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.