Miracle-dz/UniFAR-FTUs huggingface dataset:
HAEMOPHILIA PROPHYLAXIS IN SWEDEN

ABSTRACT. 29 boys (4–18 years old) with severe haemophilia A were given prophylactic infusions of AHF concentrate (human fraction I–0) for 2 to 13 years in an attempt to change the haemophilia from a severe to a moderate form and thereby prevent arthropathy and severe bleeding episodes. The sizes of the doses and the intervals at which the doses were given were titrated by AHF survival studies. As a rule, the patients received AHF in amounts sufficient to raise the AHF level to 30–45% at 5–12 day intervals. In about 50% of the infusions the AHF content was not below 1% before the next infusion. During such prophylaxis all patients except one have been in a good general condition. They have had bleeding episodes, which have, however, been much less severe and less frequent. The children have been able to live an almost normal life. The number and duration of stays in hospital have been markedly reduced. 17 of the patients had only minor or no joint defects before the start of the treatment. In this group the joint function was identical with that found in moderate haemophilia in the same age groups. Two patients developed anticoagulants. No other side effects were seen.
A new syndrome combining developmental anomalies of the eyelids, eyebrows and nose root with pigmentary defects of the iris and head hair and with congenital deafness.

First observations . 196 A search among institutionalized deafmutes . 199 Descriptions of the investigated families . 201 Cases of the syndrome not examined by myself . 225 Peculiar observations of ocular abnormalities in deafmutes, perhaps related to the syndrome 226 Dystopia canthi medialis lateroversa, as the most frequent anomaly . 227 Genetical and statistical considerations Genetics of the syndrome as a whole . 229 Degrees of penetrance of the various component anomalies . 231 Frequency of the syndrome among deafrmutes and in the general population . 234 Inheritance of the separate anomalies and their combinations in man and other mammals Dystopia canthi medialis et punctorumin lacrimalium lateroversa . 238 Hyperplasia supercilii mediadis et radicis nasi . 239 Heterochromia iridum totalis size partialis . 240 Leucisunus pilorum (poliosis, "white forelock") . 241 Surditas congenita (surdimutitas) . 242 Pigmentary abnormalities and deafness in other mammals . 245 Some remarks on the phenogenetic mechanism . 247 Summary. 249 References. 249
Culture and the Home-Field Disadvantage

The home-field disadvantage refers to the disadvantage inherent in research that takes a particular cultural group as the starting point or standard for research, including cross-cultural research. We argue that home-field status is a serious handicap that often pushes researchers toward deficit thinking, however good the researchers' intentions may be. In this article, we aim to make this home-field bias more explicit and, in doing so, more avoidable. We discuss three often-overlooked disadvantages that result from this home-field status: the problem of marked versus unmarked culture, the problem of homogenous versus heterogeneous culture, and the problem of regression toward the mean. We also recommend four interventions researchers can apply to avoid the home-field disadvantage or, at the least, attenuate its deleterious effects.
Identification of residues in the insulin molecule important for binding to insulin-degrading enzyme.

Insulin-degrading enzyme (IDE) hydrolyzes insulin at a limited number of sites. Although the positions of these cleavages are known, the residues of insulin important in its binding to IDE have not been defined. To this end, we have studied the binding of a variety of insulin analogues to the protease in a solid-phase binding assay using immunoimmobilized IDE. Since IDE binds insulin with 600-fold greater affinity than it does insulin-like growth factor I (25 nM and approximately 16,000 nM, respectively), the first set of analogues studied were hybrid molecules of insulin and IGF I. IGF I mutants [insB1-17,17-70]IGF I, [Tyr55,Gln56]IGF I, and [Phe23,Phe24,Tyr25]IGF I have been synthesized and share the property of having insulin-like amino acids at positions corresponding to primary sites of cleavage of insulin by IDE. Whereas the first two exhibit affinities for IDE similar to that of wild type IGF I, the [Phe23,Phe24,Tyr25]IGF I analogue has a 32-fold greater affinity for the immobilized enzyme. Replacement of Phe-23 by Ser eliminates this increase. Removal of the eight amino acid D-chain region of IGF I (which has been predicted to interfere with binding to the 23-25 region) results in a 25-fold increase in affinity for IDE, confirming the importance of residues 23-25 in the high-affinity recognition of IDE. A similar role for the corresponding (B24-26) residues of insulin is supported by the use of site-directed mutant and semisynthetic insulin analogues. Insulin mutants [B25-Asp]insulin and [B25-His]insulin display 16- and 20-fold decreases in IDE affinity versus wild-type insulin. (ABSTRACT TRUNCATED AT 250 WORDS)
REVISITING THE INFLUENCE OF UNIDENTIFIED BINARIES ON VELOCITY DISPERSION MEASUREMENTS IN ULTRA-FAINT STELLAR SYSTEMS

Velocity dispersion measurements of recently discovered Milky Way satellites with MV ≳ −7 imply that they posses high mass-to-light ratios. The expected velocity dispersions due to their baryonic mass are ∼0.2 km s−1, but values ≳3 km s−1 are measured. We perform Monte Carlo simulations of mock radial velocity measurements of these systems assuming that they have mass-to-light ratios similar to globular clusters and posses an unidentified binary star population, to determine if these stars could boost the velocity dispersion to the observed values. We find that this hypothesis is unlikely to produce dispersions much in excess of ∼4.5 km s−1, in agreement with previous work. However, for the systems with the potentially smallest velocity dispersions, values consistent with observations are produced in 5%–40% of our simulations for binary fractions in excess of fbin(P ⩽ 10 yr)∼ 5%. This sample includes the dwarf galaxy candidates that lie closest to classical globular clusters in MV − rh space. Considered as a population, it is unlikely that all of these dwarf galaxy candidates have mass-to-light ratios typical of globular clusters, but boosting of the observed dispersion by binaries from near-zero values cannot be ruled out at high confidence for several individual dwarf galaxy candidates. Given the importance of obtaining accurate velocity dispersions and dynamical masses for the faintest satellites, it is clearly desirable to directly exclude the possible effect of binaries on these systems. This requires multi-epoch radial velocity measurements with individual uncertainties of ≲1 km s−1 to identify spectroscopic binaries with orbital velocities of the order of the observed velocity dispersion.
Gastroenteritis associated with accidental contamination of drinking water with partially treated water

SUMMARY Due to human error, drinking water supplied to a new housing estate in The Netherlands was contaminated with grey water. The cohort of 921 accidentally exposed households (area A) had a higher attack rate for diarrhoea (54·1%) than a non-exposed cohort of 1529 households from an adjacent area (B) (24%) (RR 2·3, 95% CI 1·9–2·7). Household water score showed a dose-response with illness, in both areas A and B. For each 1000 inhabitants, 19·8 cases in area A, 7·0 cases in control area B (RRAB 2·2, 95% CI 1·3–3·8) and 3·3 cases in a more distant control area C (RRAC 4·6, 95% CI 2·7–8·0) were diagnosed with gastroenteritis by their general practitioner. A gastroenteritis outbreak associated with consumption of contaminated drinking water was observed in the exposed area. The use of grey water was banned in 2003, with the exception of rainwater use for flushing toilets. The risk of rainwater use is currently being investigated.
Do the Spanish regions converge? A unit root analysis for the HDI of the Spanish regions

This article studies the possible stochastic convergence between the Spanish regions in 1980–2010. The application of unit root techniques to the new Human Development Index recently calculated in Herrero et al. (2013) allows us to show that the evolution of the Spanish economy can be better understood as the sum of divergent forces rather than as a group of convergent regions. Similar conclusions can be drawn when the per capita GDP is used, although these two variables exhibit different patterns of behaviour at the end of the sample. Finally, we also observe that the distance between northern and southern regions has increased since 2000.
Mitral valve replacement with chordal preservation in a rheumatic population.

BACKGROUND AND AIM OF THE STUDY Mitral valve replacement (MVR) with preservation of the subvalvular apparatus may maintain postoperative left ventricular (LV) function better than conventional MVR. A prospective study was undertaken to assess the advantages of chordal preservation in rheumatic patients undergoing isolated MVR. METHODS Between January 1996 and January 1999, 240 patients with rheumatic etiology and normal LV ejection fraction (>50%) underwent isolated MVR using a St. Jude Medical prosthesis. Patients were allocated to two groups: in group I (n = 168), both the anterior and posterior chordae were preserved; in group II (n = 72), the entire native mitral valve apparatus was excised. Patients from both groups were evaluated postoperatively (mean 12.5 months) by echocardiography and treadmill testing. RESULTS Demographic and clinical profiles were comparable in both groups. There were three early deaths in group I, and one in group II. There were no late deaths. One patient in group I required reoperation for a stuck mitral prosthesis. LV function (ejection fraction >50%) was better in group I (94%) than in group II (82%) (p <0.05). No patient in either group had LV outflow tract obstruction on echocardiography. Patients in group I showed better exercise performance on treadmill (Bruce protocol): 92% of group I patients versus 88% of group II patients (p >0.05) completed stage I; 16% of group I patients, but no group II patients, were able to complete stage IV (p <0.05). CONCLUSION Mitral valve replacement with preservation of the subvalvular apparatus maintains LV function, and does not cause LV outflow tract obstruction.
How do health care organizations take on best practices? A scoping literature review

Aims: The aims of this scoping literature review are to examine and summarize the organizational-level factors, context, and processes that influence the use of evidence-based practice in healthcare organizations. Methods: A scoping literature review was done to answer the question: What is known from the existing empirical literature about factors, context, and processes that influence the uptake, implementation, and sustainability of evidence-based practice in healthcare organizations? This review used the Arksey and O’Malley framework to describe findings and to identify gaps in the existing research literature. Inclusion and exclusion criteria were developed to screen studies. Relevant studies published between January 1991 and March 2014 were identified using four electronic databases. Study abstracts were screened for eligibility by two reviewers. Following this screening process, full-text articles were reviewed to determine the eligibility of the studies by the primary author. Eligible studies were then analyzed by coding findings with descriptive labels to distinguish elements that appeared relevant to this literature review. Coding was used to form categories, and these categories led to the development of themes. Results: Thirty studies met the eligibility criteria for this literature review. The themes identified were: the process organizations use to select evidence-based practices for adoption, use of a needs assessment, linkage to the organization's strategic direction, organizational culture, the organization's internal social networks, resources (including education and training, presence of information technology, financial resources, resources for patient care, and staff qualifications), leadership, the presence of champions, standardization of processes, role clarity of staff, and the presence of social capital. Conclusion: Several gaps were identified by this review. There is a lack of research on how evidence-based practices may be sustained by organizations. Most of the research done to date has been cross-sectional. Longitudinal research would give insight into the relationship between organizational characteristics and the uptake, implementation, and sustainability of evidence-based practice. In addition, although it is clear that financial resources are required to implement evidence-based practice, existing studies contain a lack of detail about the cost of adopting and using new practices. This scoping review contains a number of implications for healthcare administrators, managers, and providers to consider when adopting and implementing evidence-based practices in healthcare organizations.
Putting sexuality (back) into HIV/AIDS: Issues, theory and practice1

Abstract After more than twenty years of programming and activism aimed at stemming the sexual transmission of HIV (and addressing the needs of those most vulnerable to infection) the HIV/AIDS epidemic continues to grow worldwide. Taking up this concern, this paper argues that one of the reasons why HIV prevention has had limited success is because of inadequate conceptualization of human sexuality in such work. Giving sexuality a more prominent position in responses to the epidemic raises a range of issues, including theorization of gender, understanding of sexual subjectivity, the significance of pleasure (or lack of pleasure) in sexual decision-making, and conceptualization of sexual behaviour and culture. Taking these themes forward entails asking significant questions about the underlying paradigmatic and methodological commitments of mainstream HIV/AIDS research, especially the tendency to reproduce accounts of human sexuality as if it were a measurable form of conduct only. Advocating new approaches that take the meaning and symbolic value of sexualities into account complicates established orthodoxies in the field whilst offering potential for more effective HIV prevention strategies.
Impact of malglycemia on clinical outcomes in hospitalized patients with cancer: a review of the literature.

PURPOSE/OBJECTIVES To examine empirical research regarding the role of malglycemia (hyperglycemia, hypoglycemia, or glycemic variability) on clinical outcomes among hospitalized patients with cancer. DATA SOURCES Research articles were obtained from the Ovid, PubMed, and CINAHL® databases. Key words used in the search included hyperglycemia or malglycemia and neoplasm combined with venous thromboembolism, infection, or mortality. DATA SYNTHESIS Eleven research articles were examined reporting the impact of malglycemia on various outcomes, including infection, mortality or survival, length of hospital stay, and toxicity. CONCLUSIONS Findings suggest that malglycemia may have a negative impact on outcomes for hospitalized patients with cancer. Increased rates of infection, mortality, length of stay, and toxicities, as well as decreased survival, were reported. IMPLICATIONS FOR NURSING Oncology nurses play an important role in the identification of patients with malglycemia. Early assessment and intervention for those patients can improve outcomes and quality of life.
Central Anterior Chamber Depth Changes After Prophylactic Laser Iridotomy

Purpose To evaluate the effect of laser peripheral iridotomy (LPI) on biometric parameters including central anterior chamber depth (CACD) in primary angle closure suspect eyes. Methods Eyes classified as primary angle closure suspect were enrolled in this interventional case series. Biometry with Lenstar (Haag-Streit AG, Koeniz, Switzerland) was performed before LPI and 6 weeks afterward. Central anterior chamber depth, lens thickness, axial length, and central corneal thickness were measured before and after the intervention. Results Forty eyes of 40 patients with mean age of 56.5 ± 5.5 years were studied. Mean central anterior chamber depth was 2.07 ± 0.21 mm before and 2.08 ± 0.19 mm after LPI (p = 0.8). There was no significant change in lens thickness, axial length, and central corneal thickness after LPI. Conclusions Central anterior chamber depth “measured by Lenstar” does not change after LPI in primary angle closure suspect eyes.
Effects of Topical Janus Kinase Inhibition on Ocular Surface Inflammation and Immunity

Purpose: To determine the effects of topical Janus kinase inhibition on ocular surface inflammation and immunity. Methods: Ophthalmic 0.003% tofacitinib (CP-690,550) was administered topically to inhibit Janus kinase activation at the ocular surface. Male BALB/c mice 6 to 8 weeks of age were subjected to corneal thermocautery and randomized to receive tofacitinib, vehicle, or no treatment. Corneas were subsequently excised for fluorescence-activated cell sorting and quantitative real-time reverse transcription polymerase chain reaction. Female C57BL/6 mice 6 to 8 weeks of age were exposed to desiccating stress to induce experimental dry eye disease and randomized to receive tofacitinib, tofacitinib and vehicle, vehicle, or no treatment. Corneal fluorescein staining was performed to evaluate clinical disease severity. The corneas and conjunctivae were harvested for immunohistochemical staining and quantitative real-time reverse transcription polymerase chain reaction. Results: After corneal thermocautery, it was found that tofacitinib treatment decreased the corneal infiltration of CD45+, Gr-1+, and CD11b+ cells on days 1 and 3. Transcripts encoding interleukin (IL)-1&bgr; and IL-6 were significantly decreased by tofacitinib treatment at post-thermocautery day 3. In experimental dry eye disease, tofacitinib treatment twice per day significantly decreased corneal fluorescein staining on days 12 and 15. The corneal infiltration of CD11b+ cells was significantly decreased by tofacitinib treatment twice per day. Tofacitinib treatment twice per day significantly increased the corneal expression of IL-1RA, and significantly decreased the corneal expression of tumor necrosis factor and IL-23. Further, tofacitinib treatment twice per day significantly decreased the conjunctival expression of IL-17A and significantly increased the conjunctival expression of FoxP3. Conclusions: Topical ophthalmic tofacitinib, a Janus kinase inhibitor, suppressed ocular surface inflammation and immunity in experimental corneal thermocautery and dry eye disease.
The LIM-Only Protein PINCH Directly Interacts with Integrin-Linked Kinase and Is Recruited to Integrin-Rich Sites in Spreading Cells

ABSTRACT PINCH is a widely expressed and evolutionarily conserved protein comprising primarily five LIM domains, which are cysteine-rich consensus sequences implicated in mediating protein-protein interactions. We report here that PINCH is a binding protein for integrin-linked kinase (ILK), an intracellular serine/threonine protein kinase that plays important roles in the cell adhesion, growth factor, and Wnt signaling pathways. The interaction between ILK and PINCH has been consistently observed under a variety of experimental conditions. They have interacted in yeast two-hybrid assays, in solution, and in solid-phase-based binding assays. Furthermore, ILK, but not vinculin or focal adhesion kinase, has been coisolated with PINCH from mammalian cells by immunoaffinity chromatography, indicating that PINCH and ILK associate with each other in vivo. The PINCH-ILK interaction is mediated by the N-terminal-most LIM domain (LIM1, residues 1 to 70) of PINCH and multiple ankyrin (ANK) repeats located within the N-terminal domain (residues 1 to 163) of ILK. Additionally, biochemical studies indicate that ILK, through the interaction with PINCH, is capable of forming a ternary complex with Nck-2, an SH2/SH3-containing adapter protein implicated in growth factor receptor kinase and small GTPase signaling pathways. Finally, we have found that PINCH is concentrated in peripheral ruffles of cells spreading on fibronectin and have detected clusters of PINCH that are colocalized with the α5β1 integrins. These results demonstrate a specific protein recognition mechanism utilizing a specific LIM domain and multiple ANK repeats and suggest that PINCH functions as an adapter protein connecting ILK and the integrins with components of growth factor receptor kinase and small GTPase signaling pathways.
An adaptive MAC protocol for MC-CDMA ad hoc wireless LAN

Multi-carrier code division multiple access (MC-CDMA), a novel, high capacity, multicarrier modulation scheme, is developing to a key radio transmission technology for future wireless local area networks (W-LANs). However in ad hoc code division multiple access (CDMA) networks near-far effects can block a receiver due to high interference. These effects occur when a receiving station is closer to an interferer than to its corresponding transmitter. Accordingly, the receiver cannot detect the intended signal out of the received one and the data transmission fails. In this work, we present an adaptive medium access control (MAC) protocol for ad hoc MC-CDMA based W-LANs, based on the IEEE 802.11 MAC protocol. The new protocol has the ability to overcome the near-far-problem by employing a frequency adaptation method. The key of the proposed method is an interference estimate built at each receiving mobile station (MS). Aided by its estimate, a MS can calculate whether the quality of service (QoS) expectations of the incoming link can be accomplished. If the interference is too high, the receiving MS initiates a frequency channel change and informs the corresponding transmitter over the control packets about the Id of the new channel. It is shown that this concept can deploy the high capacity characteristics of MC-CDMA in wireless environments
Nasal Obstruction Due to Enchondromas in Ollier's Disease

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