Atypical Hemolytic-Uremic Syndrome: Clinical manifestations and challenges in diagnosis
DOI:
https://doi.org/10.33448/rsd-v10i9.18365Keywords:
Microangiopathies; Hemolytic anemia; Uremic Syndrome; Diagnosis.Abstract
Atypical hemolytic uremic syndrome (aHUS) is characterized by a triad of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure. The pathogenesis of HUS corresponds to thrombotic microangiopathy (THA). The work aims to expose through the analysis of scientific articles the main clinical manifestations and challenges in the diagnosis of Atypical Hemolytic-Uremic Syndrome. This is a literature review of a qualitative nature based on scientific production based on studies published between 2018 and 2021. Although atypical hemolytic uremic syndrome is rare, it is related to complications and important sequelae secondary to alterations of kidney function; 42% of children and 27% of adults may have severe thrombocytopenia (<50,000 platelets/mL). Similarly, related acute kidney injury is severe in 59% of children and 81% of adults, and may be accompanied by glomerular involvement, creatinine, hematuria, proteinuria, edema, and hypertension. The treatment that is most effective against aHUS is the use of the drug Eculizumab. This drug has a high cost, but studies show an increase in life expectancy and quality of life for these patients with the use of this medication. It is necessary that more studies about this disease are carried out for the development of new diagnostic measures and therapies against aHUS at a lower cost and with easy access for the population due to the seriousness of the disease and its high mortality rate.
References
Bello-Marquez, D. C., Nieto-Rios, J. F., Serna-Higuita, L. M., & Gonzalez-Vergara, A. J. (2020). Síndrome nefrótica associada à síndrome hemolítico-urêmica atípica primária. Brazilian Journal of Nephrology, (AHEAD).
Caetano, R., Rodrigues, P. H. A., Corrêa, M. C. V., Villardi, P., & Osorio-de-Castro, C. G. S. (2020). O caso do eculizumabe: judicialização e compras pelo Ministério da Saúde. Revista de Saúde Pública, 54, 22.
Chaturvedi, S., Dhaliwal, N., Hussain, S., Dane, K., Upreti, H., Braunstein, E. M., & Brodsky, R. A. (2021). Resultados de um protocolo dirigido por um médico para a descontinuação da terapia de inibição do complemento na síndrome hemolítica urêmica atípica. Blood Advances , 5 (5), 1504-1512.
Lopes, A. J. B., Magalhães, B. A., Bezerra, F. C. M., Megali, H. A., de Souza Queiroz, H., de Oliveira, M. T., & Silva, T. P. (2020). Eculizumab no Transplante Renal: Eficácia na prevenção da síndrome hemolítica urêmica. Brazilian Journal of Health Review, 3(4), 9890-9896.
Maximiano, C., Silva, A., Duro, I., Branco, T., Correia-Costa, L., Teixeira, A., & Afonso, A. C. (2021). Síndrome hemolítica urêmica atípica genética em crianças: uma experiência de 20 anos a partir de um centro terciário. Brazilian Journal of Nephrology.
Mena Miranda, V. R., & Puig Souza, L. (2019). Avances en el conocimiento de la patogénesis del síndrome hemolítico urémico. Revista Cubana de Pediatría, 91(3).
Molina, N. M., Rotondo, S., Dos Santos, C., & Sánchez-Luceros, A. (2020). Biomarcadores y blancos moleculares del complemento en el diagnóstico de las microangiopatías trombóticas. Acta Bioquímica Clínica Latinoamericana, 54(4), 437-453.
Pugh, D., D O'Sullivan, E., Duthie, F. A., Masson, P., & Kavanagh, D. (2021). Interventions for atypical haemolytic uraemic syndrome. Cochrane Database of Systematic Reviews, (3).
Roldán-Tabares, M. D., & Ruiz-Mejía, C. (2018). Síndrome hemolítico urémico atípico: papel del perfil genético. Medicina interna de México, 34(3), 394-402.
Santos, M. M. D. A. (2018). Púrpura trombocitopénica trombótica: síndrome hemolítica urémica e o sistema de complemento (Doctoral dissertation).
Sepúlveda, R. A., Tagle, R., & Jara, A. (2018). Síndrome hemolítico urémico atípico. Revista médica de Chile, 146(6), 770-779.
Tagle, R., Rivera, G., Walbaum, B., & Sepúlveda, R. A. (2018). Síndrome hemolítico urémico atípico en tratamiento con eculizumab. Casos clínicos. Revista médica de Chile, 146(2), 254-259.
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Copyright (c) 2021 Lennara Pereira Mota; Maria Vitalina Alves de Sousa; Ana Alinne Gomes da Penha; Alexandra Rodrigues Cardoso; Francisco de Assis da Silva Sousa; Francilene Vieira da Silva Freitas; Josana de Mello Dantas; Thaline Ravena Nunes Costa; Thaynara Alves Rodrigues Paulo da Silva; Eudes Rufino da Silveira Filho; Wilian Reis Rosário; Regina Célia da Silva; Lucas Costa de Gois; Jaqueline Dantas da Costa; Mayara Madeira de Carvalho; Stefânia Araújo Pereira; Joice Mara Ferreira dos Santos
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