122 results on '"Garcez, T."'
Search Results
2. Consensus clinical scoring for suspected perioperative immediate hypersensitivity reactions
- Author
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Hopkins, P.M., Cooke, P.J., Clarke, R.C., Guttormsen, A.B., Platt, P.R., Dewachter, P., Ebo, D.G., Garcez, T., Garvey, L.H., Hepner, D.L., Khan, D.A., Kolawole, H., Kopac, P., Krøigaard, M., Laguna, J.J., Marshall, S.D., Mertes, P.M., Rose, M.A., Sabato, V., Savic, L.C., Savic, S., Takazawa, T., Volcheck, G.W., Voltolini, S., and Sadleir, P.H.M.
- Published
- 2019
- Full Text
- View/download PDF
3. Management of a surgical patient with a label of penicillin allergy: narrative review and consensus recommendations
- Author
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Savic, L.C., Khan, D.A., Kopac, P., Clarke, R.C., Cooke, P.J., Dewachter, P., Ebo, D.G., Garcez, T., Garvey, L.H., Guttormsen, A.B., Hopkins, P.M., Hepner, D.L., Kolawole, H., Krøigaard, M., Laguna, J.J., Marshall, S.D., Mertes, P.M., Platt, P.R., Rose, M.A., Sabato, V., Sadleir, P.H.M., Savic, S., Takazawa, T., Voltolini, S., and Volcheck, G.W.
- Published
- 2019
- Full Text
- View/download PDF
4. Anaesthesia, surgery, and life-threatening allergic reactions: epidemiology and clinical features of perioperative anaphylaxis in the 6th National Audit Project (NAP6)
- Author
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Harper, N.J.N., Cook, T.M., Garcez, T., Farmer, L., Floss, K., Marinho, S., Torevell, H., Warner, A., Ferguson, K., Hitchman, J., Egner, W., Kemp, H., Thomas, M., Lucas, D.N., Nasser, S., Karanam, S., Kong, K.-L., Farooque, S., Bellamy, M., and McGuire, N.
- Published
- 2018
- Full Text
- View/download PDF
5. Anaesthesia, surgery, and life-threatening allergic reactions: management and outcomes in the 6th National Audit Project (NAP6)
- Author
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Harper, N.J.N., Cook, T.M., Garcez, T., Lucas, D.N., Thomas, M., Kemp, H., Kong, K.-L., Marinho, S., Karanam, S., Ferguson, K., Hitchman, J., Torevell, H., Warner, A., Egner, W., Nasser, S., McGuire, N., Bellamy, M., Floss, K., Farmer, L., and Farooque, S.
- Published
- 2018
- Full Text
- View/download PDF
6. Cross-sectional study of perioperative drug and allergen exposure in UK practice in 2016: the 6th National Audit Project (NAP6) Allergen Survey
- Author
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Marinho, S., Kemp, H., Cook, T.M., Farmer, L., Farooque, S., Lucas, D.N., Garcez, T., Floss, K., Torevell, H., Thomas, M., Warner, A., Hitchman, J., Ferguson, K., Egner, W., Nasser, S., Karanam, S., Kong, K.-L., McGuire, N., Bellamy, M., and Harper, N.J.N.
- Published
- 2018
- Full Text
- View/download PDF
7. An observational national study of anaesthetic workload and seniority across the working week and weekend in the UK in 2016: the 6th National Audit Project (NAP6) Activity Survey
- Author
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Kemp, H., Marinho, S., Cook, T.M., Farmer, L., Bellamy, M., Egner, W., Farooque, S., Ferguson, K., Floss, K., Garcez, T., Karanam, S., Hitchman, J., Kong, K.-L., McGuire, N., Nasser, S., Lucas, D.N., Thomas, M., Torevell, H., Warner, A., and Harper, N.J.N.
- Published
- 2018
- Full Text
- View/download PDF
8. Anaesthesia, surgery, and life-threatening allergic reactions: protocol and methods of the 6th National Audit Project (NAP6) of the Royal College of Anaesthetists
- Author
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Cook, T.M., Harper, N.J.N., Farmer, L., Garcez, T., Floss, K., Marinho, S., Torevell, H., Warner, A., McGuire, N., Ferguson, K., Hitchman, J., Egner, W., Kemp, H., Thomas, M., Lucas, D.N., Nasser, S., Karanam, S., Kong, K.-L., Farooque, S., Bellamy, M., McGlennan, A., and Moonesinghe, S.R.
- Published
- 2018
- Full Text
- View/download PDF
9. British Society for Immunology and United Kingdom Primary Immunodeficiency Network (UKPIN) consensus guideline for the management of immunoglobulin replacement therapy
- Author
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Grigoriadou, S, primary, Clubbe, R, additional, Garcez, T, additional, Huissoon, A, additional, Grosse-Kreul, D, additional, Jolles, S, additional, Henderson, K, additional, Edmonds, J, additional, Lowe, D, additional, and Bethune, C, additional
- Published
- 2022
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10. Specialist perioperative allergy clinic services in the UK 2016: Results from the Royal College of Anaesthetists Sixth National Audit Project
- Author
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Egner, W., Cook, T., Harper, N., Garcez, T., Marinho, S., Kong, K. L., Nasser, S., Thomas, M., Warner, A., Hitchman, J., Floss, K., Farmer, Laura, Lourtie, Jose, and Goodwin, James
- Published
- 2017
- Full Text
- View/download PDF
11. Multidimensional risk evaluation: Information visualization to support a decision-making process in the context of natural gas pipeline
- Author
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Medeiros, C, primary, Alencar, M, additional, Garcez, T, additional, and de, A, additional
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- 2016
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12. Information visualization to support the decision-making process on risk assessment
- Author
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Garcez, T, primary, Alencar, M, additional, and de Almeida, A, additional
- Published
- 2015
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13. Global sensitivity analysis based on a Monte Carlo simulation: A study based on a natural gas system
- Author
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Medeiros, C, primary, Alencar, M, additional, Garcez, T, additional, and de Almeida, A, additional
- Published
- 2015
- Full Text
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14. Teicoplanin allergy – an emerging problem in the anaesthetic allergy clinic
- Author
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Savic, L. C., Garcez, T., Hopkins, P. M., Harper, N. J. N., and Savic, S.
- Published
- 2015
- Full Text
- View/download PDF
15. Long-term safety of icatibant treatment of patients with angioedema in real-world clinical practice
- Author
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Zanichelli, A., Maurer, M., Aberer, W., Caballero, T., Longhurst, H. J., Bouillet, L., Fabien, V., Andresen, I., Grumach, A., Bygum, A., Blanchard Delaunay, C., Boccon-Gibod, I., Coppere, B., Du Thanh, A., Dzviga, C., Fain, O., Goichot, B., Gompel, A., Guez, S., Jeandel, P., Kanny, G., Launay, D., Maillard, H., Martin, L., Masseau, A., Ollivier, Y., Sobel, A., Aygören-Pürsün, E., Bas, M., Bauer, A., Bork, K., Greve, J., Magerl, M., Martinez Saguer, I., Strassen, U., Papadopoulou-Alataki, E., Psarros, F., Graif, Y., Kivity, S., Reshef, A., Toubi, E., Arcoleo, F., Bova, M., Cicardi, M., Manconi, P., Marone, G., Montinaro, V., Triggiani, M., Baeza, L., Cabañas, R., Gala Ortiz, G., Guilarte, M., Hernandez, D., Hernando de Larramendi, C., Lleonart, R., Lobera, T., Marques, L., Saenz de San Pedro, B., Björkander, J., Bethune, C., Garcez, T., Zanichelli, A., Maurer, M., Aberer, W., Caballero, T., Longhurst, H. J., Bouillet, L., Fabien, V., Andresen, I., Grumach, A., Bygum, A., Blanchard Delaunay, C., Boccon-Gibod, I., Coppere, B., Du Thanh, A., Dzviga, C., Fain, O., Goichot, B., Gompel, A., Guez, S., Jeandel, P., Kanny, G., Launay, D., Maillard, H., Martin, L., Masseau, A., Ollivier, Y., Sobel, A., Aygören-Pürsün, E., Bas, M., Bauer, A., Bork, K., Greve, J., Magerl, M., Martinez Saguer, I., Strassen, U., Papadopoulou-Alataki, E., Psarros, F., Graif, Y., Kivity, S., Reshef, A., Toubi, E., Arcoleo, F., Bova, M., Cicardi, M., Manconi, P., Marone, G., Montinaro, V., Triggiani, M., Baeza, L., Cabañas, R., Gala Ortiz, G., Guilarte, M., Hernandez, D., Hernando de Larramendi, C., Lleonart, R., Lobera, T., Marques, L., Saenz de San Pedro, B., Björkander, J., Bethune, C., and Garcez, T.
- Subjects
safety ,0301 basic medicine ,real-world ,medicine.medical_specialty ,Immunology ,Brief Communication ,Off-label use ,03 medical and health sciences ,chemistry.chemical_compound ,0302 clinical medicine ,Icatibant ,Internal medicine ,angioedema ,icatibant ,Immunology and Allergy ,Medicine ,Reflux esophagitis ,Adverse effect ,Pregnancy ,Angioedema ,business.industry ,real‐world ,medicine.disease ,030104 developmental biology ,030228 respiratory system ,chemistry ,Anesthesia ,Observational study ,Long term safety ,medicine.symptom ,business - Abstract
The Icatibant Outcome Survey (IOS) is an observational study monitoring safety and effectiveness of icatibant in the real‐world setting. We analyzed safety data from 3025 icatibant‐treated attacks in 557 patients (enrolled between July 2009 and February 2015). Icatibant was generally well tolerated. Excluding off‐label use and pregnancy, 438 patients (78.6%) did not report adverse events (AEs). The remaining 119 (21.4%) patients reported 341 AEs, primarily gastrointestinal disorders (19.6%). Of these, 43 AEs in 17 patients (3.1%) were related to icatibant. Serious AEs (SAEs) occurred infrequently. A total of 143 SAEs occurred in 59 (10.6%) patients; only three events (drug inefficacy, gastritis, and reflux esophagitis) in two patients were considered related to icatibant. Notably, no SAEs related to icatibant occurred in patients with cardiovascular disease, nor in those using icatibant at a frequency above label guidelines. Additionally, no major differences were noted in AEs occurring in on‐label vs off‐label icatibant users.
- Published
- 2017
16. Allergic reaction to mepivacaine in a child
- Author
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Sharma, V., Harper, N. J. N., Garcez, T., and Arkwright, P. D.
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- 2013
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17. An analysis of applying RCM methodology in a Brazilian thermal power plant
- Author
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Lins, P, primary, Garcez, T, additional, Alencar, M, additional, and de Almeida, A, additional
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- 2011
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18. Procedures for aggregating experts’ knowledge and group decision model approaches
- Author
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Garcez, T, primary, de Almeida-Filho, A, additional, and de Almeida, A, additional
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- 2011
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19. Multicriteria risk analysis application in a distribution gas pipeline system in Sergipe
- Author
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Alencar, M, primary, de Almeida, A, additional, de Almeida-Filho, A, additional, and Garcez, T, additional
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- 2009
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20. Anaphylaxis to levobupivacaine - probably not
- Author
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Harper, N. J. N. and Garcez, T.
- Published
- 2012
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21. Elderly versus younger patients with hereditary angioedema type I/II: patient characteristics and safety analysis from the Icatibant Outcome Survey
- Author
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Bygum A, Caballero T, Grumach A, Longhurst H, Bouillet L, Aberer W, Zanichelli A, Botha J, Andresen I, Maurer M, Delaunay C, Boccon-Gibod I, Coppere B, Du Thanh A, Dzviga C, Fain O, Goichot B, Gompel A, Guez S, Jeandel P, Kanny G, Launay D, Maillard H, Martin L, Masseau A, Ollivier Y, Sobel A, Aygoren-Pursun E, Bas M, Bauer M, Bork K, Greve J, Magerl M, Martinez-Saguer I, Strassen U, Papadopoulou-Alataki E, Psarros F, Graff Y, Kivity S, Reshef A, Toubi E, Arcoleo F, Bova M, Cicardi M, Nconi P, Marone G, Montinaro V, Triggiani M, Baeza M, Cabanas R, Guilarte M, Hernandez D, de Larramendi C, Lleonart R, Lobera T, Marques L, Pedro B, Bjorkander J, Bethune C, Garcez T, and IOS Study Grp
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Hereditary angioedema ,Elderly ,Icatibant Outcome Survey ,Safety - Abstract
Background: Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is characterized by recurrent swelling in subcutaneous or submucosal tissues. Symptoms often begin by age 5-11 years and worsen during puberty, but attacks can occur at any age and recur throughout life. Disease course in elderly patients is rarely reported. Methods: The Icatibant Outcome Survey (IOS) is an observational study evaluating the safety, tolerability, and efficacy of icatibant. We conducted descriptive analyses in younger (age < 65 years) versus elderly patients (age >= 65 years). Here, we report patient characteristics and safety-related findings. Results: As of February 2018, 872 patients with C1-INH-HAE type I/II were enrolled, of whom 100 (11.5%) were >= 65 years old. Significant differences between elderly versus younger patients, respectively, were noted for median age at symptom onset (17.0 vs 12.0 years), age at diagnosis (41.0 vs 19.4 years), and delay between symptom onset and diagnosis (23.9 vs 4.8 years) (P
- Published
- 2019
22. Consensus clinical scoring for suspected perioperative immediate hypersensitivity reactions
- Author
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Hopkins, P. M., Cooke, P. J., Clarke, R. C., Guttormsen, A. B., Platt, P. R., Dewachter, P., Ebo, D. G., Garcez, T., Garvey, L. H., Hepner, D. L., Khan, D. A., Kolawole, H., Kopac, P., Krøigaard, M., Laguna, J. J., Marshall, S. D., Mertes, P. M., Rose, M. A., Sabato, V., Savic, L. C., Savic, S., Takazawa, T., Volcheck, G. W., Voltolini, S., Sadleir, P. H.M., Hopkins, P. M., Cooke, P. J., Clarke, R. C., Guttormsen, A. B., Platt, P. R., Dewachter, P., Ebo, D. G., Garcez, T., Garvey, L. H., Hepner, D. L., Khan, D. A., Kolawole, H., Kopac, P., Krøigaard, M., Laguna, J. J., Marshall, S. D., Mertes, P. M., Rose, M. A., Sabato, V., Savic, L. C., Savic, S., Takazawa, T., Volcheck, G. W., Voltolini, S., and Sadleir, P. H.M.
- Abstract
Background: Grading schemes for severity of suspected allergic reactions have been applied to the perioperative setting, but there is no scoring system that estimates the likelihood that the reaction is an immediate hypersensitivity reaction. Such a score would be useful in evaluating current and proposed tests for the diagnosis of suspected perioperative immediate hypersensitivity reactions and culprit agents. Methods: We conducted a Delphi consensus process involving a panel of 25 international multidisciplinary experts in suspected perioperative allergy. Items were ranked according to appropriateness (on a scale of 1–9)and consensus, which informed development of a clinical scoring system. The scoring system was assessed by comparing scores generated for a series of clinical scenarios against ratings of panel members. Supplementary scores for mast cell tryptase were generated. Results: Two rounds of the Delphi process achieved stopping criteria for all statements. From an initial 60 statements, 43 were rated appropriate (median score 7 or more)and met agreement criteria (disagreement index <0.5); these were used in the clinical scoring system. The rating of clinical scenarios supported the validity of the scoring system. Although there was variability in the interpretation of changes in mast cell tryptase by the panel, we were able to include supplementary scores for mast cell tryptase. Conclusion: We used a robust consensus development process to devise a clinical scoring system for suspected perioperative immediate hypersensitivity reactions. This will enable objectivity and uniformity in the assessment of the sensitivity of diagnostic tests.
- Published
- 2019
23. Management of a surgical patient with a label of penicillin allergy:narrative review and consensus recommendations
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Savic, L. C., Khan, D. A., Kopac, P., Clarke, R. C., Cooke, P. J., Dewachter, P., Ebo, D. G., Garcez, T., Garvey, L. H., Guttormsen, A. B., Hopkins, P. M., Hepner, D. L., Kolawole, H., Krøigaard, M., Laguna, J. J., Marshall, S. D., Mertes, P. M., Platt, P. R., Rose, M. A., Sabato, V., Sadleir, P. H.M., Savic, S., Takazawa, T., Voltolini, S., Volcheck, G. W., Savic, L. C., Khan, D. A., Kopac, P., Clarke, R. C., Cooke, P. J., Dewachter, P., Ebo, D. G., Garcez, T., Garvey, L. H., Guttormsen, A. B., Hopkins, P. M., Hepner, D. L., Kolawole, H., Krøigaard, M., Laguna, J. J., Marshall, S. D., Mertes, P. M., Platt, P. R., Rose, M. A., Sabato, V., Sadleir, P. H.M., Savic, S., Takazawa, T., Voltolini, S., and Volcheck, G. W.
- Abstract
Unsubstantiated penicillin-allergy labels are common in surgical patients, and can lead to significant harm through avoidance of best first-line prophylaxis of surgical site infections and increased infection with resistant bacterial strains. Up to 98% of penicillin-allergy labels are incorrect when tested. Because of the scarcity of trained allergists in all healthcare systems, only a minority of surgical patients have the opportunity to undergo testing and de-labelling before surgery. Testing pathways can be modified and shortened in selected patients. A variety of healthcare professionals can, with appropriate training and in collaboration with allergists, provide testing for selected patients. We review how patients might be assessed, the appropriate testing strategies that can be used, and the minimum standards of safe testing.
- Published
- 2019
24. British Society for Immunology/United Kingdom Primary Immunodeficiency Network consensus statement on managing non-infectious complications of common variable immunodeficiency disorders
- Author
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Bethune, C, primary, Egner, W, additional, Garcez, T, additional, Huissoon, A, additional, Jolles, S, additional, Karim, Y, additional, Jain, R, additional, Savic, S, additional, Kelley, K, additional, Grosse-Kreul, D, additional, and Grigoriadou, S, additional
- Published
- 2019
- Full Text
- View/download PDF
25. Specialist Peri-Operative Allergy Clinic Services in the UK 2016: Results from the Royal College of Anaesthetists Sixth National Audit Project (NAP6)
- Author
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Egner, W., Cook, T., Harper, N., Garcez, T., Marinho, S., Kong, K.L., Nasser, S., Thomas, M., Warner, A., Hitchman, J., and Floss, K.
- Abstract
BACKGROUND: Guidelines for investigation of perioperative drug allergy exist, but the quality of services is unknown. Specialist perioperative anaphylaxis services were surveyed through the Royal College of Anaesthetists 6(th) National Audit Project. OBJECTIVES: We compare self-declared UK practice in specialist perioperative allergy services with national recommendations.\ud \ud METHODS: A SurveyMonkey(™) questionnaire was distributed to providers of allergy services in the UK. Responses were assessed for adherence to the best practice recommendations of the British Society for Allergy and Clinical Immunology, the Association of Anaesthetists of Great Britain and Ireland and the National Institute for Health and Care Excellence (NICE) Guidance on Drug Allergy - CG183. \ud \ud RESULTS: Over 1200 patients were evaluated in 44 centres annually. Variation in workload, waiting times, access, staffing and diagnostic approach was noted. Paediatric centres had the longest routine waiting times (most wait >13 weeks) in contrast to adult centres (most wait
- Published
- 2017
26. Chlorhexidine Allergy in 4 Specialist Allergy Centres in the UK, 2009-2013: Clinical Features and Diagnostic Tests
- Author
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Egner, W., Helbert, M., Sargur, R., Swallow, K., Harper, N., Garcez, T., Savic, S., Savic, L., and Effren, E.
- Abstract
We describe an observational survey of diagnostic pathways in 104 patients attending four specialist allergy clinics in the UK following perioperative hypersensitivity reactions to chlorhexidine reactions. The majority were life threatening. Men undergoing urological or cardiothoracic surgery predominated. Skin prick testing and sIgE testing were the most common tests used for diagnosis. Fifty-three % of diagnoses were made on the basis of a single positive test. Where multiple tests were performed the sensitivity of intradermal, basophil activation and skin prick testing was 68% (50-86%), 50% (10-90%) and 35% (17-55%) respectively. Seven percent were negative on screening tests initially, and 12 cases were only positive for a single test despite multiple testing. Intradermal tests appeared most sensitive in this context. Additional sensitisation to other substances used perioperatively, particularly neuromuscular blocking agents (NMBA), was found in 28 patients, emphasising the need to test for possible allergy to all drugs to which the patient was exposed even where chlorhexidine is positive. This article is protected by copyright. All rights reserved.
- Published
- 2017
27. Specialist perioperative allergy clinic services in the UK 2018: Results from the Royal College of Anaesthetists Sixth National Audit Project (NAP6) investigation of perioperative anaphylaxis
- Author
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Egner, W., primary, Cook, T. M., additional, Garcez, T., additional, Marinho, S., additional, Kemp, H., additional, Lucas, D. N., additional, Floss, K., additional, Farooque, S., additional, Torevell, H., additional, Thomas, M., additional, Ferguson, K., additional, Nasser, S., additional, Karanam, S., additional, Kong, K.-L., additional, McGuire, N., additional, Bellamy, M., additional, Warner, A., additional, Hitchman, J., additional, Farmer, L., additional, and Harper, N. J. N., additional
- Published
- 2018
- Full Text
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28. The United Kingdom Primary Immune Deficiency (UKPID) registry 2012 to 2017
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Shillitoe, B, primary, Bangs, C, additional, Guzman, D, additional, Gennery, A R, additional, Longhurst, H J, additional, Slatter, M, additional, Edgar, D M, additional, Thomas, M, additional, Worth, A, additional, Huissoon, A, additional, Arkwright, P D, additional, Jolles, S, additional, Bourne, H, additional, Alachkar, H, additional, Savic, S, additional, Kumararatne, D S, additional, Patel, S, additional, Baxendale, H, additional, Noorani, S, additional, Yong, P F K, additional, Waruiru, C, additional, Pavaladurai, V, additional, Kelleher, P, additional, Herriot, R, additional, Bernatonienne, J, additional, Bhole, M, additional, Steele, C, additional, Hayman, G, additional, Richter, A, additional, Gompels, M, additional, Chopra, C, additional, Garcez, T, additional, and Buckland, M, additional
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- 2018
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29. Chlorhexidine allergy in four specialist allergy centres in the United Kingdom, 2009–13: clinical features and diagnostic tests
- Author
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Egner, W, primary, Helbert, M, additional, Sargur, R, additional, Swallow, K, additional, Harper, N, additional, Garcez, T, additional, Savic, S, additional, Savic, L, additional, and Eren, E, additional
- Published
- 2017
- Full Text
- View/download PDF
30. Multidimensional risk assessment as a tool for decision analysis
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Garcez, T. V., primary
- Published
- 2015
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31. Production and Spad Reading in Marandu Palisadegrass Fertilized with Nitrogen and Magnesium
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Garcez, T. B., primary, Megda, M. M., additional, Artur, A. G., additional, and Monteiro, F. A., additional
- Published
- 2014
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32. Multidimensional Risk Assessment of Manhole Events as a Decision Tool for Ranking the Vaults of an Underground Electricity Distribution System
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Garcez, T. V., primary and de Almeida, A. T., additional
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- 2014
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33. Wiskott-Aldrich syndrome: acute retinal necrosis as a further late complication
- Author
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Garcez, T. P., primary, Johnston, S. L., additional, and Unsworth, D. J., additional
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- 2010
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34. Topical treatment of actinic keratosis with imiquimod 5% cream.
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Garcez, T. N. A., Gerardi, D. G., Ferreira, K. C., Cardoso, C. S., Möschbacher, P. D., and Contesini, E. A.
- Subjects
ACTINIC keratosis ,KERATOSIS ,IMMUNOMODULATORS ,ONCOLOGY research ,SKIN diseases - Abstract
The article discusses the effectiveness of imiquimod 5 percent cream in a cat suffering from nasal actinic keratosis. The case involves a 9-year-old semi-domiciled male cat without definite breed which was applied with Imiquimod 5 percent cream, which was performed by the owners once a week. It was found that the cream was safe and efficient, which makes it a new alternative to avoid progressions of this kind of neoplasia that is commonly seen in small animal clinics.
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- 2012
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35. Decision support system for prioritizing the multidimensional risk of natural gas pipelines: An application,Sistema de apoio a decisao para ranqueamento de risco multidimensional de gasodutos de gas natural: Uma aplicacao
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Garcez, T. V., Lopes, Y. G., Alencar, M. H., Cristiano Cavalcante, Almeida Filho, A. T., and Almeida, A. T.
36. Experts' elicitation of prior knowledge on accidental releases in a natural gas pipeline
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Garcez, T. V., Adiel T. de Almeida-Filho, Almeida, A. T., and Alencar, M. H.
37. Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations
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Tim Niehues, Catherine Waruiru, Conleth Feighery, Uwe Schauer, Virginie Courteille, Kai Lehmberg, Ingo Müller, I. Esteves, Henner Morbach, Michael Borte, Patrick Hundsdoerfer, Klaus Schwarz, Ewelina Gowin, Alessandro Aiuti, Andreas Holbro, Federica Barzaghi, João Farela Neves, Dagmar Graf, Hannah Tamary, Veneta Milenova, Benedikt Boetticher, Eleonora Gambineri, Vera Goda, Alia Eldash, Jan-Christian Wasmuth, Fabio Candotti, Svetlana O. Sharapova, Markus Metzler, Juergen Brunner, Anna Hilfanova, Brindusa Ruxandra Capilna, Pere Soler-Palacín, Arnau Antolí, Horst von Bernuth, Vassilios Lougaris, Maria Carrabba, Bernd H. Belohradsky, Julian Thalhammer, Nathalie de Vergnes, Peter Olbrich, Peter Kopač, Leif G. Hanitsch, Alexandra Nieters, Filomeen Haerynck, Juliana Gabzdilova, Sezin Aydemir, Rabab El Hawary, Patrick F.K. Yong, Maria Giovanna Danieli, Alberto Tommasini, Sandra Steinmann, Ulrich Baumann, Figen Dogu, Elisabeth Förster-Waldl, Carolina Marasco, Donato Amodio, Lorenzo Lodi, Xavier Solanich, Caterina Cancrini, Brigita Sitkauskiene, Torsten Witte, Clementina Vanessa, Nima Rezaei, Jean-Christophe Goffard, Kirsten Wittke, Emmanouil Liatsis, Helen Baxendale, Susana L. Silva, Bodo Grimbacher, Henrike Ritterbusch, Evangelia Farmaki, Safa Meshaal, Sujal Ghosh, Larysa Kostyuchenko, David Edgar, Simone Cesaro, R Zeuner, Nerea Salmón Rodríguez, Isabella Quinti, Stephan Ehl, Pauline Brosselin, Joerg C. Henes, Pilar Llobet Agulló, Rosa Maria Dellepiane, Andrea Meinhardt, Marina Kojić, Georgios Sogkas, Stephan Borte, Catharina Schuetz, Suheyla Ocak, Karin Marschall, Lukas M. Gasteiger, Stefan Raffac, Sofia Tantou, Sadia Noorani, Matthaios Speletas, Philippe Randrianomenjanahary, Ursula Holzer, Ayca Kiykim, Johannes G. Liese, Angelo Vacca, Gisela Fecker, Ekrem Unal, Koen J. van Aerde, Alba Parra-Martínez, Kaan Boztug, Sophie Stiehler, Sybille Landwehr-Kenzel, Claudio Pignata, Jennifer Neubert, Janine Reichenbach, Shahnaz Parvin, Sarah Goddard, Andrea Schroll, Dirk Holzinger, Asghar Aghamohammadi, Hassan Abolhassani, Johannes Trück, Estela Paz-Artal, Shereen M. Reda, Anna Shcherbina, Maria Raptaki, Jaroslava Orosova, Beata Wolska-Kuśnierz, Tessa Kerre, Gerrit Ahrenstorf, Ben Zion Garty, Dirk Foell, Benjamin Becker, Ulrike F. Demel, Androniki Kapousouzi, Abraham Rutgers, Klaus Warnatz, Gemma Rocamora Blanch, Stephan Rusch, Luis M. Allende, Dalia Abd Elaziz, Safa Baris, Jorisvan Montfrans, Dominik T. Schneider, Raphael Scheible, Juana Gil-Herrera, Gerhard Kindle, Annarosa Soresina, Giovanna Fabio, Uwe Wintergerst, Emilia Faria, Maria Fasshauer, Silvia Ricci, Aisha Elmarsafy, Barbara Pietrucha, Carsten Speckmann, Nizar Mahlaoui, Ulrich Heininger, Isabelle Meyts, Matthew Buckland, Efimia Papadopoulou-Alataki, Robin Kobbe, A Herwadkar, Sebastian F. N. Bode, Ali Sobh, László Maródi, Baldassarre Martire, Chiara Azzari, Maximilian Heeg, Katja Masjosthusmann, Michael H. Albert, Matteo Chinello, Juan Luis Santos-Pérez, Aarnoud Huissoon, Tanya I. Coulter, Hendrik Schulze-Koops, Norbert Graf, Radwa Alkady, Jolanta Bernatoniene, Seraina Prader, Alenka Gagro, Joachim Roesler, Taco W. Kuijpers, Ewa Więsik-Szewczyk, Maria Elena Maccari, Conrad Ferdinand Lippert, Miriam González-Amores, Johannes Dirks, Daniel E Pleguezuelo, Christof M. Kramm, Anders Fasth, Volker Schuster, Olov Ekwall, Nikolaus Rieber, Javier Carbone, Petra Kaiser-Labusch, Diana Ernst, Lucia Augusta Baselli, Luis Ignacio Gonzalez-Granado, Maria Kanariou, Stefanie S. V. Henriet, Sigune Goldacker, Kerstin Felgentreff, Oana Joean, Fine Roosens, Fabian Hauck, Eva C. Schwaneck, Milos Jesenak, Manfred Hoenig, Lenka Kapustova, Christoph Boesecke, Alain Fischer, Sara Pereira da Silva, Julia Körholz, Ansgar Schulz, Carolynne Schwarze-Zander, Mikko Seppänen, Nermeen Galal, Nora Naumann-Bartsch, Tomaz Garcez, Peter Ciznar, Klara M. Posfay-Barbe, Zelimir Pavle Eric, Reinhold E. Schmidt, Hermann J. Girschick, Sabine Heine, Anika-Kerstin Biegner, Annick A. J. M. van de Ven, Stefan Schreiber, J. Merlijn van den Berg, Nurit Assia Batzir, Alexandra Jablonka, Kim Stol, Gregor Dückers, Antonios G.A. Kolios, Ioannis Kakkas, Christian Klemann, Marina N. Guseva, Sofia Grigoriadou, Elif Karakoc-Aydiner, Antonio Marzollo, Peter D. Arkwright, Urs C. Steiner, Sara Sebnem Kilic, Romina Dieli-Crimi, Gergely Kriván, Monika Sparber-Sauer, Marco Cazzaniga, Fulvio Porta, Paraskevi Maggina, Tomas Milota, Robbert G. M. Bredius, Martine Pergent, Klaus Tenbrock, Jana Pachlopnik Schmid, Florentia Dimitriou, Cathal Laurence Steele, Helen Bourne, Anna Bobcakova, Gerd Horneff, Judith Potjewijd, Marc Schmalzing, Tobias Ankermann, Paul Ryan, Oksana Boyarchuk, Necil Kutukculer, Carl Friedrich Classen, Zita Chovancová, Moira Thomas, Cinzia Milito, Michaela Bitzenhofer-Grüber, Faranaz Atschekzei, Eva Hlaváčková, Viviana Moschese, Julie Smet, Hans-Hartmut Peter, Carla Teixeira, Sabine M El-Helou, Suzanne de Kruijf Bazen, Helmut Wittkowski, Donate Jakoby, Marina Garcia-Prat, Esther de Vries, Richard Herriot, Sven Kracker, Alessandro Plebani, Lisa Göschl, Laura Hora Marques, Anna Sediva, Jiri Litzman, Mark M. Gompels, Renate Krüger, Şefika İlknur Kökçü Karadağ, Nadine Binder, Anna Szaflarska, Peter Jandus, Lisa Ibberson, Johann Greil, Ulf Schulze-Sturm, Mehtap Sirin, Aydan Ikinciogullari, Edyta Heropolitańska-Pliszka, Michael E. Weiss, Alla Skapenko, Lukas Wisgrill, Hana Alachkar, Uta Behrends, Silvia Sánchez-Ramón, Maria N. Hatzistilianou, Otilia Petrovicova, Darko Richter, Zoreh Nademi, Jürgen K. Rockstroh, Sohilla Lotfy, Markus G. Seidel, Timothy Ronan Leahy, Audra Blažienė, Translational Immunology Groningen (TRIGR), Paediatric Infectious Diseases / Rheumatology / Immunology, AII - Inflammatory diseases, ARD - Amsterdam Reproduction and Development, University of Zurich, Ehl, Stephan, Thalhammer, J., Kindle, G., Nieters, A., Rusch, S., Seppanen, M. R. J., Fischer, A., Grimbacher, B., Edgar, D., Buckland, M., Mahlaoui, N., Ehl, S., Boztug, K., Brunner, J., Demel, U. F., Forster-Waldl, E., Gasteiger, L. M., Goschl, L., Kojic, M., Schroll, A., Seidel, M. G., Wintergerst, U., Wisgrill, L., Sharapova, S. O., Goffard, J. -C., Kerre, T., Meyts, I., Roosens, F., Smet, J., Haerynck, F., Eric, Z. P., Milenova, V., Gagro, A., Richter, D., Chovancova, Z., Hlavackova, E., Litzman, J., Milota, T., Sediva, A., Elaziz, D. A., Alkady, R. S., El Sayed El Hawary, R., Eldash, A. S., Galal, N., Lotfy, S., Meshaal, S. S., Reda, S. M., Sobh, A., Elmarsafy, A., Brosselin, P., Courteille, V., De Vergnes, N., Kracker, S., Pergent, M., Randrianomenjanahary, P., Ahrenstorf, G., Albert, M. H., Ankermann, T., Atschekzei, F., Baumann, U., Becker, B. C., Behrends, U., Belohradsky, B. H., Biegner, A. -K., Binder, N., Bode, S. F. N., Boesecke, C., Boetticher, B., Borte, M., Borte, S., Classen, C. F., Dirks, J., Duckers, G., El-Helou, S., Ernst, D., Fasshauer, M., Fecker, G., Felgentreff, K., Foell, D., Ghosh, S., Girschick, H. J., Goldacker, S., Graf, N., Graf, D., Greil, J., Hanitsch, L. G., Hauck, F., Heeg, M., Heine, S. I., Henes, J. C., Hoenig, M., Holzer, U., Holzinger, D., Horneff, G., Hundsdoerfer, P., Jablonka, A., Jakoby, D., Joean, O., Kaiser-Labusch, P., Klemann, C., Kobbe, R., Korholz, J., Kramm, C. M., Kruger, R., Landwehr-Kenzel, S., Lehmberg, K., Liese, J. G., Lippert, C. F., Maccari, M. E., Masjosthusmann, K., Meinhardt, A., Metzler, M., Morbach, H., Muller, I., Naumann-Bartsch, N., Neubert, J., Niehues, T., Peter, H. -H., Rieber, N., Ritterbusch, H., Rockstroh, J. K., Roesler, J., Schauer, U., Scheible, R., Schmalzing, M., Schmidt, R. E., Schneider, D. T., Schreiber, S., Schuetz, C., Schulz, A., Schulze-Koops, H., Schulze-Sturm, U., Schuster, V., Schwaneck, E. C., Schwarz, K., Schwarze-Zander, C., Sirin, M., Skapenko, A., Sogkas, G., Sparber-Sauer, M., Speckmann, C., Steinmann, S., Stiehler, S., Tenbrock, K., von Bernuth, H., Warnatz, K., Wasmuth, J. -C., Weiss, M., Witte, T., Wittke, K., Wittkowski, H., Zeuner, R. A., Farmaki, E., Hatzistilianou, M. N., Kakkas, I., Kanariou, M. G., Kapousouzi, A., Liatsis, E., Maggina, P., Papadopoulou-Alataki, E., Raptaki, M., Speletas, M., Tantou, S., Goda, V., Krivan, G., Marodi, L., Abolhassani, H., Aghamohammadi, A., Rezaei, N., Feighery, C., Leahy, T. R., Ryan, P., Batzir, N. A., Garty, B. Z., Tamary, H., Aiuti, A., Amodio, D., Azzari, C., Barzaghi, F., Baselli, L. A., Cancrini, C., Carrabba, M., Cazzaniga, M., Cesaro, S., Chinello, M., Danieli, M. G., Dellepiane, R. M., Fabio, G., Gambineri, E., Lodi, L., Lougaris, V., Marasco, C., Martire, B., Marzollo, A., Milito, C., Moschese, V., Pignata, C., Plebani, A., Porta, F., Quinti, I., Ricci, S., Soresina, A., Tommasini, A., Vacca, A., Vanessa, C., Blaziene, A., Sitkauskiene, B., Gowin, E., Heropolitanska-Pliszka, E., Pietrucha, B., Szaflarska, A., Wiesik-Szewczyk, E., Wolska-Kusnierz, B., Esteves, I., Faria, E., Marques, L. H., Neves, J. F., Silva, S. L., Teixeira, C., Pereira da Silva, S., Capilna, B. R., Guseva, M. N., Shcherbina, A., Bobcakova, A., Ciznar, P., Gabzdilova, J., Jesenak, M., Kapustova, L., Orosova, J., Petrovicova, O., Raffac, S., Kopac, P., Allende, L. M., Antoli, A., Blanch, G. R., Carbone, J., Dieli-Crimi, R., Garcia-Prat, M., Gil-Herrera, J., Gonzalez-Granado, L. I., Agullo, P. L., Olbrich, P., Parra-Martinez, A., Paz-Artal, E., Pleguezuelo, D. E., Rodriguez, N. S., Sanchez-Ramon, S., Santos-Perez, J. L., Solanich, X., Soler-Palacin, P., Gonzalez-Amores, M., Ekwall, O., Fasth, A., Bitzenhofer-Gruber, M., Candotti, F., Dimitriou, F., Heininger, U., Holbro, A., Jandus, P., Kolios, A. G. A., Marschall, K., Schmid, J. P., Posfay-Barbe, K. M., Prader, S., Reichenbach, J., Steiner, U. C., Truck, J., Bredius, R. G., de Kruijf- Bazen, S., de Vries, E., Henriet, S. S. V., Kuijpers, T. W., Potjewijd, J., Rutgers, A., Stol, K., van Aerde, K. J., Van den Berg, J. M., van de Ven, A. A. J. M., Montfrans, J., Aydemir, S., Baris, S., Dogu, F., Ikinciogullari, A., Karakoc-Aydiner, E., Kilic, S. S., Kiykim, A., Kokcu Karadag, S. I., Kutukculer, N., Ocak, S., Unal, E., Boyarchuk, O., Hilfanova, A., Kostyuchenko, L. V., Alachkar, H., Arkwright, P. D., Baxendale, H. E., Bernatoniene, J., Coulter, T. I., Garcez, T., Goddard, S., Gompels, M. M., Grigoriadou, S., Herriot, R., Herwadkar, A., Huissoon, A., Ibberson, L., Nademi, Z., Noorani, S., Parvin, S., Steele, C. L., Thomas, M., Waruiru, C., Yong, P. F. K., and Bourne, H.
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0301 basic medicine ,Male ,Pediatrics ,syndromic ,Sex Factor ,Disease ,registry ,medicine.disease_cause ,Cohort Studies ,0302 clinical medicine ,Primary Immunodeficiency Disease ,inborn error of immunity ,Immunology and Allergy ,warning signs ,Age Factor ,Registries ,Family history ,presenting symptom ,Child ,Primary immunodeficiency ,Granuloma ,autoimmune ,immune dysregulation ,inflammatory ,Adult ,Autoimmune Diseases ,Female ,Humans ,Infections ,Lymphoproliferative Disorders ,Middle Aged ,Primary Immunodeficiency Diseases ,Sex Factors ,Age Factors ,10177 Dermatology Clinic ,Infections/epidemiology ,3. Good health ,Settore MED/02 ,Warning signs ,Lymphoproliferative Disorder ,2723 Immunology and Allergy ,Infection ,Human ,medicine.medical_specialty ,Immunology ,610 Medicine & health ,Malignancy ,primary immunodeficiency ,Autoimmune Disease ,03 medical and health sciences ,Immunity ,Autoimmune Diseases/epidemiology ,medicine ,2403 Immunology ,business.industry ,warning sign ,Common variable immunodeficiency ,Granuloma/epidemiology ,Immune dysregulation ,medicine.disease ,Primary Immunodeficiency Diseases/epidemiology ,030104 developmental biology ,Lymphoproliferative Disorders/epidemiology ,Cohort Studie ,business ,030215 immunology - Abstract
BACKGROUND: Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a challenge. A better description of the initial presenting manifestations should improve awareness and avoid diagnostic delay. Although increased infection susceptibility is a well-known initial IEI manifestation, less is known about the frequency of other presenting manifestations.OBJECTIVE: We sought to analyze age-related initial presenting manifestations of IEI including different IEI disease cohorts.METHODS: We analyzed data on 16,486 patients of the European Society for Immunodeficiencies Registry. Patients with autoinflammatory diseases were excluded because of the limited number registered.RESULTS: Overall, 68% of patients initially presented with infections only, 9% with immune dysregulation only, and 9% with a combination of both. Syndromic features were the presenting feature in 12%, 4% had laboratory abnormalities only, 1.5% were diagnosed because of family history only, and 0.8% presented with malignancy. Two-third of patients with IEI presented before the age of 6 years, but a quarter of patients developed initial symptoms only as adults. Immune dysregulation was most frequently recognized as an initial IEI manifestation between age 6 and 25 years, with male predominance until age 10 years, shifting to female predominance after age 40 years. Infections were most prevalent as a first manifestation in patients presenting after age 30 years.CONCLUSIONS: An exclusive focus on infection-centered warning signs would have missed around 25% of patients with IEI who initially present with other manifestations.
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- 2021
38. Adrenaline Auto-Injectors for Preventing Fatal Anaphylaxis.
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Sim M, Sharma V, Li K, Gowland MH, Garcez T, Shilladay C, Pumphrey R, Patel N, Turner PJ, and Boyle RJ
- Abstract
Anaphylaxis affects up to 5% of people during their lifetime. Although anaphylaxis usually resolves without long-term physical consequences, it can result in anxiety and quality of life impairment. Rarely and unpredictably, community anaphylaxis can cause rapid physiological decompensation and death. Adrenaline (epinephrine) is the cornerstone of anaphylaxis treatment, and provision of adrenaline autoinjectors (AAI) has become a standard of care for people at risk of anaphylaxis in the community. In this article, we explore the effectiveness of AAIs for preventing fatal outcomes in anaphylaxis, using information drawn from animal and human in vivo studies and epidemiology. We find that data support the effectiveness of intravenous adrenaline infusions for reversing physiological features of anaphylaxis, typically at doses from 0.05 to 0.5 μg/kg/min for 1-2 h, or ~ 10 μg/kg total dose. Intramuscular injection of doses approximating 10 μg/kg in humans can result in similar peak plasma adrenaline levels to intravenous infusions, at 100-500 pg/mL. However, these levels are typically short-lived following intramuscular adrenaline, and pharmacokinetic and pharmacodynamic outcomes can be unpredictable. Epidemiological data do not support an association between increasing AAI prescriptions and reduced fatal anaphylaxis, although carriage and activation rates remain low. Taken together, these data suggest that current AAIs have little impact on rates of fatal anaphylaxis, perhaps due to a lack of sustained and sufficient plasma adrenaline concentration. Effects of AAI prescription on quality of life may be variable. There is a need to consider alternatives, which can safely deliver a sustained adrenaline infusion via an appropriate route., (Clinical & Experimental Allergy© 2024 The Author(s). Clinical & Experimental Allergy published by John Wiley & Sons Ltd.)
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- 2024
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39. Investigating pulmonary and non-infectious complications in common variable immunodeficiency disorders: a UK national multi-centre study.
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Bintalib HM, Grigoriadou S, Patel SY, Mutlu L, Sooriyakumar K, Vaitla P, McDermott E, Drewe E, Steele C, Ahuja M, Garcez T, Gompels M, Grammatikos A, Herwadkar A, Ayub R, Halliday N, Burns SO, Hurst JR, and Goddard S
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- Humans, Female, Male, United Kingdom epidemiology, Retrospective Studies, Middle Aged, Adult, Cross-Sectional Studies, Bronchiectasis epidemiology, Aged, Young Adult, Registries, Common Variable Immunodeficiency complications, Common Variable Immunodeficiency epidemiology, Lung Diseases, Interstitial etiology, Lung Diseases, Interstitial epidemiology
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Background: Common Variable Immunodeficiency Disorders (CVID) encompass a spectrum of immunodeficiency characterised by recurrent infections and diverse non-infectious complications (NICs). This study aimed to describe the clinical features and variation in NICs in CVID with and without interstitial lung disease (ILD) from a large UK national registry population., Methods: Retrospective, cross-sectional data from a UK multicentre database (previously known as UKPIN), categorising patients into those with CVID-ILD and those with NICs related to CVID but without pulmonary involvement (CVID-EP; EP= extra-pulmonary involvement only)., Results: 129 patients were included. Chronic lung diseases, especially CVID-ILD, are prominent complications in complex CVID, occurring in 62% of the cohort. Bronchiectasis was common (64% of the cohort) and associated with greater pulmonary function impairment in patients with CVID-ILD compared to those without bronchiectasis. Lymphadenopathy and the absence of gastrointestinal diseases were significant predictors of ILD in complex CVID. Although the presence of liver disease did not differ significantly between the groups, nearly half of the CVID-ILD patients were found to have liver disease. Patients with CVID-ILD were more likely to receive immunosuppressive treatments such as rituximab and mycophenolate mofetil than the CVID-EP group, indicating greater need for treatment and risk of complications., Conclusion: This study highlights the significant burden of CVID-ILD within the CVID population with NICs only. The lungs emerged as the most frequently affected organ, with ILD and bronchiectasis both highly prevalent. These findings emphasise the necessity of a comprehensive and multidisciplinary approach in managing CVID patients, considering their susceptibility to various comorbidities and complications., Competing Interests: LM received speaker fees and travel grants from Takeda, BioCryst and CSL. MA received honoraria from Takeda and Biocryst for educational activities in the past. No direct conflicts with this work. TG received consulting, advisory work and educational support from BioCryst, CSL Behring, KalVista, Novartis, Octapharma, Pharming, Pharvaris and Takeda. AH received support for attending meetings from Biocryst, Pharming and Takeda. NH has received honoraria from Dr Falk and Advanz Pharma for educational activities and has consultancy agreements with Mirum Pharma and Signant Health, none of which relate to this work. SB received grant support from CSL Behring and personal fees or travel expenses from Grifols, Pharming, GSK, CSL Behring, Baxalta US Inc and Biotest. CS: Received honoraria and educational support from BioCryst, CSL Behring, Pharming and Takeda. JH received grant support, and payment for educational and advisory work to his institution from pharmaceutical companies that make medicines to treat respiratory disease and immunodeficiency. Personal payment for educational and advisory work, and support to attend meetings from the same. SGo received Takeda sponsored meetings in the last 12 months and was paid to co-lead a workshop relating to immunodeficiency. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Bintalib, Grigoriadou, Patel, Mutlu, Sooriyakumar, Vaitla, McDermott, Drewe, Steele, Ahuja, Garcez, Gompels, Grammatikos, Herwadkar, Ayub, Halliday, Burns, Hurst and Goddard.)
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- 2024
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40. Anaphylaxis to Patent Blue V Dye With Clinical Deterioration Following Administration of Suxamethonium and Sensitisation on Skin Testing to Methylene Blue, Patent Blue V Dye and Suxamethonium.
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Benison EA, Garcez T, Chambers L, and Parkes A
- Abstract
Neuromuscular blocking drugs (NMBAs) and Patent Blue V dye sodium salt 2.5% (Guerbet, Roissy, France) are frequently implicated in perioperative allergic immunoglobulin E (IgE) mediated anaphylaxis. Most cases of anaphylaxis during surgery occur at induction of anaesthesia, although reactions to vital dyes injected into soft tissues often show a delayed onset. We present the case of a female in her 60s who suffered perioperative anaphylaxis to Patent Blue V dye and possibly suxamethonium during oncological breast surgery. Allergy clinic follow-up confirmed sensitivity to both drugs which may explain the unusual bi-phasic nature of the reaction. Intradermal testing also found cross-sensitivity to methylene blue, but not to other common allergens or NMBAs. This case demonstrates the importance of thorough post-anaphylaxis follow-up and raises the possibility of cross-sensitivity between unrelated compounds., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Benison et al.)
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- 2024
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41. Prophylaxis in hereditary angioedema: a United Kingdom Delphi consensus.
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Yong PFK, Annals R, Diwakar L, Elkhalifa S, Gompels M, Jain R, Karim MY, Khan S, Metcalfe A, Noorani S, Steele C, Kiani-Alikhan S, and Garcez T
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- Humans, United Kingdom, Complement C1 Inhibitor Protein therapeutic use, Angioedemas, Hereditary prevention & control, Angioedemas, Hereditary drug therapy, Consensus, Delphi Technique
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Hereditary angioedema (HAE) is a rare inherited disorder causing recurrent episodes of swelling that can be potentially life threatening. Treatment of HAE can be divided into on-demand treatment for swelling, and prophylaxis. The last UK consensus on HAE was in 2014 and since then, new medications for prophylaxis have been developed, with more drugs in the pipeline. International guidelines currently recommend the use of long-term prophylaxis (LTP) as the only way of achieving disease control and normalizing patient lives. Modern prophylactic medications are available in the UK, although access is restricted primarily by HAE attack frequency. To establish an updated view of UK clinicians and patients, a Delphi process was used to develop statements regarding LTP as well as other aspects of HAE management. There was consensus that UK access criteria for modern LTP agents based on numerical frequency of attacks alone are too simplistic and potentially disadvantage a cohort of patients who may benefit from LTP. Additionally, there was agreement that patients should be seen in expert centres, remote monitoring of patients is popular post-pandemic, and that the use of patient-reported outcome measures has the potential to improve patient care. Psychological health is an area in which patients may benefit, and recognition of this is important for future research and development., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Immunology. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)
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- 2024
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42. A National Survey of Hereditary Angioedema and Acquired C1 Inhibitor Deficiency in the United Kingdom.
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Yong PFK, Coulter T, El-Shanawany T, Garcez T, Hackett S, Jain R, Kiani-Alikhan S, Manson A, Noorani S, Stroud C, Symons C, Sargur R, Steele C, Alachkar H, Anantharachagan A, Arkwright PD, Bernatoniene J, Bhole M, Brown L, Buckland M, Burns S, Chopra C, Darroch J, Drewe E, Edmonds J, Ekbote A, Elkhalifa S, Goddard S, Grosse-Kreul D, Gurugama P, Hague R, Herriot R, Herwadkar A, Hughes SM, Jones L, Lear S, McDermott E, Kham Murng SH, Price A, Redenbaugh V, Richter A, Riordan A, Shackley F, Stichbury J, Springett D, Tarzi MD, Thomas M, Vijayadurai P, and Worth A
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- Humans, Female, Male, Complement C1 Inhibitor Protein therapeutic use, Danazol therapeutic use, United Kingdom epidemiology, Surveys and Questionnaires, Angioedemas, Hereditary epidemiology, Angioedemas, Hereditary drug therapy
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Background: Detailed demographic data on people with hereditary angioedema (HAE) and acquired C1 inhibitor deficiency in the United Kingdom are relatively limited. Better demographic data would be beneficial in planning service provision, identifying areas of improvement, and improving care., Objective: To obtain more accurate data on the demographics of HAE and acquired C1 inhibitor deficiency in the United Kingdom, including treatment modalities and services available to patients., Methods: A survey was distributed to all centers in the United Kingdom that look after patients with HAE and acquired C1 inhibitor deficiency to collect these data., Results: The survey identified 1152 patients with HAE-1/2 (58% female and 92% type 1), 22 patients with HAE with normal C1 inhibitor, and 91 patients with acquired C1 inhibitor deficiency. Data were provided by 37 centers across the United Kingdom. This gives a minimum prevalence of 1:59,000 for HAE-1/2 and 1:734,000 for acquired C1 inhibitor deficiency in the United Kingdom. A total of 45% of patients with HAE were on long-term prophylaxis (LTP) with the most used medication being danazol (55% of all patients on LTP). Eighty-two percent of patients with HAE had a home supply of acute treatment with C1 inhibitor or icatibant. A total of 45% of patients had a supply of icatibant and 56% had a supply of C1 inhibitor at home., Conclusions: Data obtained from the survey provide useful information about the demographics and treatment modalities used in HAE and acquired C1 inhibitor deficiency in the United Kingdom. These data are useful for planning service provision and improving services for these patients., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)
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- 2023
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43. Reporting anaphylaxis deaths to UK Fatal Anaphylaxis Registry (UKFAR).
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Sharma V, Garcez T, Shilladay C, Parkes A, and Pumphrey R
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- Humans, Risk Factors, Registries, United Kingdom epidemiology, Anaphylaxis
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Competing Interests: Richard Pumphrey is chair of the British Society for Allergy and Clinical Immunology UKFAR Steering Committee Competing interests: None declared.
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- 2023
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44. Lanadelumab for the prevention of hereditary angioedema attacks: A real-world UK audit.
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Dorr AD, Chopra C, Coulter TI, Dempster J, Dziadzio M, El-Shanawany T, Garcez T, Gompels M, Herriot R, Jain R, Levi M, Lorenzo L, Makki I, Mapazire E, Murng SHK, Noorani S, Savic S, Steele CL, Symons C, Tarzi M, Yong PFK, and Kiani-Alikhan S
- Subjects
- Humans, Antibodies, Monoclonal, Humanized, Plasma Kallikrein, United Kingdom epidemiology, Complement C1 Inhibitor Protein therapeutic use, Angioedemas, Hereditary drug therapy, Angioedemas, Hereditary prevention & control
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- 2023
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- View/download PDF
45. Berotralstat for the prophylaxis of hereditary angioedema-Real-world evidence data from the United Kingdom.
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Ahuja M, Dorr A, Bode E, Boulton APR, Buckland M, Chee S, Dalley C, Denman S, Ekbote A, Elkhalifa S, El-Shanawany T, Eren E, Herwadkar A, Garcez T, Ghanta H, Grammatikos A, Grigoriadou S, Jain R, Lorenzo L, Manson A, Moon E, Murng S, Murphy A, Mutlu L, Peters N, Sooriyakumar K, Stroud C, Townsend K, Yellon RL, Yong P, and Kiani-Alikhan S
- Subjects
- Humans, Pyrazoles therapeutic use, United Kingdom epidemiology, Complement C1 Inhibitor Protein therapeutic use, Angioedemas, Hereditary drug therapy, Angioedemas, Hereditary epidemiology, Angioedemas, Hereditary prevention & control, Angioedema drug therapy
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- 2023
- Full Text
- View/download PDF
46. Addressing the Challenges of Penicillin Allergy Delabeling With Electronic Health Records and Mobile Applications.
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Powell N, Elkhalifa S, Guyer A, Garcez T, Sandoe J, and Zhou L
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- Humans, Electronic Health Records, Penicillins adverse effects, Anti-Bacterial Agents adverse effects, Mobile Applications, Drug Hypersensitivity diagnosis, Drug Hypersensitivity therapy, Hypersensitivity
- Abstract
Allergy labels are common, often incorrect, and potentially harmful. There are many opportunities for clinical decision support (CDS) tools integrated in the electronic health record (EHR) and mobile apps to address the challenges with drug allergy management, including penicillin allergy delabeling (PADL). Effective delabeling solutions must consider multidisciplinary clinical workflow and multistep processes, including documentation, assessment, plan (eg, allergy testing and referral), record update, drug allergy alert management, and allergy reconciliation over time. Developing a systematic infrastructure to manage allergies across the EHR is critical to improve the accuracy and completeness of a patient's allergy and avoid inadvertently relabeling. Improving the appropriateness and relevancy of drug allergy alerts is important to reduce alert fatigue. Using alerts to guide clinicians on appropriate antibiotic use may reduce unnecessary β-lactam avoidance. To date, EHR CDS tools have facilitated non-allergists to provide PADL at the point of care. A mobile app was shown to support PADL and provide specialist support and education. Future research is needed to standardize, integrate, and evaluate innovative CDS tools in the EHR to demonstrate patient safety and clinical utility and facilitate wider adoption., (Copyright © 2022 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)
- Published
- 2023
- Full Text
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47. BSACI guideline for the set-up of penicillin allergy de-labelling services by non-allergists working in a hospital setting.
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Savic L, Ardern-Jones M, Avery A, Cook T, Denman S, Farooque S, Garcez T, Gold R, Jay N, Krishna MT, Leech S, McKibben S, Nasser S, Premchand N, Sandoe J, Sneddon J, and Warner A
- Subjects
- Adult, Anti-Bacterial Agents adverse effects, Child, Hospitals, Humans, beta-Lactams adverse effects, Drug Hypersensitivity diagnosis, Drug Hypersensitivity epidemiology, Drug Hypersensitivity therapy, Penicillins adverse effects
- Abstract
The Standards of Care Committee of the British Society for Allergy and Clinical Immunology (BSACI) and a committee of experts and key stakeholders have developed this guideline for the evaluation and testing of patients with an unsubstantiated label of penicillin allergy. The guideline is intended for UK clinicians who are not trained in allergy or immunology, but who wish to develop a penicillin allergy de-labelling service for their patients. It is intended to supplement the BSACI 2015 guideline "Management of allergy to penicillin and other beta-lactams" and therefore does not detail the epidemiology or aetiology of penicillin allergy, as this is covered extensively in the 2015 guideline (1). The guideline is intended for use only in patients with a label of penicillin allergy and does not apply to other beta-lactam allergies. The recommendations include a checklist to identify patients at low risk of allergy and a framework for the conduct of drug provocation testing by non-allergists. There are separate sections for adults and paediatrics within the guideline, in recognition of the common differences in reported allergy history and likelihood of true allergy., (© 2022 John Wiley & Sons Ltd.)
- Published
- 2022
- Full Text
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48. Tissue engineering application combining epoxidized natural rubber blend and mesenchymal stem cells in in vivo response.
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Henckes NAC, Chuang L, Bosak I, Carazzai R, Garcez T, Kuhl CP, de Oliveira FDS, Loureiro Dos Santos LA, Visioli F, and Cirne-Lima EO
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- Animals, Collagen metabolism, Female, Ki-67 Antigen metabolism, Lactic Acid, Polyglycolic Acid, Polylactic Acid-Polyglycolic Acid Copolymer, Rats, Rubber metabolism, Tissue Scaffolds, Mesenchymal Stem Cells metabolism, Tissue Engineering methods
- Abstract
This study aimed to investigate biocompatibility, integration, and tissue host response of the Poly (Lactic-co-Glycolic acid) (PLGA)/Poly (isoprene) (PI) epoxidized (PLGA/PIepox) innovative scaffold combined with adipose derived mesenchymal stem cells (ADSC). We implanted the scaffold subcutaneously on the back of 18 female rats and monitored them for up to 14 days. When compared to controls, PLGA/PIepox + ADSC demonstrated an earlier vascularization, a tendency of inflammation reduction, an adequate tissue integration, higher cell proliferation, and a tendency of expression of collagen decreasing. However, 14 days post-implantation we found similar levels of CD31, Ki67 and AE1/AE3 in PLGA/PIepox when compared to control groups. The interesting results, lead us to the assumption that PLGA/PIepox is able to provide an effective delivery system for ADSC on tissue host. This animal study assesses PLGA/PIepox + ADSC in in vivo tissue functionality and validation of use, serving as a proof of concept for future clinical translation as it presents an innovative and promising tissue engineering opportunity for the use in tissue reconstruction.
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- 2022
- Full Text
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49. Allergies and COVID-19 vaccines: An ENDA/EAACI Position paper.
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Barbaud A, Garvey LH, Arcolaci A, Brockow K, Mori F, Mayorga C, Bonadonna P, Atanaskovic-Markovic M, Moral L, Zanoni G, Pagani M, Soria A, Jošt M, Caubet JC, Carmo A, Mona AA, Alvarez-Perea A, Bavbek S, Benedetta B, Bilo MB, Blanca-López N, Bogas HG, Buonomo A, Calogiuri G, Carli G, Cernadas J, Cortellini G, Celik G, Demir S, Doña I, Dursun AB, Eberlein B, Faria E, Fernandes B, Garcez T, Garcia-Nunez I, Gawlik R, Gelincik A, Gomes E, Gooi JHC, Grosber M, Gülen T, Hacard F, Hoarau C, Janson C, Johnston SL, Joerg L, Kepil Özdemir S, Klimek L, Košnik M, Kowalski ML, Kuyucu S, Kvedariene V, Laguna JJ, Lombardo C, Marinho S, Merk H, Meucci E, Morisset M, Munoz-Cano R, Murzilli F, Nakonechna A, Popescu FD, Porebski G, Radice A, Regateiro FS, Röckmann H, Romano A, Sargur R, Sastre J, Scherer Hofmeier K, Sedláčková L, Sobotkova M, Terreehorst I, Treudler R, Walusiak-Skorupa J, Wedi B, Wöhrl S, Zidarn M, Zuberbier T, Agache I, and Torres MJ
- Subjects
- Humans, Vaccines, Synthetic, mRNA Vaccines, Anaphylaxis diagnosis, COVID-19 prevention & control, COVID-19 Vaccines adverse effects, Drug Hypersensitivity diagnosis, Drug Hypersensitivity etiology, Drug Hypersensitivity therapy, Vaccines
- Abstract
Background: Anaphylaxis, which is rare, has been reported after COVID-19 vaccination, but its management is not standardized., Method: Members of the European Network for Drug Allergy and the European Academy of Allergy and Clinical Immunology interested in drug allergy participated in an online questionnaire on pre-vaccination screening and management of allergic reactions to COVID-19 vaccines, and literature was analysed., Results: No death due to anaphylaxis to COVID-19 vaccines has been confirmed in scientific literature. Potential allergens, polyethylene glycol (PEG), polysorbate and tromethamine are excipients. The authors propose allergy evaluation of persons with the following histories: 1-anaphylaxis to injectable drug or vaccine containing PEG or derivatives; 2-anaphylaxis to oral/topical PEG containing products; 3-recurrent anaphylaxis of unknown cause; 4-suspected or confirmed allergy to any mRNA vaccine; and 5-confirmed allergy to PEG or derivatives. We recommend a prick-to-prick skin test with the left-over solution in the suspected vaccine vial to avoid waste. Prick test panel should include PEG 4000 or 3500, PEG 2000 and polysorbate 80. The value of in vitro test is arguable., Conclusions: These recommendations will lead to a better knowledge of the management and mechanisms involved in anaphylaxis to COVID-19 vaccines and enable more people with history of allergy to be vaccinated., (© 2022 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.)
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- 2022
- Full Text
- View/download PDF
50. Development and Validation of a Mobile Clinical Decision Support Tool for the Diagnosis of Drug Allergy in Adults: The Drug Allergy App.
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Elkhalifa S, Bhana R, Blaga A, Joshi S, Svejda M, Kasilingam V, Garcez T, and Calisti G
- Subjects
- Adult, Anti-Bacterial Agents therapeutic use, Humans, Overdiagnosis, Penicillins, Retrospective Studies, Decision Support Systems, Clinical, Drug Hypersensitivity diagnosis, Drug Hypersensitivity drug therapy, Drug Hypersensitivity epidemiology, Mobile Applications
- Abstract
Background: Penicillin allergy overdiagnosis has been associated with inappropriate antibiotic prescribing, increased antimicrobial resistance, worse clinical outcomes, and increased health care costs., Objective: To develop and validate a questionnaire-based algorithm built in a mobile application to support clinicians in collecting accurate history of previous reactions and diagnosing drug allergy appropriately., Methods: A survey was completed by 164 medical and nonmedical prescribers to understand barriers to best practice. Based on the survey recommendations, we created a 10-item questionnaire-based algorithm to allow classification of drug allergy history in line with the National Institute for Health and Care Excellence guidelines on drug allergy. The algorithm was incorporated into a mobile application and retrospectively validated using anonymized clinical databases at regional immunology and dermatology centers in Manchester, United Kingdom., Results: A total of 55.2% of prescribers (95% confidence interval, 47% to 63.4%) thought it impossible to draw a firm conclusion based on history alone and 59.4% (95% CI, 51.4% to 67.5%) believed that regardless of the details of the penicillin allergy history, they would avoid all β-lactams. A drug allergy mobile application was developed and retrospectively validated, which revealed a low risk for misclassification of outcomes compared with reference standard drug allergy investigations in the allergy and dermatology clinics., Conclusions: Perceived lack of time and preparedness to collect an accurate drug allergy history appear to be important barriers to appropriate antimicrobial prescribing. The Drug Allergy App may represent a useful clinical decision support tool to diagnose drug allergy correctly and support appropriate antibiotic prescribing., (Copyright © 2021 American Academy of Allergy, Asthma & Immunology. All rights reserved.)
- Published
- 2021
- Full Text
- View/download PDF
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