(Infected Related) Hemolytic Uremic Syndrome (HUS)

DISEASE DEFINITION

HUS is a thrombotic microangiopathy characterized by the presence of hemolytic anemia (destruction of red blood cells), low platelets, and organ damage due to the formation of microscopic blood clots in capillaries and small arteries, in this case especially acute kidney failure. Hemolytic uremic syndrome can lead to kidney failure, which can be life-threatening.


WHAT IS THE CAUSE?

When categorized as typical, HUS is caused by Shiga toxin–producing Escherichia coli (STEC) gastrointestianl infection. Atypical HUS (aHUS), is associated primarily with mutations or autoantibodies leading to dysregulated complement activation. Secondary HUS relates to a coexisting disease.

Invasive Streptococcus pneumoniae infection continues to be an uncommon but important cause of HUS in children. Most cases can be confirmed or at least considered probable without performing a renal biopsy.


SYMPTOMS

  • Bloody diarrhea, usually a few days earlier
  • Abdominal pain
  • Vomiting
  • Fatigue
  • Pallor
  • Reduced urine output
  • Unexplained bruising or bleeding

DIAGNOSIS

A diagnosis of STEC HUS should be considered in any patient developing a thrombotic microangiopathy, particularly if it follows a diarrheal illness and is associated with acute kidney failure. As atypical forms of HUS also may be associated with diarrhea, therefore STEC infection should be confirmed.

Stool culture

To identify the presence of Shiga toxin-producing E. coli. This may be laso negative, particularly if sample collection is delayed and diarrhea may have stopped by the time of presentation. In those cases STEC can still be cultured from feces or rectal swab. Samples should also be assessed for the presence of Shiga toxin.

Blood tests

To check for anemia, low platelet count, and kidney function.


TREATMENT

Supportive Care

Including fluid and electrolyte management and blood transfusions if necessary. The improved survival in patients with STEC HUS in recent years probably reflects better supportive care.

Avoiding Antibiotics

Antibiotics are generally not recommended as they may increase toxin release.