Abstract
The Hemolytic Uremic Syndrome constitutes a thrombotic microangiopathy, it is one of the main causes of acute kidney injury in children, it also has a high risk of morbimortality. Its etiology is variable, with infectious and / or genetic factors and may even be secondary to a pathology. The clinical findings include acute kidney injury, thrombocytopenia and microangiopathic hemolytic anemia, more than half of the patients have watery diarrhea with dysentery, accompanied by other gastrointestinal symptoms such as abdominal pain, nausea and vomiting. Other manifestations that occur in the minority of patients are cardiological, neurological, ophthalmological and dermatological. For the diagnosis, it is first necessary to identify the Hemolytic Uremic Syndrome clinically and after that it is necessary to investigate the cause of it. Laboratory tests that are required for diagnosis include a blood count, kidney function tests, liver function tests, a general urinalysis and stool smear, and complementary tests to identify the cause. Treatment should be established as soon as possible in order to avoid or reduce acute complications and long-term sequelae, with supportive treatment being the essential management.
Keywords
References
2. Cody E, Dixon B. Hemolytic Uremic Syndrome. Pediatr Clin N Am [Internet]. 2019 [Consultado 3 marzo 2019]; 66(1):235-246. https://doi.org/10.1016/j.pcl.2018.09.011
3. Jenssen, G., Vold, L., Hovland, E., Bangstad, H-J., Nygård, K., Bjerre, A. Clinical features, therapeutic interventions and long-term aspects of hemolytic-uremic syndrome in Norwegian children: a nationwide retrospective study from 1999–2008. BMC Infectious Diseases [Internet]. 2016 [Consultado 1 marzo 2019]; 16:285. https://doi.org/10.1186/s12879-016-1627-7
4. Fakhouri F, Zuber J, Frémeaux-Bacchi V, Loirat C. Haemolytic uraemic síndrome. Seminar. 2017 Aug 12; 390(10095):681-696. https://doi.org/10.1016/S0140-6736(17)30062-4.
5. Lunn A, Forbes T. Haematuria and proteinuria in childhood. Paediatrics and Child Health. 2019 [Consultado 9 marzo 2019];28(8):315-321. https://doi.org/10.1016/j.paed.2012.02.011
6. Niaudet, P., Gillion, O. Overview of hemolytic uremic syndrome in children. UpToDate [Internet]. 2019 [Consultado 7 marzo 2019]. Disponible en: https://www.uptodate.com/contents/complement-mediated-hemolytic-uremic-syndrome
7. Dixon B, Gruppo R. Atypical Hemolytic Uremic Syndrome. Pediatr Clin N Am [Internet]. 2018 [Consultado 7 marzo 2019]; 65(3):509-525. Disponible en: https://doi.org./10.1016/j.pcl.2018..02.003
8. Kaur A., Kerecuk L. Haemolytic uraemic syndrome. Paediatrics and Child Health. 2016; 26(8): 344-348. https://doi.org/10.1016/j.paed.2012.01.002
9. Loirat C, Fakhouri F, Ariceta G, et al. An international consensus approach to the managementof atypical hemolytic uremic syndrome in children. Pediatr Nephrol [Internet]. 2016 [Consultado 6 marzo 2019]; 31(1):15-39. https://doi.org./10.1007/s00467-015-3076-8
10. Cubillos M, Salas P, Zambrano P. Microalbuminuria en pacientes pediátricos con diagnóstico de síndrome hemolítico urémico. Revista Chilena de Pediatría [Internet]. 2015 [Consultado 6 marzo 2019]; 86(2):92-96. http://dx.doi.org/10.1016/j.rchipe.2015.04.019
11. Brocklebank V, Johnson S, Sheerin TP, Marks SD, Gilbert RD, Tyerman K, et al. Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland. Kidney Int [Internet]. 2017 [Consultado 5 marzo 2019]; 92(5):1261–1271. https://doi.org/10.1016/j.kint.2017.04.028
12. Cavero T, Alonso M. Where are we with haemolytic uremic syndrome?. Med Clin (Barc) [Internet]. 2018 [Consultado el 10 de marzo 2019]; 151(8):329-335. http://dx.doi.org/10.1016/j.medcle.2018.08.010
13. Greenbaum L. Atypical Hemolytic Uremic Syndrome. Advances in Pediatrics [Internet]. 2014 [Consultado 8 marzo 2019;61(1):335-356. http://dx.doi.org/10.1016/j.yapd.2014.04.001
14. López M, Huete I, Hernández M. Compromiso cerebrovascular agudo en síndrome hemolítico urémico: descripción de dos casos pediátricos. Rev Chil pediatría [Internet]. 2017 [Consultado 8 marzo 2019]; 88(5):640–646. http://dx.doi.org/10.4067/S0370-41062017000500011
15. Spartà G, Nef S, Neuhaus TJ, Latal B, Buder K, Laube GF. Neurodevelopmental long-term outcome in children after hemolytic uremic syndrome. Pediatr Nephrol [Internet]. 2015 [Consultado 5 marzo 2019]; 30(3):503–513. https://doi.org./10.1007/s00467-014-2950-0
16. Rigamonti D, Simonetti GD. Direct cardiac involvement in childhood hemolytic-uremic syndrome: case report and review of the literature. Eur J Pediatr [Internet]. 2016 [Consultado 8 marzo 2019]; 175(12):1927–1931. https://doi.org/10.1007/s00431-016-2790-y
17. Niaudet P, Mattoo TK, Kim MS. Treatment and prognosis of Shiga toxin-producing Escherichia coli (STEC) hemolytic uremic syndrome (HUS) in children. Uptodate [Internet]. 2019 [Consultado 7 marzo 2019]. Disponible en: https://www.uptodate.com/contents/treatment-and-prognosis-of-shiga-toxin-producing-escherichia-coli-stec-hemolytic-uremic-syndrome-hus-in-children
18. Greenbaum L, Fila M, Ardissino G, Al-Akash S, Evans J, Henning P, et.al. Eculizumab is a safe and effective treatment inpediatric patients with atypical hemolytic uremic syndrome. Kidney International [Internet]. 2016 [Consultado 11 marzo 2019]; 89(3):701-711. http://dx.doi.org/10.1016/j.kint.2015.11.026
19. Niaudet P, Gillion O. Complement-mediated hemolytic uremic syndrome. Uptodate [Internet]. 2019 [Consultado 8 marzo 2019]. Disponible en: https://www.uptodate.com/contents/complement-mediated-hemolytic-uremic-syndrome