Chronic kidney disease secondary to atypical hemolytic uremic syndrome in a pediatric patient: Case report
DOI:
https://doi.org/10.33448/rsd-v12i14.44632Keywords:
Atypical hemolytic uremic syndrome; Renal insufficiency, chronic; Thrombotic microangiopathies.Abstract
Introduction: Atypical hemolytic uremic syndrome (aHUS) is a rare condition of thrombotic microangiopathy, with multisystem involvement and is clinically manifested by the triad of non-immune microangiopathic hemolytic anemia, thrombocytopenia and renal injury. The prognosis in these cases is unfavorable due to the high rate of morbidity and mortality in the acute phase, in addition to the risk of progression to end-stage chronic kidney disease in around 50% of cases. The objective of this work is to report the case of a pediatric patient with the development of chronic kidney disease (CKD)., demonstrating the importance of early diagnosis, in addition to longitudinal monitoring for adequate management of comorbidities. Clinical case: Student, 9 years old, female, diagnosed with atypical hemolytic-uremic syndrome 5 years ago and undergoing irregular outpatient follow-up with pediatric nephrology since then. During this period, he presented a gradual deterioration in renal function, developing CKD and currently, he presents associated complications, such as anemia, short stature and hypertension. Discussion: As stated, one of the consequences of aHUS is the progression to CKD.
References
Ardissino G., Testa S., Possenti I. et al. (2014). Discontinuation of Eculizumab Maintenance Treatment for Atypical Hemolytic Uremic Syndrome: A Report of 10 Cases. American Journal of Kidney Diseases. 64(4), 633-7. https://doi.org/10.1053/j.ajkd.2014.01.434
Cugno, M., Gualtierotti, R., Possenti, I., Testa, S., Tel, F., Griffini, S., Grovetti, E., Tedeschi, S., Salardi, S., Cresseri, D., Messa, P., & Ardissino, G. (2014). Complement functional tests for monitoring eculizumab treatment in patients with atypical hemolytic uremic syndrome. J Thromb Haemost. 12(9), 1440-8. 10.1111/jth.12615.
Dixon, B. P. & Gruppo, R. A. (2018). Atypical Hemolytic Uremic Syndrome. Pediatr Clin North Am. 65(3), 509-25. 10.1016/j.pcl.2018.02.003.
Habib R. (1992). Pathology of the hemolytic uremic syndrome. In: Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura, Kaplan B, Trompeter R, Moake J (Eds), Marcel Dekker, p.315.
Laurence J. (2012). Atypical hemolytic uremic syndrome (aHUS): making the diagnosis. Clin Adv Hematol Oncol. 10(10), 1-12.
Laurence, J., Haller, H., Mannucci, P. M., Nangaku, M., Praga, M., & Rodriguez de Cordoba S. (2016). Síndrome hemolítica urêmica atípica (HUSA): aspectos essenciais de um diagnóstico preciso. Clin Adv Hematol Oncol. 14(11):2-15.
Legendre, C. M., Licht, C., Muus, P., Greenbaum, L. A., Babu, S., Bedrosian, C., Bingham, C., Cohen, D. J., Delmas, Y., Douglas, K., Eitner, F., Feldkamp, T., Fouque, D., Furman, R. R., Gaber, O., Herthelius, M., Hourmant, M., Karpman, D., Lebranchu, Y., Mariat, C., Menne. J., Moulin, B., Nürnberger, J., Ogawa, M., Remuzzi, G., Richard, T., Sberro-Soussan, R., Severino. B., Sheerin, N. S., Trivelli, A., Zimmerhackl, L. B., Goodship, T. & Loirat, C. (2013). Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 368(23), 2169-81. 10.1056/NEJMoa1208981.
Niaudet, P., & Boyer, O. Complement-mediated hemolytic uremic syndrome in children. In: Mattoo, T., Hoppin, A, ed. UpToDate. Waltham, Mass.: UpToDate, 2023. https://www.uptodate.com/contents/complement-mediated-hemolytic-uremic-syndrome-in-children?search=s%C3%ADndrome%20hemol%C3%ADtico-urêmica%20at%C3%ADpica&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1.
Palma, L. (2023). Ainda precisamos falar sobre a Síndrome Hemolítico-Urêmica: Reconhecimento precoce é fundamental! Braz. J. Nephrol. 45(1), 5-7.
Panzarino, V., Lesser, J. & Cassani, F. (2022). Pediatric Chronic Kidney Disease. Adv Pediatr. 69(1), 123-32. 10.1016/j.yapd.2022.03.008.
Pereira, et. al. (2018). Metodologia da Pesquisa Científica. 70-73. UFSM.
Sengul Samanci N, Ayer M, Ergen A, & Ozturk S. (2015). An effective treatment of atypical hemolytic uremic syndrome with plasma exchange and eculizumab: A case report. Transfus Apher Sci. 52(3), 314-6. 10.1016/j.transci.2015.01.006.
Serna, A., & Boedeker, E. C. (2008) Pathogenesis and treatment of Shiga toxin-producing Escherichia coli infections. Curr Opin Gastroenterol 24:38-47. http://dx.doi.org/10.1097/MOG.0b013e3282f2dfb8
Sociedade Brasileira de Pediatra. Departamento Científico de Nefrologia (2019-2021). Tratamento conservador da doença renal crônica em pediatria: Noções gerais. (5), Outubro de 2020.
Sociedade Brasileira de Pediatria. Departamento Científico de Nefrologia (2019-2021). Doença Renal Crônica em Pediatria: Diagnóstico e Prevenção. (4) Maio de 2020.
Spero, R., Cataland, V., Michael Holers, S. G., Shangbin Yang, Haifeng M. Wu. (2014). Biomarkers of terminal complement activation confirm the diagnosis of aHUS and differentiate aHUS from TTP, Blood, 123(24), 3733-3738. https://doi.org/10.1182/blood-2013-12-547067. (https://www.sciencedirect.com/science/article/pii/S0006497120401077)
Sridharan, M., Go, R. S., & Willrich, M. A. V. (2018). Atypical hemolytic uremic syndrome: Review of clinical presentation, diagnosis and management. J Immunol Methods. 461, 15-22. 10.1016/j.jim.2018.07.006.
Talarico, V., Aloe, M., Monzani, A., Miniero, R., & Bona, G. (2016). Hemolytic uremic syndrome in children. Minerva Pediatr. 68(6), 441-455.
Vaisbich, M. H. (2014). Síndrome hemolítico-urêmica na infância. J Bras Nefrol 36(2). https://doi.org/10.5935/0101-2800.20140032
Vilardouro, A. S., et al. (2022). Síndrome hemolítico-urêmica: 24 anos de experiência de uma unidade de nefrologia pediátrica. J Bras Nefrol. 2022,45(1):51-9. 10.1590/2175-8239-JBN-2021-0206
Warady, B., & Weidemann, D. (2023). Chronic kidney disease in children: Definition, epidemiology, etiology, and course. In: Matto T, Hoppin A, ed. UpToDate. Waltham, Mass.: UpToDate 2023. Acessado em 05 de julho de 2023.
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