Hemolytic-uremic syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2] Parth Vikram Singh, MBBS[3]

Overview

The mainstay of therapy for hemolytic uremic syndrome (HUS) is supportive therapy. Antibiotic therapy is not recommended among patients with HUS. Hydration and intravenous (IV) isotonic saline replacment decrease the risk of hemolytic-uremic syndrome (HUS). Patients with HUS who have significant clinical bleeding or who require an invasive procedure should receive platelet transfusions.

Medical Therapy

• The mainstay of therapy for HUS is supportive therapy and almost all cases of HUS are self-limited. However any patient suspected of HUS should be referred to a kidney center or nephrology unit or be managed in an intensive care unit.
• Potentially harmful interventions should be avoided. Multiple studies have shown an association between antibiotic administration and increased risk of HUS among patients infected with high-risk STEC; therefore, empiric antibiotics should be avoided in immunocompetent patients with bloody diarrhea when STEC is suspected. Narcotics and antimotility drugs may prolong bloody diarrhea and have been associated with increased risk of HUS and neurologic complications. Nonsteroidal antiinflammatory drugs may precipitate acute kidney injury and should be avoided. A single oral dose of ondansetron may facilitate oral rehydration, but multiple-dose or intravenous ondansetron should not be routinely used.
• Antibiotic therapy is not recommended among patients with E. coli O157:H7 infection. This increases the risk of HUS. However there are some antibiotics that decrease the risk of HUS such as quinolones.
• Heparin, anti-platelet agents, immunoadsorption, thrombomodulin (rhTM).
• Fosfomycin should be started within 48-72 hours of the patient's presentation of HUS.
• Empiric antibiotic therapy such as cephalosporins for invasive pneumococcal disease should be used following culture results.
• Recombinant human soluble thrombomodulin (rTM) is a novel medicine that use to treat children with HUS.
• Eculizumab is used to treat atypical HUS.

HUS

• Acute kidney injury
  • First discontinue medicines that have renal toxicity.
  • Fluid replacement is the mainstay of treatment.
    • Early volume support is an important management principle in suspected high-risk STEC infection. Observational studies have associated intravenous isotonic fluids given before HUS is confirmed with reduced incidence of anuria and shorter hospital stay. In nonrandomized studies, infusion of 0.9% sodium chloride to target a weight 7 to 10% above presumed preillness weight, or 10 ml/kg/hour of isotonic crystalloid for 3 hours followed by maintenance isotonic fluids for 48 hours, was associated with reduced use of kidney-replacement therapy. Because fluid overload remains a concern, daily biochemical, hematologic, and clinical monitoring with fluids tailored to hydration status is a reasonable approach.
  • Maintain adequate hydration and electrolytes during infection by ample fluid therapy effects on short and long-term disease outcomes.
  • Indications for dialysis in children with HUS are similar to those in children with other forms of acute kidney injury (AKI).
    • More than 50% of patients with STEC-related HUS receive kidney-replacement therapy in many case series. Indications include severe electrolyte or acid-base imbalance, uremic symptoms, fluid overload not responsive to conservative measures, and the need to advance nutrition. Peritoneal dialysis is relatively safe and effective, whereas hemodialysis may be preferred when rapid fluid and solute removal are required. CRRTC-replacement therapy should be considered in children with hemodynamic instability and multiorgan dysfunction.
• Anemia
  • Transfuse packed red blood cellsls (RBC):
    • Hemoglobin <6 g/dL
    • Hematocrit < 18
  • Transfusion should be done slowly and patients should be obsereved closely.
  • Monitor the plasma potassium to prevent hyperkalemia.
  • Maintain a goal Hgb level after transfiution of between 8-9 g/dL.
  • Do not try to normalize the hemoglobin as it may lead to heart failure, pulmonary edema and hypertension due to volume overload.
  • Patients who undergo hemodialysis, transfusions should be given during dialysis to minimize the risks of hypervolemia and hyperkalemia.
• Thrombocytopenia
  • Patients with HUS who are noted to have significant clinical bleeding or who require an invasive procedure should receive platelet transfusions. Platelet transfusions should generally be limited to patients with hemodynamically significant bleeding, although platelets are usually given before insertion of a central venous catheter.
  • Studies have not shown benefit of eculizumab in STEC-associated HUS, and its use should be limited to clinical trials. Evidence supporting plasma exchange is extremely limited, and plasma exchange is not currently recommended.

• Hypertension
  • At the first indication of hypertension fluids should be restricted. Dialysis is more effective than diuretics.
  • Calcium channel blockers should be used as the initial choice of antihypertensive agents and after this phase antihypertensive therapy should be changed to ACE inhibitors in patients who appear to have long-term renal sequelae.

• Neurologic dysfunction
  • Patients with serious neurologic dysfunction should undergo brain imaging performed to assess CNS involvement.Seizures are treated with antiepileptic agents.