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Severely immunocompromised NOD.Cg-Prkdc

BACKGROUND/OBJECTIVES Curbside consultations are common in pediatric dermatology. There are both associated risks and benefits. Email, text messaging, and cell phone cameras have greatly facilitated this practice. We sought to characterize the nature of this practice among pediatric dermatologists and highlight concerns. METHODS A 21-question anonymous survey was sent to the 486 active members of the Society for Pediatric Dermatology. RESULTS There were 156 responses (32%). Over 45% of respondents received at least six consults per week. About half (49%) spent 6 minutes or more per case. Almost none of these consultations (3%) were compensated or captured in work relative value units. Most (87%) did not document or have a practice or institutional policy in place to address these consultations. A similar majority (80%) were uncertain if their existing liability insurance covered these activities. Over three-quarters (76%) of respondents did not think or were unsure that Health Insurance Portability and Accountability Act concerns were appropriately addressed. CONCLUSIONS Curbside consultations in pediatric dermatology are common. They increase access, promote collegiality, and can be used for educational gain. They are also generally not compensated, consume considerable time, risk liability exposure for providers, and potentially compromise patient confidentiality. Effort should be made to standardize this practice so that the benefits are not outweighed by risks.

Essentials In 2016 the SSC proposed definitions for effective hemostasis in management of major bleeding. To validate these definitions, we studied the use in three large anticoagulant-reversal studies. Method agreement analysis and interobserver reliability showed at least acceptable agreement. Recommendations were made, advising use of the definition in hemostatic effectiveness studies. SUMMARY: Introduction In 2016 the Scientific and Standardization Subcommittee (SSC) on Control of Anticoagulation of the International Society on Thrombosis and Haemostasis (ISTH) proposed criteria to evaluate the effectiveness of anticoagulant reversal in major bleeding management. Testing and validation of these criteria are required. Objective To investigate the method agreement, interobserver reliability and applicability of the ISTH proposed definitions for hemostatic effectiveness. Methods Patient data from three anticoagulant-antidote studies were used for hemostatic effectiveness assessment using the ISTH-proposed definitions and clinical opinion. For every patient a case document was produced. For each cohort, four adjudicators were asked to assess the hemostatic effectiveness independently on a case-by-case basis. Agreement between the two methods of hemostatic effectiveness assessment was calculated using Cohen's kappa (κ), with a calculated sample size of at least 73 cases. Results The full dataset consisted of 116 cases, resulting in 464 assessments. Method agreement in outcome was observed in 364 of 464 assessments (78.5%), resulting in κ of 0.634 (95% CI: 0.575-0.694), or "substantial agreement." Interobserver reliability analysis of the proposed definitions computed an overall agreement of 54.2% with κ of 0.312 ("fair agreement"). Discussion Method agreement analysis shows that the conclusions drawn using the ISTH definitions have "substantial agreement" with clinical opinion. Interobserver reliability analysis demonstrated acceptable agreement. In-depth analysis provided minor opportunities for further improvement and correct application of the definition. The definition is recommended to be used in all future studies evaluating hemostatic effectiveness, taking the suggested recommendations into account.

Today smartphones are inseparable parts of modern life and are capable of performing many desktop computers' tasks such as scientific analysis with greater convenience. Here, we present OligoCOOL, which is an Android application for analyzing nucleic sequences. This application enables users to perform several common biomedical analyses for a given nucleotide sequence. OligoCOOL is a freely accessible Android app at http://bioinf.modares.ac.ir/software/OligoCOOL, which can be a suitable tool for the experimental design in the laboratories. This application also can be used to learn the basics of nucleotide sequence analysis. © 2019 International Union of Biochemistry and Molecular Biology, 47(2): 201-206, 2019.

Background: Andexanet alfa (andexanet) and prothrombin complex concentrate (PCC) are both reversal agents for major bleeding in patients using factor Xa inhibitors (FXaIs). Our aim was to evaluate the current evidence for the effectiveness and safety of andexanet and PCC in a systematic review and meta-analysis.Objectives: Primary objective was hemostatic effectiveness. Secondary objectives were thromboembolic event rate and mortality.Methods: A systematic review was performed in PubMed and Embase. Studies describing the effectiveness and/or safety of PCC or andexanet in patients with major bleeding using FXaIs were included. Meta-analysis was performed using a random-effects model.Results: Seventeen PCC studies, 3 andexanet studies, and 1 study describing PCC and andexanet were included, comprising 1428 PCC-treated and 396 and exanet-treated patients. None of the included studies had control groups, hampering a pooled meta-analysis to compare the two reversal agents. Separate analyses for andexanet and PCC were performed. In subgroup analysis, the pooled proportion of patients with effective hemostasis in studies that used Annexa-4 criteria demonstrated a hemostatic effectiveness of 0.85 (95% confidence interval [CI], 0.80-0.90) in PCC and 0.82 (95% CI, 0.78-0.87) in andexanet studies. The pooled proportion of patients with thromboembolic events was 0.03 (95% CI, 0.02-0.04) in PCC and 0.11 (95% CI, 0.04-0.18) in andexanet studies.Conclusion: Based on the available evidence with low certainty from observational studies, PCC and andexanet demonstrated a similar, effective hemostasis in the treatment of major bleeding in patients using FXaIs. Compared to PCC, the thromboembolic event rate appeared higher in and exanet-treated patients.

The effect of injected gas composition (as minimum miscible enrichment) on minimum miscibility pressure (MMP) and respective oil recovery has been studied through numerical slim tube simulation on a wide range of gas compositions. An initial comparative study on the performances of representative equations of state for predicting MMP in slim tube simulations has proven Peng–Robinson equation of state to provide results with better agreement with experimental data. Different injection scenarios are then designed to understand the effects of varying percentages of each component (like CO2) into the injected gas mixture on MMP. MMP decreased when the concentration of CO2 and intermediate components (C2–C6) increased. It was also found that when C7+ content in the injected gas increased, recovery factor decreased significantly. Further investigations on the effect of adding benzene, toluene, and xylene as chemicals into the injected gas (in slim tube simulations) also prove that addition of these aromatics can obviously improve oil recovery and decrease MMP and toluene gives the better results in comparison with the other ones. © 2017 Curtin University of Technology and John Wiley & Sons, Ltd.