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Typical Hemolytic Uremic Syndrome

Abstract

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NORD is very grateful to Bernard S. Kaplan, MB BCh, Division of Pediatric Nephrology, The Children's Hospital of Philadelphia, for assistance in the preparation of this report.

Synonyms of Typical Hemolytic Uremic Syndrome

  • classic hemolytic uremic syndrome
  • diarrhea-associated (D+) hemolytic uremic syndrome
  • shigatoxin-associated hemolytic uremic syndrome
  • Stx HUS [STEC HUS]
  • typical HUS

Disorder Subdivisions

  • No subdivisions found.

General Discussion

The hemolytic uremic syndrome is defined by the sudden occurrence of acute hemolytic anemia with fragmented red blood cells, low levels of platelets in the blood (thrombocytopenia), and acute kidney injury. Hemolytic uremic syndrome is a general term that covers three main subtypes STEC (typical), atypical hemolytic uremic syndrome, and Sp HUS (Streptococcal pneumonia associated HUS). This report covers STEC (typical) hemolytic uremic syndrome, which is most often associated with E. coli infection and bloody diarrhea. NORD has a separate report on the rarer atypical hemolytic uremic syndrome, which is not caused by infection with E. coli and is often the result of a genetic mutation.

Typical hemolytic uremic syndrome (HUS) is an uncommon disease that occurs in 5 to 15 percent of individuals, especially children, who are infected by the Escherichia coli (E. coli) bacterium, usually O157:H7. This organism releases toxins into the gut that are absorbed into the bloodstream and transported by white blood cells (leukocytes) to the kidneys. This results in acute renal injury. There may also be damage to the brain with seizures and even coma, the pancreas with pancreatitis and occasionally diabetes mellitus, and other organs.

Typical HUS mainly affects young children between one and 10 years. More recently large numbers of adults were affected by STEC HUS in Europe. The onset of HUS is preceded by an illness characterized by vomiting, abdominal pain, fever, and, usually, bloody diarrhea.

Organizations related to Typical Hemolytic Uremic Syndrome

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