Low Penetrance, Broad Resistance, and Favorable Outcome of Interleukin 12 Receptor β1 Deficiency: Medical and Immunological Implications

The clinical phenotype of interleukin 12 receptor β1 chain (IL-12Rβ1) deficiency and the function of human IL-12 in host defense remain largely unknown, due to the small number of patients reported. We now report 41 patients with complete IL-12Rβ1 deficiency from 17 countries. The only opportunistic infections observed, in 34 patients, were of childhood onset and caused by weakly virulent Salmonella or Mycobacteria (Bacille Calmette-Guérin -BCG- and environmental Mycobacteria). Three patients had clinical tuberculosis, one of whom also had salmonellosis. Unlike salmonellosis, mycobacterial infections did not recur. BCG inoculation and BCG disease were both effective against subsequent environmental mycobacteriosis, but not against salmonellosis. Excluding the probands, seven of the 12 affected siblings have remained free of case-definition opportunistic infection. Finally, only five deaths occurred in childhood, and the remaining 36 patients are alive and well. Thus, a diagnosis of IL-12Rβ1 deficiency should be considered in children with opportunistic mycobacteriosis or salmonellosis; healthy siblings of probands and selected cases of tuberculosis should also be investigated. The overall prognosis is good due to broad resistance to infection and the low penetrance and favorable outcome of infections. Unexpectedly, human IL-12 is redundant in protective immunity against most microorganisms other than Mycobacteria and Salmonella. Moreover, IL-12 is redundant for primary immunity to Mycobacteria and Salmonella in many individuals and for secondary immunity to Mycobacteria but not to Salmonella in most

A Hybrid Fuzzy Multi-criteria Decision Making Model to Evaluate the Overall Performance of Public Emergency Departments: A Case Study

[EN] Performance evaluation is relevant for supporting managerial decisions related to the improvement of public emergency departments (EDs). As different criteria from ED context and several alternatives need to be considered, selecting a suitable Multicriteria Decision-Making (MCDM) approach has become a crucial step for ED performance evaluation. Although some methodologies have been proposed to address this challenge, a more complete approach is still lacking. This paper bridges this gap by integrating three potent MCDM methods. First, the Fuzzy Analytic Hierarchy Process (FAHP) is used to determine the criteria and sub-criteria weights under uncertainty, followed by the interdependence evaluation via fuzzy Decision-Making Trial and Evaluation Laboratory(FDEMATEL). The fuzzy logic is merged with AHP and DEMATEL to illustrate vague judgments. Finally, the Technique for Order of Preference by Similarity to Ideal Solution (TOPSIS) is used for ranking EDs. This approach is validated in a real 3-ED cluster. The results revealed the critical role of Infrastructure (21.5%) in ED performance and the interactive nature of Patient safety (C+R =12.771). Furthermore, this paper evidences the weaknesses to be tackled for upgrading the performance of each ED.Ortiz-Barrios, M.; Alfaro Saiz, JJ. (2020). A Hybrid Fuzzy Multi-criteria Decision Making Model to Evaluate the Overall Performance of Public Emergency Departments: A Case Study. International Journal of Information Technology & Decision Making. 19(6):1485-1548. https://doi.org/10.1142/S0219622020500364S14851548196Lord, K., Parwani, V., Ulrich, A., Finn, E. B., Rothenberg, C., Emerson, B., … Venkatesh, A. K. (2018). Emergency department boarding and adverse hospitalization outcomes among patients admitted to a general medical service. The American Journal of Emergency Medicine, 36(7), 1246-1248. doi:10.1016/j.ajem.2018.03.043Sørup, C. M., Jacobsen, P., & Forberg, J. L. (2013). 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(2018). A Fuzzy AHP–TOPSIS-Based Group Decision-Making Approach to IT Personnel Selection. International Journal of Fuzzy Systems, 20(5), 1576-1591. doi:10.1007/s40815-018-0474-7CHEN, M.-F., TZENG, G.-H., & TANG, T.-I. (2005). FUZZY MCDM APPROACH FOR EVALUATION OF EXPATRIATE ASSIGNMENTS. International Journal of Information Technology & Decision Making, 04(02), 277-296. doi:10.1142/s0219622005001520Gul, M., Celik, E., Gumus, A. T., & Guneri, A. F. (2016). Emergency department performance evaluation by an integrated simulation and interval type-2 fuzzy MCDM-based scenario analysis. European J. of Industrial Engineering, 10(2), 196. doi:10.1504/ejie.2016.075846Jovčić, Průša, Dobrodolac, & Švadlenka. (2019). A Proposal for a Decision-Making Tool in Third-Party Logistics (3PL) Provider Selection Based on Multi-Criteria Analysis and the Fuzzy Approach. Sustainability, 11(15), 4236. doi:10.3390/su11154236Saaty, T. L., & Vargas, L. G. (2012). 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A new multi-criteria decision making approach for sustainable material selection problem: A critical study on rank reversal problem. Journal of Cleaner Production, 182, 466-484. doi:10.1016/j.jclepro.2018.02.062Chen, Z., Ming, X., Zhang, X., Yin, D., & Sun, Z. (2019). A rough-fuzzy DEMATEL-ANP method for evaluating sustainable value requirement of product service system. Journal of Cleaner Production, 228, 485-508. doi:10.1016/j.jclepro.2019.04.145Jumaah, F. M., Zadain, A. A., Zaidan, B. B., Hamzah, A. K., & Bahbibi, R. (2018). Decision-making solution based multi-measurement design parameter for optimization of GPS receiver tracking channels in static and dynamic real-time positioning multipath environment. Measurement, 118, 83-95. doi:10.1016/j.measurement.2018.01.011Singh, A., & Prasher, A. (2017). Measuring healthcare service quality from patients’ perspective: using Fuzzy AHP application. Total Quality Management & Business Excellence, 30(3-4), 284-300. doi:10.1080/14783363.2017.1302794Otay, İ., Oztaysi, B., Cevik Onar, S., & Kahraman, C. (2017). Multi-expert performance evaluation of healthcare institutions using an integrated intuitionistic fuzzy AHP&DEA methodology. Knowledge-Based Systems, 133, 90-106. doi:10.1016/j.knosys.2017.06.028Awasthi, A., Govindan, K., & Gold, S. (2018). Multi-tier sustainable global supplier selection using a fuzzy AHP-VIKOR based approach. International Journal of Production Economics, 195, 106-117. doi:10.1016/j.ijpe.2017.10.013Gul, M., Guneri, A. F., & Nasirli, S. M. (2018). A fuzzy-based model for risk assessment of routes in oil transportation. International Journal of Environmental Science and Technology, 16(8), 4671-4686. doi:10.1007/s13762-018-2078-zKazancoglu, Y., Kazancoglu, I., & Sagnak, M. (2018). Fuzzy DEMATEL-based green supply chain management performance. 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Multi-criteria decision analysis for supporting the selection of medical devices under uncertainty. European Journal of Operational Research, 247(1), 216-228. doi:10.1016/j.ejor.2015.05.075Kovacs, E., Strobl, R., Phillips, A., Stephan, A.-J., Müller, M., Gensichen, J., & Grill, E. (2018). Systematic Review and Meta-analysis of the Effectiveness of Implementation Strategies for Non-communicable Disease Guidelines in Primary Health Care. Journal of General Internal Medicine, 33(7), 1142-1154. doi:10.1007/s11606-018-4435-5Morley, C., Unwin, M., Peterson, G. M., Stankovich, J., & Kinsman, L. (2018). Emergency department crowding: A systematic review of causes, consequences and solutions. PLOS ONE, 13(8), e0203316. doi:10.1371/journal.pone.0203316Hermann, R. M., Long, E., & Trotta, R. L. (2019). Improving Patients’ Experiences Communicating With Nurses and Providers in the Emergency Department. Journal of Emergency Nursing, 45(5), 523-530. doi:10.1016/j.jen.2018.12.001Hawley, K. L., Mazer-Amirshahi, M., Zocchi, M. S., Fox, E. R., & Pines, J. M. (2015). Longitudinal Trends in U.S. Drug Shortages for Medications Used in Emergency Departments (2001-2014). Academic Emergency Medicine, 23(1), 63-69. doi:10.1111/acem.12838Stang, A. S., Crotts, J., Johnson, D. W., Hartling, L., & Guttmann, A. (2015). Crowding Measures Associated With the Quality of Emergency Department Care: A Systematic Review. Academic Emergency Medicine, 22(6), 643-656. doi:10.1111/acem.12682Chanamool, N., & Naenna, T. (2016). Fuzzy FMEA application to improve decision-making process in an emergency department. Applied Soft Computing, 43, 441-453. doi:10.1016/j.asoc.2016.01.007Farup, P. G. (2015). Are measurements of patient safety culture and adverse events valid and reliable? Results from a cross sectional study. BMC Health Services Research, 15(1). doi:10.1186/s12913-015-0852-xCarter, E. J., Pouch, S. M., & Larson, E. L. (2013). The Relationship Between Emergency Department Crowding and Patient Outcomes: A Systematic Review. Journal of Nursing Scholarship, 46(2), 106-115. doi:10.1111/jnu.12055Ebben, R. H. A., Siqeca, F., Madsen, U. R., Vloet, L. C. M., & van Achterberg, T. (2018). Effectiveness of implementation strategies for the improvement of guideline and protocol adherence in emergency care: a systematic review. BMJ Open, 8(11), e017572. doi:10.1136/bmjopen-2017-017572Innes, G. D., Sivilotti, M. L. A., Ovens, H., McLelland, K., Dukelow, A., Kwok, E., … Chochinov, A. (2018). Emergency overcrowding and access block: A smaller problem than we think. CJEM, 21(2), 177-185. doi:10.1017/cem.2018.446Di Somma, S., Paladino, L., Vaughan, L., Lalle, I., Magrini, L., & Magnanti, M. (2014). Overcrowding in emergency department: an international issue. Internal and Emergency Medicine, 10(2), 171-175. doi:10.1007/s11739-014-1154-8Uthman, O. A., Walker, C., Lahiri, S., Jenkinson, D., Adekanmbi, V., Robertson, W., & Clarke, A. (2018). General practitioners providing non-urgent care in emergency department: a natural experiment. BMJ Open, 8(5), e019736. doi:10.1136/bmjopen-2017-019736Razzak, J. A., Baqir, S. M., Khan, U. R., Heller, D., Bhatti, J., & Hyder, A. A. (2013). Emergency and trauma care in Pakistan: a cross-sectional study of healthcare levels. Emergency Medicine Journal, 32(3), 207-213. doi:10.1136/emermed-2013-202590Dart, R. C., Goldfrank, L. R., Erstad, B. L., Huang, D. T., Todd, K. H., Weitz, J., … Anderson, V. E. (2018). Expert Consensus Guidelines for Stocking of Antidotes in Hospitals That Provide Emergency Care. Annals of Emergency Medicine, 71(3), 314-325.e1. doi:10.1016/j.annemergmed.2017.05.021Mkoka, D. A., Goicolea, I., Kiwara, A., Mwangu, M., & Hurtig, A.-K. (2014). Availability of drugs and medical supplies for emergency obstetric care: experience of health facility managers in a rural District of Tanzania. BMC Pregnancy and Childbirth, 14(1). doi:10.1186/1471-2393-14-108Beck, M. J., Okerblom, D., Kumar, A., Bandyopadhyay, S., & Scalzi, L. V. (2016). Lean intervention improves patient discharge times, improves emergency department throughput and reduces congestion. Hospital Practice, 44(5), 252-259. doi:10.1080/21548331.2016.1254559Morais Oliveira, M., Marti, C., Ramlawi, M., Sarasin, F. P., Grosgurin, O., Poletti, P.-A., … Rutschmann, O. T. (2018). Impact of a patient-flow physician coordinator on waiting times and length of stay in an emergency department: A before-after cohort study. PLOS ONE, 13(12), e0209035. doi:10.1371/journal.pone.0209035Vermeulen, M. J., Stukel, T. A., Boozary, A. S., Guttmann, A., & Schull, M. J. (2016). The Effect of Pay for Performance in the Emergency Department on Patient Waiting Times and Quality of Care in Ontario, Canada: A Difference-in-Differences Analysis. Annals of Emergency Medicine, 67(4), 496-505.e7. doi:10.1016/j.annemergmed.2015.06.028Singh, S., Lin, Y.-L., Nattinger, A. B., Kuo, Y.-F., & Goodwin, J. S. (2015). Variation in readmission rates by emergency departments and emergency department providers caring for patients after discharge. Journal of Hospital Medicine, 10(11), 705-710. doi:10.1002/jhm.2407Källberg, A.-S., Göransson, K. E., Florin, J., Östergren, J., Brixey, J. J., & Ehrenberg, A. (2015). Contributing factors to errors in Swedish emergency departments. International Emergency Nursing, 23(2), 156-161. doi:10.1016/j.ienj.2014.10.002Riga, M., Vozikis, A., Pollalis, Y., & Souliotis, K. (2015). MERIS (Medical Error Reporting Information System) as an innovative patient safety intervention: A health policy perspective. 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A., Yang, J., Altieri, M., Patterson, W., Peoples, B., Chen, H., … Pryor, A. D. (2016). Rates and Risk Factors for Unplanned Emergency Department Utilization and Hospital Readmission Following Bariatric Surgery. Annals of Surgery, 263(5), 956-960. doi:10.1097/sla.0000000000001536Rigobello, M. C. G., Carvalho, R. E. F. L. de, Guerreiro, J. M., Motta, A. P. G., Atila, E., & Gimenes, F. R. E. (2017). The perception of the patient safety climate by professionals of the emergency department. International Emergency Nursing, 33, 1-6. doi:10.1016/j.ienj.2017.03.003Farmer, B. (2016). Patient Safety in the Emergency Department. Emergency Medicine, 48(9), 396-404. doi:10.12788/emed.2016.0052Liu, H.-C., You, J.-X., Zhen, L., & Fan, X.-J. (2014). A novel hybrid multiple criteria decision making model for material selection with target-based criteria. Materials & Design, 60, 380-390. doi:10.1016/j.matdes.2014.03.071Kou, G., Ergu, D., & Shang, J. (2014). Enhancing data consistency in decision matrix: Adapting Hadamard model to mitigate judgment contradiction. European Journal of Operational Research, 236(1), 261-271. doi:10.1016/j.ejor.2013.11.035Keshavarz Ghorabaee, M., Amiri, M., Zavadskas, E. K., & Antucheviciene, J. (2017). Supplier evaluation and selection in fuzzy environments: a review of MADM approaches. Economic Research-Ekonomska Istraživanja, 30(1), 1073-1118. doi:10.1080/1331677x.2017.1314828Barrios, M. A. O., De Felice, F., Negrete, K. P., Romero, B. A., Arenas, A. Y., & Petrillo, A. (2016). An AHP-Topsis Integrated Model for Selecting the Most Appropriate Tomography Equipment. International Journal of Information Technology & Decision Making, 15(04), 861-885. doi:10.1142/s021962201640006xYeh, D.-Y., & Cheng, C.-H. (2016). Performance Management of Taiwan’s National Hospitals. International Journal of Information Technology & Decision Making, 15(01), 187-213. doi:10.1142/s0219622014500199Chen, T.-Y. (2014). An Interactive Signed Distance Approach for Multiple Criteria Group Decision-Making Based on Simple Additive Weighting Method with Incomplete Preference Information Defined by Interval Type-2 Fuzzy Sets. International Journal of Information Technology & Decision Making, 13(05), 979-1012. doi:10.1142/s0219622014500229Gou, X., Xu, Z., & Liao, H. (2019). Hesitant Fuzzy Linguistic Possibility Degree-Based Linear Assignment Method for Multiple Criteria Decision-Making. International Journal of Information Technology & Decision Making, 18(01), 35-63. doi:10.1142/s0219622017500377Saksrisathaporn, K., Bouras, A., Reeveerakul, N., & Charles, A. (2016). Application of a Decision Model by Using an Integration of AHP and TOPSIS Approaches within Humanitarian Operation Life Cycle. International Journal of Information Technology & Decision Making, 15(04), 887-918. doi:10.1142/s0219622015500261Hsiao, B., & Chen, L.-H. (2019). 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Influences of using bovine somatotropin on estimates of genetic parameters and genetic evaluation of dairy cows

Records of 142,937 of registered and grade Holstein cows for yield traits and somatic cell scores (SCS) some with and some without bovine somatotropin (bST) treatment provided by the Dairy Records Processing Center at Raleigh, NC. were used to assess potential impact of bST on estimation of genetic parameters and on genetic evaluations when not all cows receive bST treatment. To investigate the relationship between genotype and response to bST, for yield traits and SCS, REML with multiple trait animal models, which considered yields with bST and without bST as different traits, were used. Animal models with and without adjustment for bST were also used for prediction of breeding values, and to study the effects of bST on genetic response to selection and on ranking of superior cows. Genetic parameters for first lactation yields were estimated with different two-trait animal models. Significant effects of bST treatment on yield traits were found, with responses to bST greater for registered than for grade cows. Management practices for herds with grade cows could be the main reason for this difference. Estimates of heritability for both registered and grade cows with bST treatment were 0.22, 0.19, 0.18 and 0.23 and repeatability estimates were 0.44, 0.45, 0.43, and 0.41 for milk, fat and protein yields and SCS, respectively. Estimates of heritability for cows without bST treatment were 0.17, 0.19, 0.18, and 0.21, and repeatability estimates were 0.39, 0.40, 0.39 and 0.36 for milk, fat, and protein yields and SCS, respectively. Strong favorable genetic and permanent environmental correlations were found between the traits with and without bST treatment. Correlations among breeding values for all traits predicted by different models were greater than 0.968. A large proportion of top 2% of cows were chosen when ranked by genetic evaluations from all models. The decline in genetic progress for yield traits due to ignoring the use of bST would be negligible. Estimation of genetic parameters of first lactation yields obtained using models that adjust for or do not adjust for bST treatment were similar

Activity of Some Disinfectants, Detergents and Essential Oils on Growth of the yeast Candida albicans

The study was conducted for estimating antifungal activity against Candida albicans of ten essential oil, garlic and onion juice, eight disinfectants and detergents by using agar diffusion well method. The results showed high activity of clove oil, onion juice 50%, thyme oil, hydrogen peroxide (H2O2), lugol's iodine and detol for all the five concentration, sodium chloride (NaCl) and sodium carbonate (Na2CO3) at 5%, while the effect of apple cider vinegar were at 60% and 80%. Sodium hypochlorite showed moderate activity at all concentration. The result of combination between clove oil and coconut oil led to synergistic effect while the combination between each of (1, 2, 3 % H2O2 with each of apple cider vinegar and NaCl), (sodium chloride with apple cider vinegar) and (pumpkin oil with clove oil) lead to antagonism as well as the same results were reported when apple cider vinegar mixed with each of (thyme oil) and (clove oil with coconut oil). On the other hand, there is no synergistic or antagonistic effect of combination between 4-5% of H2O2 with apple cider vinegar and NaCl solution to the growth of C. albicans. It was concluded that solitary use of compounds (clove oil, onion juice, thyme oil, H2O2, NaCl, lugol's iodine, detol, and apple cider vinegar) was associated with high antifungal response regarding C. albicans; efficacy was reduced when used in combination. In exception to above finding synergistic effect was identified when a combination between clove oil and coconut oil

113 Invasive fungal infections among pediatric patients with hematologic malignancies at KFSH&RC/KFCCC&R

Objectives: To define the magnitude of the problem, study factors associated with increased risk of invasive fungal, infections (IFI) and outcome.Methods: From June 1998 to March 2003, all, radiological, studies of patients with hematologic/ oncologic disorders were evaluated for inclusion. AII, cases of invasive fungal, infection were reviewed. The criteria for inclusion were obvious lesion suggestive of fungal, infection shown on radiological, studies, and fungal, infections were classified as proven , probable\u27, possible or insufficient evidence according to a prior definitions.Results: A total, of 1615 patient charts were reviewed. The underlying diagnoses include ALL 410, SCT 293, AML 133, non-malignant hematology 288, NHL/solid tumors 491. 152 (9%) had evidence of fungal, infection (55 [36%] \u27definite = proven/probable\u27, 97 [64%] \u27possible\u27). Biopsy was performed in 94 cases and the findings included budding yeast in 10 patients, septated hyphae in 19, and hyphae with no specifications in 12 patients. Delays in performing diagnostic procedures possibly resulted in the lower incidence of \u27definite\u27 IFI (36% vs 64% \u27possible ). The overall, incidence of fungal, infection was 9%, being highest for AML (39%), followed by ALL (17%). The majority of IFI developed during or immediately after induction (42% of IFI in AML and 53% of IFI in ALL), which can be a target for intervention. The infections included disseminated fungal, infection (36%), CDC (11%), pulmonary fungal, infection (43%) and aspergillosis (9.5%) including pulmonary, Para nasal, sinuses, skin and disseminated. IFI was radiologically diagnosed during neutropenia in 123 patients (81%). Ten patients died due to fungal, infection (7%), 75 (49%) were cured, 26 (17%) were alive with fungal, infection, and 39 patients (26%) died due to primary disease seemingly unrelated to fungal, infection. Mortality due to IFI in this study is less than what is reported in the literature and could be a result of our practice of early intervention. The average LOS for IFI was 56 days compared with the usual. 12 days, which can add to the increased cost.Conclusions: Invasive fungal, infection is becoming a serious problem. Furthermore, acute invasive fungal, infection is associated with a much higher mortality. Early diagnosis with prompt antifungal, therapy, or even with surgical, intervention, might be warranted to save patients\u27 lives