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Hemolytic Uremic Syndrome informationHemolytic Uremic Syndrome & Your Family

Unless you or someone in your family has a severe E. coli infection, it is unlikely that you would ever even hear about Hemolytic Uremic Syndrome (HUS). A rare condition that usually manifests itself as an unfortunate after-effect of a food borne illness, the syndrome does not touch many people's lives. However, because the potential harm from HUS is so great, it is important to have a basic knowledge of the condition.

Hemolytic Uremic Syndrome--pronounced HEE-mo-LIT-ik yoo-REE-mik syndrome--is an illness that most commonly occurs in the aftermath of other food borne illnesses. The illness usually affects children under the age of 10 but adolescents and adults can get HUS as well.

Hemolytic Uremic Syndrome is characterized by damage and destruction of the red blood cells, which leads to a lower than normal number of red blood cells (a condition called anemia), blood clots, and damage to blood vessel walls. In the most severe cases, HUS can include damage to the kidneys, which can lead to failure of the kidneys (renal failure). Other complications that can accompany the illness are thrombocytopenia (platelet deficiency in the blood) and neurologic signs, including irritability and, very rarely, seizures.

Most cases of Hemolytic Uremic Syndrome are preceded by gastrointestinal illness, often from a food borne germ, that causes bloody diarrhea. These cases of HUS are referred to as Typical HUS. The most common microorganisms associated with Typical HUS are Escherichia coli O157:H7 (E. coli) and Shigella, although it seems that nearly any infection that results in bloody diarrhea could lead to Hemolytic Uremic Syndrome. The majority of HUS cases result from E. coli contamination of food like meat, dairy products, and juice. According to some studies, as many as 15 percent of children who are infected with E. coli O157:H7 develop HUS.

Most patients with HUS experience complete recovery and nearly no risk of recurring symptoms. With careful and aggressive medical attention, the risk of fatality due to HUS is less than four percent. Up to 30 percent of the children who survive Hemolytic Uremic Syndrome, however, will be left with permanent damage to their kidneys. Children who recover usually do so quickly, while afflicted adults may experience longer recovery times since kidney damage is usually more extensive in adult HUS cases.

Recent studies show that Hemolytic Uremic Syndrome caused by E. coli infection is now the most common cause of renal failure for children in the U.S. It is recommended that people who recover from HUS undergo long term follow-up and observation to monitor for the onset of potential chronic kidney disease, hypertension (high blood pressure), and chronic neurologic damage.

Approximately 7,500 cases of Hemolytic Uremic Syndrome are diagnosed each year in the U.S.--similar to the rate of incidence for leukemia in the general population--but until all fifty states classify HUS as a reportable disease, an accurate rate of incidence will be impossible to determine.

Although much more rare, Atypical HUS, characterized by many of the same symptoms and risks that are common to Typical HUS, will sometimes follow the use of certain drugs, or follow pregnancy or cancer. Rather than being caused by an external agent--such as a food borne pathogen--these cases of HUS seem to be caused by some internal factor. One theory suggests that some people have a genetic weakness that means their bodies do not make a protein or enzyme which is crucial to resisting Hemolytic Uremic Syndrome. An important distinction between Typical and Atypical HUS concerns the severity and likelihood of recurrence of the symptoms. Although Typical HUS often starts with more severe symptoms, Atypical HUS often has lingering effects.



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