EpidemiologyThe STEC-HUS represents more than 90% of cases of HSU in children, mainly less than five years of age. The incidence of the disease is about 2-3 for 100,000 people with peak incidence in children under the five years of age (6.1 every 100000/year)1. Cattle are the main vectors of Shiga toxin, with the bacteria being present in the cattle intestine and feces. Infection in humans occurs following ingestion of contaminated undercooked meat, unpasteurized milk or milk products, water, fruits or vegetables3. The Non-STEC-HUS is a rare form and accounts for about 5-10% of all cases of SEU4. It occurs at any age, from the neonatal period to the adult age. Seventy percent of children have the first episode of the disease before the age of 2 years and approximately 25% before the age of 6 months3.
- Central nervous system, involvement occurring in 20–50% of children with HUS, with seizures, coma, stroke, hemiparesis, facial palsy, pyramidal or extrapyramidal syndromes, dysphasia, diplopia and cortical blindness.
- Gastrointestinal tract: possible manifestations include severe hemorrhagic colitis, bowel necrosis and perforation, rectal prolapse, peritonitis, and intussusceptions.
- Cardiac dysfunction, that may be due ad cardiac ischemia, detected by elevated levels of troponin 1, uremia, and high levels of liquid overload.
- Additionally, the pancreas, may be involved in acute phase, in less than 10% of patients with reduced glucose tolerance and transitional diabetes mellitus.
- Hepatomegaly and/or increased serum Transaminases are frequent findings.
- In addition to anemia and thrombocytopenia, leukocytosis is common.
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- Mele C, Remuzzi G, Noris M. Hemolytic uremic syndrome. Semin Immunopathol. 2014 Feb 14
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- Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS. Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatr Nephrol. 2013 Nov; 28(11):2097-105
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- Zuber J, Le Quintrec M, Krid S, Bertoye C, Gueutin V, Lahoche A et al. Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation. Am J Transplant. 2012 Dec; 12(12):3337-54.