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Hemolytic-uremic syndrome

HUS; STEC-HUS

 

Shiga-like toxin producing E coli hemolytic-uremic syndrome (STEC-HUS) is a disorder that most often occurs when an infection in the digestive system produces toxic substances. These substances destroy red blood cells and cause kidney injury.

Causes

 

Hemolytic-uremic syndrome (HUS) often occurs after a gastrointestinal infection with E coli bacteria (Escherichia coli O157:H7). However, the condition has also been linked to other gastrointestinal infections, including shigella and salmonella. It has also been linked to nongastrointestinal infections.

HUS is most common in children. It is the most common cause of acute kidney failure in children. Several large outbreaks have been linked to undercooked hamburger meat contaminated with E coli.

STEC-HUS is not to be confused with atypical HUS (aHUS) which is not infection-related. It is similar to another disease called thrombotic thrombocytopenic purpura (TTP).

 

Symptoms

 

STEC-HUS often begins with vomiting and diarrhea, which may be bloody. Within a week, the person may become weak and irritable. People with this condition may urinate less than normal. Urine output may almost stop.

Red blood cell destruction leads to symptoms of anemia.

Early symptoms:

  • Blood in the stools
  • Irritability
  • Fever
  • Lethargy
  • Vomiting and diarrhea
  • Weakness

Later symptoms:

  • Bruising
  • Decreased consciousness
  • Low urine output
  • No urine output
  • Pallor
  • Seizures -- rare
  • Skin rash that looks like fine red spots (petechiae)
  • Yellow skin (jaundice)

 

Exams and Tests

 

The health care provider will perform a physical exam. This may show:

  • Liver or spleen swelling
  • Nervous system changes

Laboratory tests will show signs of hemolytic anemia and acute renal failure. Tests may include:

  • Blood clotting tests (PT and PTT)
  • Comprehensive metabolic panel may show increased levels of BUN and creatinine
  • Complete blood count (CBC) may show increased white blood cell count and decreased red blood cell count
  • Platelet count is usually reduced
  • Urinalysis may reveal blood and protein in the urine
  • Urine protein test can show the amount of protein in the urine

Other tests:

  • Kidney biopsy
  • Stool culture may be positive for a certain type of E coli bacteria or other bacteria

 

Treatment

 

Treatment may involve:

  • Dialysis
  • Medicines such as corticosteroids
  • Transfusions of packed red blood cells and platelets

 

Outlook (Prognosis)

 

This is a serious illness in both children and adults, and it can cause death. With proper treatment, more than half of people will recover. The outcome is better in children than adults.

 

Possible Complications

 

Complications may include:

  • Blood clotting problems
  • Hemolytic anemia
  • Kidney failure
  • Nervous system problems
  • Too few platelets (thrombocytopenia)
  • Uremia

 

When to Contact a Medical Professional

 

Call your provider if you develop symptoms of HUS. Emergency symptoms include:

  • Blood in the stool
  • No urination
  • Reduced alertness (consciousness)

Call your provider if you have had an episode of HUS and your urine output decreases, or you develop other new symptoms.

 

Prevention

 

You can prevent the known cause, E coli, by cooking hamburger and other meats well. You should also avoid contact with unclean water.

 

 

References

Abrams CS. Thrombocytopenia. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 172.

Ruggenenti R, Cravedi P, Remuzzi G. Microvascular and macrovascular diseases of the kidney. In: Skorecki K, Chertow GM, Marsden PA, Taal MW, eds. Brenner and Rector's The Kidney. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 35.

Van Why SK, Avner ED. Hemolytic-uremic syndrome. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 518.

 
  • Male urinary system

    Male urinary system - illustration

    The urinary system is made up of the kidneys, ureters, urethra and bladder.

    Male urinary system

    illustration

    • Male urinary system

      Male urinary system - illustration

      The urinary system is made up of the kidneys, ureters, urethra and bladder.

      Male urinary system

      illustration

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          Review Date: 2/1/2016

          Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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