Abstract

GHEZZI, Lidia et al. Use Of Eculizumab In Typical Hemolytic Uremic Syndrome: A Therapeutic Option In Severe Neurological Compromise. Two Cases Report. Rev. nefrol. dial. traspl. [online]. 2020, vol.40, n.1, pp.39-45. ISSN 2346-8548.

Hemolytic Uremic Syndrome is an endemic disease in Latin America. Argentina is one of the countries where most cases are reported, with a rate of ten cases per 100,000 children under five years old. It is the first cause of acute renal failure (ARF), and responsible for 9% of kidney transplants. This pathology is characterized by a classic triad: microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. The main etiological agent of HUS is the bacterium Shiga toxin-producing Escherichia coli. HUS has an acute mortality lower than 5 %.

There is evidence of the active role of the Shiga toxin in the activation of the complement by binding to factor H. Eculizumab is a monoclonal antibody which inhibits the formation of the membrane attack complex (C5b-9), given its great affinity for C5 of the complement cascade. Its infusion is approved to treat atypical HUS, posing its usefulness to treat severe typical HUS with acute neurological involvement as an alternative to inhibit the complement cascade and stop toxin damage.

We present two pediatric patients with SUH diagnosis with shiga toxin rescue; these patients, who showed severe neurological involvement, were treated with Eculizumab and had a favorable response.

Keywords : typical hemolytic uremic syndrome; eculizumab; anuria; renal replacement therapy; plasmapheresis; central nervous system.

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