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Eur J Pediatr ; 11 :Nov. We performed genetic analysis of 21 Czech children with atypical haemolytic uraemic syndrome.

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The incidence of atypical haemolytic uraemic syndrome in the Czech paediatric population was estimated to be 0. Ten patients were initially treated with plasma exchange and eight with eculizumab or with a combination of eculizumab and plasma exchange. At the last follow-up, 20 patients were alive and one patient had end-stage renal disease.

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Conclusion: The incidence of atypical haemolytic uraemic syndrome in the Czech paediatric population corresponds to the reported incidence in Europe. Treatment by eculizumab led to superior outcomes and prevention of the disease relapses compared with plasma exchange therapy.

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Our results may help to understand the polygenic nature of atypical haemolytic uraemic syndrome as a disease that results from a combination of various risk factors.

What is Known: • Atypical haemolytic uraemic syndrome aHUS is considered a polygenic and multifactorial disease.

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What is New: • Potentially causative genetic or acquired factors were confirmed in the majority of patients.

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