Review article
https://doi.org/10.21860/medflum2023_306341
Hemolytic Uremic Syndrome in Children
Petra Ptiček
orcid.org/0009-0002-7672-8834
; Zavod za hitnu medicinu DNŽ, Dubrovnik, Hrvatska
Ana Milardović
; Klinički bolnički centar Rijeka, Klinika za pedijatriju, Rijeka, Hrvatska
Abstract
Hemolytic uremic syndrome is a clinical syndrome characterized by simultaneous occurrence of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal injury, which is the main cause in children. A significant role in the pathogenesis of HUS plays an excessive activation of the complement system. According to the latest classification we distinguish hereditary and acquired HUS. Shiga toxin produced by E. coli (STEC) is responsible for about 90% of HUS cases, while about half of the other cases are caused by complement gene mutations. Hereditary HUS can also be associated with THBD, PLG and DGKE mutations or with a cobalamin C metabolism disorder. The group of acquired HUS, mediated by infections, also includes those caused by other microorganisms such as Streptococcus pneumoniae, Shigella dysenteriae, influenza virus and HIV. On the other hand, non-infectious causes of acquired HUS are adverse effects of medication, specific conditions such as autoimmune disorders, tissue and organ transplantation, and malignancies, as well as autoantibodies to complement factors. In addition to the urinary system, HUS can affect other organ systems. The diagnosis is clinical, and it is confirmed by laboratory tests. After it is established, the etiology must be determined. Therapeutic procedures are mostly supportive measures. Specific procedures used in the treatment of HUS mediated by complement disorders are eculizumab, plasmapheresis, fresh frozen plasma infusions, and kidney or combined kidney-liver transplantation.
Keywords
Acute Kidney Injury; Complement System Proteins; Hemolytic-Uremic Syndrome; Shiga-Toxigenic Escherichia coli
Hrčak ID:
306341
URI
Publication date:
1.9.2023.
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