Hemolytic uremic syndrome

Hemolytic uremic syndrome (HUS) is characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury.

Most cases of HUS occur in children and are diarrhea-associated (D+ HUS). Diarrhea-associated HUS is usually caused by Shiga toxin-producing Escherichia coli.

Rarely other organisms, such as Shigella and Streptococcus pneumoniae, are implicated.

Acute kidney injury necessitating dialysis develops in approximately half of children with diarrhea-associated HUS.

Adequate hydration is important to minimize renal damage in HUS associated with Shiga toxin-producing E coli infections. Avoidance of antibiotics, antimotility agents, and nonsteroidal anti-inflammatory drugs is advised. Cautious use of opioids is advised; there are insufficient data on the effect of opioids on the course of HUS.

Anemia can be treated with red cell transfusion. Platelet transfusions are generally avoided in the absence of active bleeding.

Atypical HUS can occur due to genetic or acquired abnormalities in the alternative complement regulatory pathway. HUS can also occur as a secondary phenomenon due to medications, cancer, and other systemic diseases.

Hemolytic uremic syndrome (HUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Ninety percent of HUS cases occur in the pediatric population, due to Shiga toxin-producing Escherichia coli (STEC).[1]Mead PS, Griffin PM. Escherichia coli O157:H7. Lancet. 1998 Oct 10;352(9135):1207-12. http://www.ncbi.nlm.nih.gov/pubmed/9777854?tool=bestpractice.com [2]Keir LS, Saleem MA. Current evidence for the role of complement in the pathogenesis of Shiga toxin haemolytic uraemic syndrome. Pediatr Nephrol. 2014 Oct;29(10):1895-902. http://www.ncbi.nlm.nih.gov/pubmed/23843163?tool=bestpractice.com Atypical HUS occurs due to abnormalities in the alternative complement regulatory pathway, resulting in endothelial cell damage and causing microvascular thrombosis. Atypical HUS can occur in adults, and in some children.