Clinical profile of patients with Hereditary Angioedema treated at a teaching hospital in the City of Maceió, Alagoas

Authors

DOI:

https://doi.org/10.33448/rsd-v10i9.17959

Keywords:

Hereditary angioedema; C1 inhibitor deficiency; Tranexemic acid.

Abstract

Hereditary angioedema (HAE) is a rare genetic disease characterized by recurrent cutaneous angioedema, severe abdominal pain, and airway compromise. This study aimed to describe the clinical profile of patients with HAE treated at the Clinic of Clinical Immunology and Allergy of a university hospital in the city of Maceió, Alagoas, Brazil. This is a retrospective, cross-sectional and descriptive study, where data such as sex, age at onset of HAE, manifestations, triggering factors, prophylactic therapy and crisis therapy were collected. Thirteen participants formed the total sample, eight male and aged between 08 and 40 years. Main triggering factor for trauma, with edema in the extremities being the most frequent sign. Tranexemic acid was the drug referred to as a therapeutic agent in times of crisis. The family history factor was present in 69.2% of the participants and, even though tranaxemic acid is not recommended for long-term prophylaxis for HAE, it is still a reference among the patients in this study, revealing the need to disseminate knowledge about the pathophysiological aspects of disease processes directed at health teams, in order to guide adequate therapy and, thus, reduce morbidity and mortality, ensuring a better quality of life for patients, as well as their families.

References

Araújo-Simões, J., Boanova, A., Constantino-Silva, R. N., Fragnan, N., Pinto, J. A., Minafra, F. G., Gonçalves, R. F., Valle, S., Alonso, M., Dortas, S. D., Goudouris, E. S., Rêgo-Silva, A. M., Marques, M. M., Serpa, F. S., Chong-Neto, H. J., Nelson, R. F., Mansour, E., Moreira, I. F., Moreno, A. S., Arruda, L. K., … Grumach, A. S. (2021). The Challenges in the Follow-Up and Treatment of Brazilian Children with Hereditary Angioedema. International archives of allergy and immunology, 182(7), 585–591.

Arruda, L. K., & Leme Ferriani, M. P. (2021). Treatment of hereditary angioedema: When the goal is having a normal life. The Journal of allergy and clinical immunology, S0091-6749(21)00828-9. Advance online publication. https://doi.org/10.1016/j.jaci.2021.05.023.

Aygören-Pürsün, E., & Bork, K. (2019). Hereditäres Angioödem [Hereditary angioedema]. Der Internist, 60(9), 987–995.

Bellanti, J. A., & Settipane, R. A. (2018). Hereditary angioedema revisited. Allergy and asthma proceedings, 39(5), 329–331. https://doi.org/10.2500/aap.2018.39.4169.

Betschel, S., Badiou, J., Binkley, K., Borici-Mazi, R., Hébert, J., Kanani, A., Keith, P., Lacuesta, G., Waserman, S., Yang, B., Aygören-Pürsün, E., Bernstein, J., Bork, K., Caballero, T., Cicardi, M., Craig, T., Farkas, H., Grumach, A., Katelaris, C., Longhurst, H., … Tsai, E. (2019). The International/Canadian Hereditary Angioedema Guideline. Allergy, asthma, and clinical immunology: official journal of the Canadian Society of Allergy and Clinical Immunology, 15, 72.

Bork, K., Hardt, J., & Witzke, G. (2012). Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. The Journal of allergy and clinical immunology, 130(3), 692–697.

Busse, P. J., & Christiansen, S. C. (2020). Hereditary Angioedema. The New England journal of medicine, 382(12), 1136–1148.

Caballero, M. T., Pedrosa, D.M., & Gómez, T.C. (2015). Angioedema hereditario. Medicina Clínica, 145(8), 356–365.

Campos, R. A., Valle, S., & Toledo, E. C. (2021). Hereditary angioedema: a disease seldom diagnosed by pediatricians. Jornal de pediatria, 97 Suppl 1, S10–S16. https://doi.org/10.1016/j.jped.2020.10.011.

Christiansen, S. C., Davis, D. K., Castaldo, A. J., & Zuraw, B. L. (2016). Pediatric Hereditary Angioedema: Onset, Diagnostic Delay, and Disease Severity. Clinical pediatrics, 55(10), 935–942.

Estrela, C. (2018). Metodologia Científica: Ciência, Ensino, Pesquisa. Editora Artes Médicas.

Fabiani, J., Valle, S. O., Olivares, M., Nieto, S., Landeros, E. H., Ginaca, A., Bezrodnik, L., Nievas, E., Oleastro, M., Barrera, O. M., Gallardo, A. M., King, A., Galindo, J. R., Carabantes, M. J., Craig, T., Alfonso, M. M., Montenegro, C., & Grumach, A. S. (2014). Improving C1 inhibitor deficiency (type 1 and type 2 hereditary angioedema) in Latin America. Journal of investigational allergology & clinical immunology, 24(6), 445–447.

Giavina-Bianchi, P., Arruda, L. K., Aun, M. V., Campos, R. A., Chong-Neto, H. J., Constantino-Silva, R. N., Fernandes, F. R., Ferraro, M. F., Ferriani, M., França, A. T., Fusaro, G., Garcia, J., Komninakis, S., Maia, L., Mansour, E., Moreno, A. S., Motta, A. A., Pesquero, J. B., Portilho, N., Rosário, N. A., … Grumach, A. S. (2018). Brazilian Guidelines for Hereditary Angioedema Management - 2017 Update Part 1: Definition, Classification and Diagnosis. Clinics (Sao Paulo, Brazil), 73, e310.

Johnson, N. M., & Phillips, M. A. (2018). New Treatments for Hereditary Angioedema. Skin therapy letter, 23(1), 6–8.

Pancholy, N., & Craig, T. (2019). Hereditary angioedema in children: a review and update. Current opinion in pediatrics, 31(6), 863–868.

Patel G, Pongracic JA (2019). Hereditary and acquired angioedema. Allergy Asthma Proc. 1;40(6):441-445. PMID: 31690390.

Prematta, M. J., Bewtra, A. K., Levy, R. J., Wasserman, R. L., Jacobson, K. W., Machnig, T., & Craig, T. J. (2012). Per-attack reporting of prodromal symptoms concurrent with C1-inhibitor treatment of hereditary angioedema attacks. Advances in therapy, 29(10), 913–922.

Rasmussen, E. R., de Freitas, P. V., & Bygum, A. (2016). Urticaria and Prodromal Symptoms Including Erythema Marginatum in Danish Patients with Hereditary Angioedema. Acta dermato-venereologica, 96(3), 373–376.

Serpa, F. S., Mansour, E., Aun, M. V., Giavina-Bianchi, P., Chong Neto, H. J., Arruda, L. K., Campos, R. A., Motta, A. A., Toledo, E., Grumach, A. S., & Valle, S. (2021). Hereditary angioedema: how to approach it at the emergency department?Einstein (Sao Paulo, Brazil), 19, eRW5498. https://doi.org/10.31744/einstein_journal/2021RW5498.

Wang, K., Geiger, H., & McMahon, A. (2021). Tranexamic acid for ACE inhibitor induced angioedema. The American journal of emergency medicine, 43, 292.e5–292.e7. https://doi.org/10.1016/j.ajem.2020.10.029.

Zotter, Z., Csuka, D., Szabó, E., Czaller, I., Nébenführer, Z., Temesszentandrási, G., Fust, G., Varga, L., & Farkas, H. (2014). The influence of trigger factors on hereditary angioedema due to C1-inhibitor deficiency. Orphanet journal of rare diseases, 9, 44.

Zuraw, B., Lumry, W. R., Johnston, D. T., Aygören-Pürsün, E., Banerji, A., Bernstein, J. A., Christiansen, S. C., Jacobs, J. S., Sitz, K. V., Gower, R. G., Gagnon, R., Wedner, H. J., Kinaciyan, T., Hakl, R., Hanzlíková, J., Anderson, J. T., McNeil, D. L., Fritz, S. B., Yang, W. H., Tachdjian, R., … Riedl, M. A. (2020). Oral once-daily berotralstat for the prevention of hereditary angioedema attacks: A randomized, double-blind, placebo-controlled phase 3 trial. The Journal of allergy and clinical immunology, S0091-6749(20)31484-6. Advance online publication. https://doi.org/10.1016/j.jaci.2020.10.015.

Published

23/07/2021

How to Cite

TENÓRIO, J. L. .; SILVA, N. M. B. .; TÁVORA, P. M. .; CALUMBY, R. J. N. .; MOREIRA, R. T. de F.; MOREIRA, I. F. . Clinical profile of patients with Hereditary Angioedema treated at a teaching hospital in the City of Maceió, Alagoas. Research, Society and Development, [S. l.], v. 10, n. 9, p. e16610917959, 2021. DOI: 10.33448/rsd-v10i9.17959. Disponível em: https://rsdjournal.org/index.php/rsd/article/view/17959. Acesso em: 20 sep. 2021.

Issue

Section

Health Sciences