Miracle-dz/UniFAR-FTUs huggingface dataset:
Laboratory diagnosis of hereditary bleeding disorders: external quality assessment

A World Federation of Hemophilia external quality assessment scheme has been established to promote high standards of laboratory performance in haemophilia centres world-wide. Results from 22 International Haemophilia Training Centres (IHTCs) provide target values for the prothrombin time (PT), activated partial thromboplastin time (APTT), factor VIII:C, IX:C and von Willebrand factor assays, against which the performance of haemophilia centres (HCs) in developing countries can be assessed. A higher proportion of HCs failed to identify an abnormal PT or APTT in samples from donors with mild deficiencies of the extrinsic and intrinsic systems, respectively. For factor VIII:C and IX:C assays, agreement between HC results was consistently poorer than between IHTCs. However, improvement in between-centre agreement could be seen for two samples distributed on more than one occasion. A minority of HCs perform assays for von Willebrand factor, and a questionnaire revealed equipment and reagent costs as limiting the range of assays which could be carried out in several centres. However, agreement was in some cases better between those HCs that did perform VWF assays, than between IHTCs. The problems of screening test sensitivity, and between-centre agreement for factor assays need to be addressed, together with the limitations which prevent HCs from performing a full range of tests in the diagnosis and treatment of bleeding disorders.
Use of non-thermal atmospheric plasmas to reduce the viability of Bacillus subtilis on spacecraft surfaces

Abstract Atmospheric pressure glow-discharge (APGD) plasmas have been proposed for sterilizing spacecraft surfaces prior to launch. The advantages of APGD plasmas for the sterilization of spacecraft surfaces include low temperatures at treatment sites, rapid inactivation kinetics of exposed microbial cells, physical degradation and removal of microbial cells, physical removal of organic biosignature molecules, and short exposure times for the materials. However, few studies have tested APGD plasmas on spacecraft materials for their effectiveness in both sterilizing surfaces and removal of microbial cells or spores. A helium (He)+oxygen (O2) APGD plasma was used to expose six spacecraft materials (aluminum 6061, polytetrafluoroethylene (PTFE), polycarbonate, Saf-T-Vu, Rastex, and Herculite 20) doped with spores of the common spacecraft contaminant, Bacillus subtilis, for periods of time up to 6 min. Results indicated that greater than six orders of magnitude reductions in viability were observed for B. subtilis spores in as short of time as 40 s exposure to the APGD plasmas. Spacecraft materials were not affected by exposures to the APGD plasmas. However, Saf-T-Vu was the only material in which spores of B. subtilis adhered more aggressively to plasma-treated coupons when compared to non-plasma treated coupons; all other materials exhibited no significant differences between plasma and non-plasma treated coupons. In addition, spores of B. subtilis were physically degraded by exposures to the plasmas beginning at the terminal ends of spores, which appeared to be ruptured after only 30 s. After 300 s, most bacteria were removed from aluminium coupons, and only subtle residues of bacterial secretions or biofilms remained. Results support the conclusion that APGD plasmas can be used as a prelaunch cleaning and sterilization treatment on spacecraft materials provided that the biocidal and cleaning times are shorter than those required to alter surface properties of materials.
Kinetics of T-cell Receptor Binding by Bivalent HLA-DR·Peptide Complexes That Activate Antigen-specific Human T-cells*

Monovalent major histocompatibility complex-peptide complexes dissociate within seconds from the T-cell receptor (TCR), indicating that dimerization/multimerization may be important during early stages of T-cell activation. Soluble bivalent HLA-DR2·myelin basic protein (MBP) peptide complexes were expressed by replacing the F(ab) arms of an IgG2a antibody with HLA-DR2·MBP peptide complexes. The binding of bivalent HLA-DR2·peptide complexes to recombinant TCR was examined by surface plasmon resonance. The bivalent nature greatly enhanced TCR binding and slowed dissociation from the TCR, with a t 1 2 of 2.1 to 4.6 min. Soluble bivalent HLA-DR2·MBP peptide complexes activated antigen-specific T-cells in the absence of antigen presenting cells. In contrast, soluble antibodies to the TCR·CD3 complex were ineffective, indicating that they failed to induce an active TCR dimer. TCR/CD3 antibodies induced T-cell proliferation when bound by antigen presenting cells that expressed Fc receptors. In the presence of dendritic cells, bivalent HLA-DR2·MBP peptide complexes induced T-cell activation at >100-fold lower concentrations than TCR/CD3 antibodies and were also superior to peptide or antigen. These results demonstrate that bivalent HLA-DR·peptide complexes represent effective ligands for activation of the TCR. The data support a role for TCR dimerization in early TCR signaling and kinetic proofreading.
Perspectivesin CancerResearch Conditioned Tumorigenicity of Activated Oncogenes 1

In his classical review on conditioned and autonomous neo plasms (1) Jacob Furili defined distinct stages in the develop ment and progression of hormone-dependent tumors. His in terest in the endocrine organs and their target tissues was based on the insight that they provided the best available system to analyze the relationship between well defined host factors that regulate the number of cells in a given tissue and their targets. He suggested that the principles derived from the study of hormone-dependent tissues and their tumors may also be ap plicable to other cells that are regulated by unknown homeostatic controls. He found that an initial proliferative lesion could be induced either by chronic overproduction of a stimu latory hormone or by prolonged deficiency of an inhibitory hormone. The same regulatory circuit can be affected by both, with different consequences, as exemplified by the appearance of thyroid tumors under the influence of thyrotropic hormone, and by the development of thyrotropic pituitary adenomas in animals deprived of thyroid hormone. Much of Furth's reasoning was based on naturally occurring
Mechanism of perpetuation of animal viruses in nature.

INTRODUCTION. 404 PERPETUATION BY SHORT CYCLE. 404 Measles Virus . 405 Viruses Perpetuating in Man. 405 Viruses Perpetuating in Animals. 406 PERPETUATION BY RESISTANT VIRUS. 409 Fowl Pox and Variola Viruses. 409 Newcastle Disease Virus. 409 PERPETUATION BY CHRONIC INFECTION. 410 Herpesviruses. 410 Rabies virus. 411 Adenoviruses. 412 Rabbit Myxoma Virus . 412 Viruses Perpetuating by Congenital Transmission. 412 PERPETUATION BY LOWER-ANIMAL VECTORS. 413 Endoparasite Vector Type. 414 Arthropod Vector Type. 414 SUMMARY. 416 LITERATURE CITED . 417
PREFERENCES for Teaching Methods in a Baccalaureate Nursing Program: HOW Second-Degree and Traditional Students DIFFER

Accelerated baccalaureate nursing programs are in great demand in the United States. Currently there are 197 such programs, but little research has been conducted on student characteristics and program outcomes. This quantitative study explores preferences of second-degree students and traditional generic students with regard to teaching methods and relationships with faculty. The results indicate that statistically significant differences exist between the two groups of students. Three areas of significance are ability for self-directed learning, expectations of faculty and classroom structure, and obtaining a grade that really matters.
The long-term outcome in children with late-onset aqueductal stenosis resulting from benign intrinsic tectal tumors.

Benign intrinsic tumors arising in the dorsal midbrain have long been recognized as a potential cause of late-onset aqueductal stenosis. Where histopathological studies of such lesions have been performed, the majority have been reported to be low-grade gliomas. Because these tumors often present with a paucity of neurological findings and a characteristic radiographic appearance and because there has been substantial uncertainty regarding their potential for long-term progression, the authors have routinely deferred biopsy and/or radiotherapy for these lesions until there has been clear-cut evidence of disease progression. Herein, the authors report their experience with 16 children manifesting this syndrome who were treated between 1979 and 1992. The patients ranged in age from 6 months to 14 years at presentation (median 9.75 years). In general, symptoms of increased intracranial pressure developed insidiously; three of the older children had exhibited profound macrocephaly since infancy, which predated the onset of other symptoms of hydrocephalus by several years. Only one of the 16 children showed evidence of brain-stem dysfunction at presentation, a partial Parinaud's syndrome that resolved following placement of a ventriculoperitoneal shunt. In 12 patients, the tumor was detected by magnetic resonance (MR) imaging at initial evaluation as a bulbous enlargement of the tectal plate. In four patients who presented before the advent of MR imaging, initial computerized tomography (CT) scans failed to delineate the tectal lesion convincingly; however, subsequent MR studies clearly demonstrated the presence of an intrinsic tectal mass. All 16 patients underwent cerebrospinal fluid diversion initially, with conservative management of the tectal lesion and close long-term follow-up monitoring. Four children ultimately demonstrated clinical signs of progressive tumor growth with the insidious onset of partial or complete Parinaud's syndrome, despite the presence of a functioning shunt. The median interval to symptom progression was 7.8 years from the time of shunt insertion and 11.5 years from the onset of initial symptoms and signs of hydrocephalus. Follow-up CT and MR studies demonstrated obvious tumor enlargement in three of the four patients who then underwent stereotactic or open biopsy. The histological diagnosis in these three was benign mixed glioma, anaplastic astrocytoma, and low-grade astrocytoma. All four patients with clinical evidence of disease progression were treated with conventional radiotherapy; the patient with an anaplastic astrocytoma also received focal stereotactic radiosurgery. These patients subsequently remained clinically stable, with three showing tumor regression and one showing stable disease on serial MR studies (median follow-up period from tumor progression, 4.25 years). (ABSTRACT TRUNCATED AT 400 WORDS)
Protective Role of Silymarin on Hepatic and Renal Toxicity Induced by MTX Based Chemotherapy in Children with Acute Lymphoblastic Leukemia

Background ALL is the most common childhood malignancy. The children with ALL are treated with methotrexate (MTX) based chemotherapy protocols. MTX causes unpredictable serious hepatic and renal side effects. Silymarin has antioxidant and anti-inflammatory activities and stimulates tissue regeneration. This study aims to evaluate the protective effects of Silymarin on MTX-based chemotherapy-induced Hepatic and renal toxicity in children with ALL. Patients and Methods 80 children with newly diagnosed ALL were enrolled in the study. They were randomly divided into two groups. Group I included 40 children with ages ranging from 4–13 years and the mean age of 6.85± 2.89 years, who received Silymarin 420 mg/day in 3 divided doses for one week after each MTX dose. Group II included 40 children, with ages ranging from 4–12 years and the mean age of 7.30±2.6 years, who received placebo for one week after MTX therapy. For all patients liver functions including serum bilirubin, total proteins, albumin, globulin and albumin-globulin ratio, alkaline phosphatase, ALT and AST, prothrombin time and activity and renal functions including blood urea and serum creatinine, serum cystatin C and urinary N-acetyl-beta-D-glucosaminidase were done to assess hepatic and renal toxicity before and after chemotherapy. Results There were no significant differences between group I and II as regard liver and renal functions before chemotherapy. After chemotherapy, there were significantly higher values of ALT and AST and alkaline phosphatase, and significantly lower Prothrombin activity in group II compared with group I. No significant differences between group I and II were found in total bilirubin, serum protein, and albumin levels. There was significantly lower blood urea, serum creatinine, and cystatin C and urinary N-acetyl-beta-D-glucosaminidase in group I compared with group II. Conclusion Silymarin improved some hepatic and renal functions in children with ALL who received MTX-based chemotherapy protocols. Recommendations: Extensive multicenter studies could be recommended to prove the hepatic and renal protective effects of Silymarin in patients with ALL who received MTX-based chemotherapy protocols.
Confirmation of the assignment of the Sanjad-Sakati (congenital hypoparathyroidism) syndrome (OMIM 241410) locus to chromosome lq42-43

Editor—Hereditary pancreatitis (HP) is an autosomal dominant disease with about 80% penetrance that mainly afflicts white families.1 Although pancreatitis was hypothesised to result from inappropriate activation of pancreatic zymogens by Chiara2 in 1896, and the genetic nature of HP was identified by Comfort et al 3 in 1952, the precise mechanism underlying the pathogenesis of HP has remained a mystery until recently. By familial linkage analysis, a genetic defect was mapped to chromosome 7q35 by Le Bodic et al ,4 and independently confirmed by two other groups in 1996.5 6 Soon after, a single G to A mutation resulting in an arginine (R) to histidine (H) substitution (R117H) in the third exon of the cationic trypsinogen gene was identified as being associated with HP by Whitcomb et al . 7 Trypsinogen is synthesised in the acinar cells of the pancreas and is activated into trypsin upon cleavage of the activation peptide by enterokinase. Trypsin plays a central role in pancreatic exocrine physiology by acting as the trigger enzyme which leads to the activation of all the pancreatic digestive proenzymes as well as trypsinogen itself. When the R117H mutation was identified, Whitcomb et al 7 concluded that this mutation did not affect the tertiary structure of trypsin, nor alter its catalytic activity or interfere with trypsin inhibitor binding, since the three dimensional position of R117 was located on the opposite surface of the trypsin molecule to the catalytic and trypsin inhibitor binding sites. They hypothesised instead that the R117H mutation eliminated a “fail safe” mechanism for the inactivation of trypsin by abolishing an important autolytic site. Thus, the stabilised mutant enzyme would disrupt the trypsin activation/inhibition balance and trigger the pancreatic autodigestion process which results in pancreatitis under certain conditions. This model coincided with Chiara's …
Valuing Companies by Cash Flow Discounting: Only APV Does Not Require Iteration

The most used methods for valuing companies by Cash Flow Discounting are equity cash flow, free cash flow, capital cash flow and APV (Adjusted Present Value). Only APV does not require iteration All four methods, if properly applied, always give the same value. This result is logical, as all the methods analyze the same reality under the same hypotheses; they differ only in the cash flows or parameters taken as the starting point for the valuation. Many valuations are incorrect because the authors do not iterate and, therefore, the four methods do not provide the same value.
Quality of life and psychosocial issues are important outcome measures in eczema treatment

Abstract Background: Atopic eczema (AE) is a common relapsing inflammatory skin disease in children associated with chronicity and poor quality of life. Many children also display depressive, anxiety and stress symptoms. Aim: To investigate the prevalence of depressive, anxiety and stress symptoms, and if these symptoms are associated with disease severity, quality of life and skin biophysiology in childhood AE. Methods: Psychological symptoms, eczema severity, quality of life and biophysical skin condition of consecutive adolescents at the pediatric dermatology clinic of a teaching hospital were evaluated with the validated Chinese versions of Depressive, Anxiety, Stress Scales (DASS-42), Beck Depression Inventory (BDI-13), Nottingham Eczema Severity Score (NESS), Children’s Dermatology Life Quality Index (CDLQI), transepidermal water loss (TEWL) and stratum corneum skin hydration (SH), respectively. Results: AE patients (n = 120) had lower SH, higher TEWL, worse CDLQI and reported higher overall, depressive and stress symptom scores, personal history of atopy, current topical corticosteroid usage and food avoidance than non-AE patients (n = 26). Depressive, anxiety and stress symptoms were reported in 21%, 33% and 23% of AE patients, respectively. Multivariate analyses showed that these symptoms were significantly correlated with a poor quality of life (partial correlations of 0.40–0.49; p < 0.001). Male patients had more severe disease (higher NESS, p = 0.036) and DASS-depressive symptoms (multivariate OR = 3.2, p = 0.034) than females. Patients who reported current topical steroid usage generally practiced food avoidance (p = 0.047), had poor quality of life (p = 0.043) but less DASS-depression (multivariate OR = 0.354, p = 0.043). Only 6% of the 120 AE patients reported prior psychology consultation. Conclusions: Quality of life impairments correlate with disease severity, aberrant skin biophysiology, depression, anxiety and stress symptoms in adolescents with AE. Physicians caring for these patients must evaluate the different but inter-correlated medical, biophysiological and pertinent psychosocial domains. These significant correlations imply that a holistic approach should encompass psychotherapy, behavioral therapy and coping strategies in conjunction with dermatologic therapy.
Detection and localization of prostate cancer: correlation of (11)C-choline PET/CT with histopathologic step-section analysis.

UNLABELLED This study evaluated the potential usefulness of (11)C-choline PET/CT for detection and localization of tumors within the prostate. We used the results of step-section histopathologic examination as the standard of reference. METHODS The results were analyzed on a sextant basis. We reviewed the results of the (11)C-choline PET/CT scans of 36 patients with prostate cancer and of 5 control subjects with bladder cancer. All patients underwent (11)C-choline PET/CT and, subsequently, radical prostatectomy with lymph node dissection within 1 mo. (11)C-Choline PET/CT scans were obtained 5-10 min after intravenous injection of 370-555 MBq of (11)C-choline. Images were reviewed visually and semiquantitatively using maximum SUV and tumor-to-background ratio. RESULTS On a sextant basis, histopathologic analysis detected cancer foci in 143 of 216 sextants; high-grade prostate intraepithelial neoplasm foci were detected in 89 of 216 sextants (in 59 sextants in association with carcinoma, in 30 sextants alone), acute prostatitis was detected in 7 of 216 sextants (in 3 sextants in association with carcinoma, in 4 sextants alone), and 39 of 216 sextants were normal. PET/CT demonstrated focal (11)C-choline uptake in 108 sextants (94 of which involved tumor), and 108 sextants showed no abnormal (11)C-choline uptake (49 of which were false negative). The sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of PET/CT were 66%, 81%, 71%, 87%, and 55%, respectively. In the 5 control subjects, high-grade prostate intraepithelial neoplasm was detected at histologic examination in 16 of 30 sextants. PET/CT showed increased (11)C-choline uptake in 5 of 16 sextants. CONCLUSION This study demonstrated the feasibility of using (11)C-choline PET/CT to identify cancer foci within the prostate. However, we also found that (11)C-choline PET/CT has a relative high rate of false-negative results on a sextant basis and that prostatic disorders other than cancer may accumulate (11)C-choline. Therefore, our data do not support the routine use of PET/CT with (11)C-choline as a first-line screening procedure for prostate cancer in men at high risk.
Temporal trends in cause-specific late mortality among 5-year survivors of childhood cancer.

PURPOSE Five-year survival rates for childhood cancer have improved over the past four decades. However, it is unknown whether changes in primary cancer therapy have improved rates of long-term (> 5 years from diagnosis) durable remissions and reduced treatment-related deaths. We investigated changes in patterns of late mortality over time and cause-specific attribution of late-mortality among 5-year survivors. PATIENTS AND METHODS Using data from the Surveillance, Epidemiology and End Results (SEER) population-based registry, we assessed all-cause and cause-specific (recurrence/progression of primary disease, external cause, and nonrecurrence/nonexternal cause) late mortality during four consecutive time periods from 1974 through 2000 among 26,643 5-year survivors of childhood cancer. RESULTS All-cause late mortality improved during more recent eras, dropping from 7.1% (95% CI, 6.4% to 7.8%) among children diagnosed during 1974 to 1980 to 3.9% (95% CI, 3.3% to 4.4%) among children diagnosed during 1995 to 2000 (P < .001), largely because of reduced mortality from recurrence or progression. While there was no significant reduction in mortality attributable to other health conditions (including treatment-related health conditions), analysis controlling for demographic characteristics identified a trend toward reduced risk during more recent eras (P = .007). Disparity by race/ethnicity was identified, with higher mortality among non-Hispanic blacks than among non-Hispanic whites for all-cause and nonrecurrence/nonexternal -cause late mortality. CONCLUSION While overall patterns of mortality from other health conditions do not differ over time, adjustment for demographic characteristics provides evidence that risk of treatment-related mortality may be lower in more recent eras. Disparities in health care utilization among survivors should be explored.
Efficacy of day care treatment against readmission in patients with schizophrenia: A comparison between out‐patients with and without day care treatment

Abstract The present study examined the efficacy of day care (DC) treatment against readmission to a mental hospital. Subjects were 65 out‐patients with chronic schizophrenia after discharge. Day care treatment was defined as positive if patients were under DC treatment constantly for 4 months or more with a frequency of at least one visit per week. Information regarding demographic and disease‐related factors was obtained from medical records at the time of discharge. Logistic regression analysis was used to calculate odds ratios (OR) and 95% confidence intervals (CI) with adjustment for confounding variables. The modestly preventive efficacy of DC treatment against readmission was observed within 2 years after discharge (adjusted OR 0.52; 95% CI 0.12–2.35). The present findings suggest that DC treatment may be preventive against readmission for schizophrenic out‐patients.
A Theoretical Perspective of Negotiation Support Systems

Abstract:This paper provides an overview of the existing negotiation literature under the headings of game theory, economic models, political models, and social-psychological models. Inferring from the review the need for computer support for negotiation, the paper then proposes a theory for understanding the effects due to a support system in a two-party, monolithic, and multiple-issue setting. The theory conceptualizes a negotiation support system as consisting of individual decision support systems interconnected with an electronic communication channel; accordingly, it postulates two sets of effects, one owing to the decision aid, and the other to the communication support.