenoma-carcinoma sequence, although a certain percentage develops through APC mutation.
Development of the nucleus isthmi in Xenopus, II: Branching patterns of contralaterally projecting isthmotectal axons during maturation of binocular maps

Abstract The tectum of Xenopus frogs receives input from both eyes. The contralateral eye's projection reaches the tectum directly, via the optic nerve, and the ipsilateral eye's projection reaches the tectum indirectly, via the nucleus isthmi. Under normal conditions, the topography of the ipsilateral map relayed from the nucleus isthmi is in register with the topography of the retinotectal map from the contralateral eye. During development, the process of aligning the two maps is complicated by the dramatic changes in binocular overlap of the two eyes' visual fields which take place during late tadpole and juvenile stages. The goal of this study is to determine the branching patterns of contralaterally projecting isthmotectal axons before, during, and after the period of rapid eye migration. Isthmotectal axons were filled by anterograde transport of horseradish peroxidase (HRP) from the nucleus isthmi. The results show that crossed isthmotectal axons enter the entire extent of the tectum before binocular overlap begins to increase. Therefore, binocular overlap is not necessary for the initial isthmotectal projection to span the tectum. The density of isthmotectal branches rises dramatically at the same time that the eyes begin to shift. During the period when eye migration is most rapid, many isthmotectal axons form arbors which resemble adult arbors but which extend over greater proportions of the tectal surface. The axons appear to be directed toward appropriate mediolateral positions as they enter the tectum. Their trajectories are roughly rostocaudal, with relatively little change along the mediolateral dimension. These data, when combined with available physiological data, suggest that mediolateral order is initially established by vision-independent mechanisms but can be altered by vision-dependent mechanisms. Rostrocaudal order becomes discernable only at the time when binocular visual cues become available and appears to be established primarily on the basis of the activity of the retinotectal and isthmotectal axons.
A Strange Disposition? Option Trading, Reference Prices, and Volatility

Using individual stock option data, we document a classic disposition effect in addition to an effect whereby highly unfavorable positions are more likely to be liquidated. Both behaviors are more prevalent for less sophisticated investors. However, for a given gain or loss, option traders are less likely to sell winners and extreme losers as volatility increases. The disposition effect also induces return predictability. When underlying stock prices increase relative to reference prices, next day option delta hedged call returns are significantly higher and put returns are significantly lower.
European guidelines for topical photodynamic therapy part 1: treatment delivery and current indications – actinic keratoses, Bowen’s disease, basal cell carcinoma

Topical photodynamic therapy (PDT) is a widely used non‐invasive treatment for certain non‐melanoma skin cancers, permitting treatment of large and multiple lesions with excellent cosmesis. High efficacy is demonstrated for PDT using standardized protocols in non‐hyperkeratotic actinic keratoses, Bowen’s disease, superficial basal cell carcinomas (BCC) and in certain thin nodular BCC, with superiority of cosmetic outcome over conventional therapies. Recurrence rates following PDT are typically equivalent to existing therapies, although higher than surgery for nodular BCC. PDT is not recommended for invasive squamous cell carcinoma. Treatment is generally well tolerated, but tingling discomfort or pain is common during PDT. New studies identify patients most likely to experience discomfort and permit earlier adoption of pain‐minimization strategies. Reduced discomfort has been observed with novel protocols including shorter photosensitizer application times and in daylight PDT for actinic keratoses.
Hepatic and neuromuscular forms of glycogenosis type III: nine mutations in AGL

Glycogenosis type III (Cori disease) is an autosomal recessive disorder caused by the deficiency of the glycogen debranching enzyme, encoded by the AGL gene, and existing in six isoforms alternately spliced in a tissue‐specific way. Generally, disease onset occurs early on starting from the first year of life, with hepatomegaly, hypoglycemia, hyperlipidemia, increased CK levels, and, in some cases, short stature and slight mental retardation. Frequently, hepatomegaly tends to resolve spontaneously and inexplicably during childhood, when myopathy, often associated with cardiomyopathy, arises. This disease is known to lack almost invariably clear links between the genotype and clinical phenotype. We describe nine new mutations in Italian patients: four nonsense (p.Arg285X, p.Lys422X, p.Arg910X, p.Arg977X), three frameshift (c.442delA, c.753_756delGACA, c.3963delG), and two missense (p.Ala1120Pro, p.Arg524His). Particularly, the nonsense p.Arg285X is linked to an exonic splicing enhancer and it was found to produce two species of transcripts at the same time. Moreover, we discuss a subgroup of subjects carrying c.2681+1G>A, which has proven to be the most frequent mutation among our patients. The previously described c.664+3A>G was also detected in two patients, both homozygous. The present work is yet another confirmation that the individual genetic background plays a pivotal role in influencing the phenotypes, as occurs in other metabolic diseases. © 2006 Wiley‐Liss, Inc.
Liver Transplantation for Nonalcoholic Steatohepatitis: The New Epidemic

Objective:To analyze incidence, outcomes, and utilization of health care resources in liver transplantation (LT) for nonalcoholic steatohepatitis (NASH). Summary of Background Data:With the epidemic of obesity and metabolic syndrome in nearly 33% of the US population, NASH is projected to become the leading indication for LT in the next several years. Data on predictors of outcome and utilization of health care resources after LT in NASH is limited. Methods:We conducted an analysis from our prospective database of 144 adult NASH patients who underwent LT between December 1993 and August 2011. Outcomes and resource utilization were compared with other common indications for LT. Independent predictors of graft and patient survival were identified. Results:The average Model for End-Stage Liver Disease score was 33. The frequency of NASH as the primary indication for LT increased from 3% in 2002 to 19% in 2011 to become the second most common indication for LT at our center behind hepatitis C. NASH patients had significantly longer operative times (402 vs 322 minutes; P < 0.001), operative blood loss (18 vs 14 packed red blood cell units; P = 0.001), and posttransplant length of stay (35 vs 29 days; P = 0.032), but 1-, 3-, and 5-year graft (81%, 71%, 63%) and patient (84%, 75%, 70%) survival were comparable with other diagnoses. Age greater than 55 years, pretransplant intubation, dialysis, hospitalization, presence of hepatocellular carcinoma on explant, donor age greater than 55 years, and cold ischemia time greater than 550 minutes were significant independent predictors of survival for all patients, whereas body mass index greater than 35 was a predictor in NASH patients only. Conclusions:We report the largest single institution experience of LT for NASH. Over a 10-year period, the frequency of LT for NASH has increased 5-fold. Although outcomes are comparable with LT for other indications, health care resources are stressed significantly by this new and increasing group of transplant candidates.
The Pasteurella multocida toxin is encoded within a lysogenic bacteriophage

Toxigenic strains of Pasteurella multocida produce a 146 kDa toxin (PMT) that acts as a potent mitogen. Sequence analysis of the structural gene for PMT, toxA, previously suggested it was horizontally acquired, because it had a low G + C content relative to the P. multocida genome. To address this, the sequence of DNA flanking toxA was determined. The sequence analysis showed the presence of homologues to bacteriophage tail protein genes and a bacteriophage antirepressor, suggesting that the toxin gene resides within a prophage. In addition to phage genes, the toxA flanking DNA contained a homologue of a restriction/modification system that was shown to be functional. The presence of a bacteriophage was demonstrated in spent medium from toxigenic P. multocida isolates. Its production was increased by mitomycin C addition, a treatment that is known to induce the lytic cycle of many temperate bacteriophages. The genomes of bacteriophages from three different toxigenic P. multocida strains had similar but not identical restriction profiles, and were approximately 45–50 kb in length. The prophages from two of these had integrated at the same site in the chromosome, in a tRNA gene. Southern blot analysis confirmed that these bacteriophages contained the toxA gene.
Impact of a Better Adherence to Antihypertensive Agents on Cerebrovascular Disease for Primary Prevention

Background and Purpose— The benefits of antihypertensive (AH) drugs on the risks of major cardiovascular outcomes have been demonstrated in clinical trials. However, approximately half of hypertensive patients do not adhere well to their prescribed AH therapy in actual practice. The purpose of this study was to assess the impact of adherence to AH agents on the incidence of cerebrovascular disease (CD) in real-world practice. Methods— A cohort of 83 267 hypertensive patients was reconstructed from the Régie de l'assurance maladie du Québec databases. Subjects included were between 45 and 85 years old, initially free of cardiovascular disease, and newly treated for hypertension with AH agents between 1999 and 2004. A nested case–control design was conducted to study CD occurrence. Every case was matched for age and duration of follow-up with up to 15 randomly selected control subjects. The adherence to AH drugs was measured by calculating the medication possession ratio. Conditional logistic regression models were performed to assess the association between adherence to AH agents and CD adjusting for various potential confounders. Results— At cohort entry, the mean patient age was 65 years, 37.3% were male, 8.6% had diabetes, and 19.5% had dyslipidemia. High adherence (≥80%) to AH drugs significantly decreased the risk of CD by 22% (rate ratio, 0.78; 95% CI, 0.70 to 0.87) compared with lower adherence. Male gender, occurrence of cardiovascular disease during follow-up, and dyslipidemia were risk factors for CD. Conclusion— High adherence to AH therapy is associated with a reduced risk of CD outside the context of clinical trials in primary prevention.
Alanine Expansions Associated with Congenital Central Hypoventilation Syndrome Impair PHOX2B Homeodomain-mediated Dimerization and Nuclear Import*

Heterozygous mutations of the human PHOX2B gene, a key regulator of autonomic nervous system development, lead to congenital central hypoventilation syndrome (CCHS), a neurodevelopmental disorder characterized by a failure in the autonomic control of breathing. Polyalanine expansions in the 20-residues region of the C terminus of PHOX2B are the major mutations responsible for CCHS. Elongation of the alanine stretch in PHOX2B leads to a protein with altered DNA binding, transcriptional activity, and nuclear localization and the possible formation of cytoplasmic aggregates; furthermore, the findings of various studies support the idea that CCHS is not due to a pure loss of function mechanism but also involves a dominant negative effect and/or toxic gain of function for PHOX2B mutations. Because PHOX2B forms homodimers and heterodimers with its paralogue PHOX2A in vitro, we tested the hypothesis that the dominant negative effects of the mutated proteins are due to non-functional interactions with the wild-type protein or PHOX2A using a co-immunoprecipitation assay and the mammalian two-hybrid system. Our findings show that PHOX2B forms homodimers and heterodimerizes weakly with mutated proteins, exclude the direct involvement of the polyalanine tract in dimer formation, and indicate that mutated proteins retain partial ability to form heterodimers with PHOX2A. Moreover, in this study, we investigated the effects of the longest polyalanine expansions on the homeodomain-mediated nuclear import, and our data clearly show that the expanded C terminus interferes with this process. These results provide novel insights into the effects of the alanine tract expansion on PHOX2B folding and activity.
INTRASPECIFIC VARIATION IN EGG SIZE AND EGG COMPOSITION IN BIRDS: EFFECTS ON OFFSPRING FITNESS

1. There is little unequivocal evidence to date in support of a positive relationship between egg size and offspring fitness in birds. Although 40 studies (of 34 species) have considered the effect of variation in egg size on chick growth and/or survival up to fledgling only 12 studies have controlled for other characters potentially correlated both with egg size and offspring fitness. Of these only two have reported a significant residual effect of egg size on chick growth (in the roseate tern and European blackbird) and three a residual effect on chick survival (all in seabirds: common tern, lesser black-backed gull and kittiwake). 2. More consistent evidence exists, though from fewer studies, for a positive relationship between egg size and offspring fitness early in the chick-rearing period; chick growth and chick survival being dependent on egg size in 8 of 10 studies and 4 of 5 studies respectively. It is suggested that the most important effect of variation in egg size might be in determining the probability of offspring survival in the first few days after hatching. 3. Egg size explains on average 66% of the variation in chick mass at hatching (n = 35 studies) but only 30% of the variation in chick body size (n = 18). When effects of hatching body size are controlled for chick mass remains significantly correlated with egg size, though the reverse is not true. This supports the hypothesis that large eggs give rise to heavier chicks at hatching, i.e., chicks with more nutrient (yolk) reserves, rather than structurally larger chicks. 4. Egg composition increased isometrically with increasing egg size in about half the studies so far reported (n equals approximately 20). However, in seabirds, and some passerines, larger eggs contain disproportionately more albumen, whilst in some waterfowl percentage yolk content increases with increasing egg size. Changes in albumen content largely reflect variation in the water content of eggs, but changes in yolk content involve variation in lipid content, and therefore in egg 'quality. ' The adaptive significance of variation in egg composition is considered; females may adjust egg composition facultatively to maximise the benefits to their offspring of increased reproductive investment. 5. Considerations for future research are discussed with particular emphasis on experimental studies and the application of new techniques.
Complete mitochondrial genome of Skylark, Alauda arvensis (Aves: Passeriformes): The first representative of the family Alaudidae with two extensive heteroplasmic control regions

The circular mitochondrial genome of Alauda arvensis is 17,018 bp in length, containing 13 protein-coding genes (PCGs), 2 ribosomal RNA genes, 22 transfer RNA (tRNA) genes, and 2 extensive heteroplasmic control regions. All of the genes encoded on the H-strand, with the exceptions of one PCG (nad6) and eight tRNA genes (tRNAGln, tRNAAla, tRNAAsn, tRNACys, tRNATyr, tRNASer(UCN), tRNAPro, and tRNAGlu), as found in other birds' mitochondrial genomes. All of these PCGs are initiated with ATG, while stopped by six types of stop codons. All tRNA genes have the potential to fold into typical clover-leaf structure. Two extensive heteroplasmic control regions were found, and more interestingly, a minisatellite of 37 nucleotides (5′-TCAATCCCATTGATTTCATTATATTAGTATAAAGAAA-3′) with 6 tandem repeats was detected at the end of CR2.