Hemolytic Uremic Syndrome
1) Pediatric First Aid, Unit of Pediatrics, “Pugliese-Ciaccio” Hospital, Catanzaro, Italy
2) Unit of Pediatrics, “Pugliese-Ciaccio” Hospital, Catanzaro, Italy
3) Unit of Pediatrics, “Giovanni Paolo II “Hospital, Lamezia Terme, Italy
4) Department of Pediatrics, “San Matteo” University, Pavia, Italy
5) Department of Pediatrics Sapienza university of Rome, Pediatrics and Neonatology Unit, “Santa Maria Goretti” Hospital, Latina, Italy
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, non immune microangiopathic hemolytic anemia and acute renal failure.
HUS is typically classified into two primary types: HUS due to infections, often associated with diarrhea and HUS related to complement, also known as “atypical HUS”.
Early diagnosis and identification of underlying pathogenic mechanism allow instating specific support measures and therapies. Typical management relies on supportive care of electrolyte and water imbalance, anemia, hypertension and renal failure. Currently it is possible a new therapeutic approaches, first of all a monoclonal antibody that blocks the C5 cascade for aHUS.
shiga toxin, atypical hemolytic uremic syndrome, eculizumab, plasma therapy.
The 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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