Assoc. Prof. Jakub Zieg, MD, PhD the Head of Pediatric Nephrology Working Group with inpatient and outpatient department, Pediatric Nephrology Unit, Department of Pediatrics, University Hospital Motol, Prague, Czech Republic
Thrombotic microangiopathy (TMA) is a group of diseases characterized by the presence of thrombocytopenia, microangiopathic haemolytic anaemia and organ involvement. The basis is endothelial damage to arterioles and capillaries leading to microvascular thrombosis. Hemolytic-uremic syndrome (HUS), which belongs to a large subgroup of TMA, is currently considered one of the most common causes of acute renal failure in childhood. Proper diagnosis of TMA and possible determination of the cause of HUS are essential to choosing the appropriate treatment. Progress in immunology, microbiology and molecular genetics have allowed us to better understand the pathophysiology of HUS. The treatment of HUS associated with Shiga-toxin infection producing E. coli (formerly typical) is symptomatic. The majority of children needs an elimination method and the prognosis is usually favourable. However, we rarely encounter a very severe course of the disease, sometimes with a fatal end. In the case of HUS associated with the activation of an alternative complement pathway (atypical HUS), the treatment includes a monoclonal antibody blocking complement activity, which has dramatically changed the prognosis of patients. The processed algorithm clearly and comprehensibly shows the issue of diagnosis and treatment of HUS. This interactive teaching aid significantly contributes to understanding the approach to this serious disease.
BLÁHOVÁ, Květa. Hemolyticko-uremický syndrom. Praha: Triton, 2000. ISBN 8072540947.
A Case of Escherichia coli Hemolytic Uremic Syndrome in a 10-Year-Old Male With Severe Neurologic Involvement Successfully Treated With Eculizumab [online]. J Investig Med High Impact Case Rep. [accessed 6.11.2017]. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5680941/#section2-2324709617741144title
Chapter XIII.5. Hemolytic Uremic Syndrome [online]. Alicia G. Turlington, MD [accessed October 2013]. Available at: https://www.hawaii.edu/medicine/pediatrics/pedtext/s13c05.html
Management of hemolytic uremic syndrome [online]. F1000Prime Rep. [accessed 1.12.2014]. Available at: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4278190/
An international consensus approach to the management of atypical hemolytic uremic syndrome in children. [online]. Loirat, C., Fakhouri, F., Ariceta, G. et al. Pediatr Nephrol 31, 15–39 [accessed 11.4.2015]. Available at: https://doi.org/10.1007/s00467-015-3076-8
The Sims™ 4, © 2017 Electronic Arts Inc., Update: 25. 2. 2021, PC: 220.127.116.110, Available at: https://www.ea.com/cs-cz/games/the-sims/the-sims-4/news/update-notes-feb-25-2021, Snapshots made 02/2021