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Hemolytic-Uremic Syndrome in Children
Olivia Boyer1, Patrick Niaudet2
1Pediatric Nephrology, Necker Enfants Malades Hospital, Université Paris Cité, France; Néphrologie Pédiatrique, Hôpital Necker, 149 Rue de Sèvres, Paris 75015, France.
Abstract:
Hemolytic uremic syndrome is characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. Most cases are caused by Shiga-toxin-producing bacteria, especially Escherichia coli. Transmission occurs through ground beef and unpasteurized milk. STEC-HUS is the main cause of acute renal failure in children. Management remains supportive. Immediate outcome is most often. Atypical HUS represents about 5% of cases, has a relapsing course with more than half of the patients progressing to end-stage kidney failure. Most cases are due to variants in complement regulators of the alternative pathway. Complement inhibitors, such as eculizumab, have considerably improved the prognosis.
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