Neurological involvement in patients with STEC-Associated Hemolytic Uremic Syndrome (STEC-HUS): modern aspects of pathogenesis, clinical features and treatment modalities

Acute neurologic involvement is a life threatening and not rare (>20%) complication of the STEC-HUS in children and often determines prognosis and outcome of the disease. The most frequent neurologic signs are seizures (>70%), impaired consciousness (70-80%), coma (≈20%), paresis, oculomotor disturbances. The prognosis of typical HUS has improved over the last decades mainly due to the better general medical care resulting in lower mortality rate in pediatric STEC-HUS. More than 50% of children completely recovered from their neurological complication. This review reports current state of pathogenesis, clinical features and treatment approaches for typical STEC-HUS with neurological complications. A lot of data are concerned of the recent outbreak of HUS caused by shiga toxin producing E.coli O104:Н4, but clinical feature and short-term outcome in pediatric patients were similar to previous outbreaks of STEC-HUS. The treatment modalities in children were generally less aggressive than those in adult patients. Most of children with O104:H4 HUS (74%) recovered with supportive treatment alone, including those with neurological complications. Plasmapheresis was performed in 19% pediatric patients, mainly with neurological complications (n=16). Retrospective analysis of treatment strategies showed no significant short term benefit of eculizumab compared with patients with the same severity of HUS treated with plasmapheresis. IgG-immunoadsorption turned out to be unexpectedly effective in treating the severe neurological complications of HUS, but antibody-mediated theory of CNS injury in HUS is not yet proved. Current treatment approaches will undoubtedly allow one to treat typical HUS with severe complications in children more successfully.

STEC-ГУС, дети, неврологические осложнения, плазмаферез, экулизумаб, IgG-иммуноадсорбция, STEC-ГУС, children, neurological complications, plasmapheresis, eculizumab, IgG-immunoadsorption

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