Treatment

• Avoid antibiotics and antimotility agents (may increase the likelihood of HUS in suspected infection with EHEC).

• Monitor and correct:

  • Fluid status abnormalities (for more information, see “Clinical assessment of volume status”)

  • Electrolyte disturbances

  • Acid-base disorders

  • Blood pressure (due to hypertension or septic shock)

  • RBC transfusions

• Antiepileptic drugs (e.g., diazepam, phenytoin) in patients with seizures

• Dialysis (as indicated for AKI): Up to 50% of HUS patients require dialysis.

• Plasma exchange therapy: only in refractory cases

• Eculizumab

  • Effective for the treatment of aHUS [5]

  • May be beneficial in HUS with neurological symptoms [6]

Post-diarrheal HUS is a nationally notifiable condition. [7]

Platelet transfusions should be administered with caution unless patients are bleeding or require an invasive procedure. Some studies suggest that they can exacerbate microangiopathy.

HUS can result in microthrombus formation and complications in various organs:

• CNS

  • Seizures

  • Paresis

  • Stroke

  • Coma

• GI tract

  • Hemorrhagic colitis

  • Bowel necrosis, perforation, stricture

  • Peritonitis

  • Intussusception

• Heart: ischemia and fluid overload

• Pancreas: transient or permanent diabetes mellitus

• Liver: hepatomegaly, transaminase elevations

• Kidney

  • Hypertension

  • Chronic kidney disease (CKD)

  • End-stage renal disease (ESRD)

The prognosis depends primarily on prompt initiation of treatment. Timely treatment can prevent acute complications (AKI, coma, and death) as well as progression to chronic renal failure.

• With treatment, the mortality rate of HUS is low: < 10%.

• Long-term renal sequelae occur in 35–55% of children with HUS.

• Atypical HUS has a less favorable prognosis and a higher risk of progressing to end-stage renal disease.