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Diseases and Conditions
Hemolytic uremic syndrome (HUS)
From MayoClinic.com
Special to CNN.com

Introduction

Hemolytic uremic syndrome (HUS) is an uncommon but serious condition that can cause life-threatening kidney failure, especially for children and older adults.

In children, most cases of hemolytic uremic syndrome develop after several days of diarrhea — often bloody — due to infection with a specific strain of Escherichia coli (E. coli) known as O157:H7. Adults also may develop hemolytic uremic syndrome after an E. coli infection, but more often the cause is obscure or even unknown.

E. coli refers to a group of bacteria normally found in the intestines of healthy humans and animals. Of the hundreds of types of E. coli, most are harmless. But a few strains of E. coli are responsible for serious food-borne infections, including those that can lead to hemolytic uremic syndrome. E. coli is most often found in unwashed produce or undercooked meat. E. coli may also be present in swimming pools or lakes contaminated with feces.

Simple precautions — such as washing and cooking foods adequately and avoiding unclean swimming areas — can help prevent infections that could lead to hemolytic uremic syndrome, especially for children and older adults.

Signs and symptoms

Signs and symptoms of hemolytic uremic syndrome may include:

  • Fever
  • Abdominal pain
  • Pale skin tone
  • Fatigue and irritability
  • Small, unexplained bruises or bleeding from the nose and mouth
  • Decreased urination
  • Swelling of the face, hands, feet or entire body

Sometimes neurological symptoms, such as seizures, develop as well.

Causes

In children, most cases of hemolytic uremic syndrome develop after infection with a specific strain of E. coli known as O157:H7.

It's important to remember that not all strains of disease-causing E. coli bacteria are created equal. For example, the strain of E. coli responsible for most cases of traveler's diarrhea often runs its course in just a few days. Even most people affected by the more virulent E. coli O157:H7 recover completely within five to 10 days. Progression to hemolytic uremic syndrome is uncommon.

It's also possible for hemolytic uremic syndrome in children to follow infection with other types of bacteria, including shigella, salmonella, yersinia and campylobacter.

In adults, hemolytic uremic syndrome is more likely to be caused by an autoimmune disease, a blood infection or the use of certain medications. More often, however, the cause of hemolytic uremic syndrome in adults is obscure or even unknown.

Risk factors

Hemolytic uremic syndrome is most common in children ages 6 months to 4 years. People who have immature or weak immune systems — such as young children and older adults — have the highest risk of developing hemolytic uremic syndrome after an E. coli infection.

When to seek medical advice

Call your doctor immediately if you or your child experiences unexplained bruises, unusual bleeding, swollen limbs, extreme fatigue or decreased urine output after several days of diarrhea. Seek emergency care if you or your child goes 12 hours without urinating.

Screening and diagnosis

The doctor will ask questions about your signs and symptoms or your child's signs and symptoms, including the diarrhea. If the doctor suspects hemolytic uremic syndrome, various lab tests may be done to confirm the diagnosis. Blood tests may reveal a low platelet count or low red blood cell count. Other lab tests may reveal blood in your urine and a higher than normal level of creatinine — a breakdown product of creatine, an important part of muscle — in your blood.

Complications

About 50 percent of people who develop hemolytic uremic syndrome experience sudden kidney failure. In fact, hemolytic uremic syndrome is the most common cause of sudden (acute) kidney failure in children. High blood pressure, chronic kidney failure, heart problems, stroke and coma are possible as well.

Treatment

Hemolytic uremic syndrome requires careful management in the hospital. To ease immediate signs and symptoms and prevent further problems, hemolytic uremic syndrome treatment may include:

  • Red blood cell transfusions. If you don't have enough red blood cells, you may feel chilled, fatigued and short of breath. You may have a rapid heart rate, yellow skin and dark urine. Red blood cell transfusions, given through an intravenous (IV) needle, may help reverse these signs and symptoms.
  • Platelet transfusions. If you're bleeding or bruising easily, platelet transfusions can help your blood to clot. Like red blood cell transfusions, platelet transfusions are given through an IV needle.
  • Plasma exchange. Plasma is the part of blood that supports the circulation of blood cells and platelets. Sometimes a machine is used to clear the blood of its own plasma and replace it with fresh or frozen donor plasma.
  • Kidney dialysis. Sometimes dialysis is needed to filter waste and excess fluid from the blood. Dialysis is usually a temporary treatment until the kidneys begin functioning normally again. If the kidney damage is significant, however, permanent kidney failure — requiring long-term dialysis or a kidney transplant — is possible.

Despite the severity of the condition, appropriate treatment leads to a full recovery for most people with hemolytic uremic syndrome — especially young children.

Prevention

Specific preventive measures for hemolytic uremic syndrome aren't clear. However, it's always a good idea to protect yourself — and your children — from E. coli infection:

  • Wash your hands, utensils and food surfaces often.
  • Keep raw foods separate from ready-to-eat foods.
  • Thoroughly cook ground beef to at least 160 F. (Hamburgers should be well-done.)
  • Wash fruits and vegetables under running water.
  • Avoid unpasteurized milk, juice and cider.
  • Avoid swimming in water potentially contaminated with feces.

Also make sure that everyone in your family — including children — washes his or her hands after using the toilet or changing diapers and before eating. In child care facilities, diapers shouldn't be changed or disposed of in the same room where food is prepared or eaten.

December 20, 2006

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