Hemolytic uremic syndrome is a condition where blood vessels located inside of an individual's kidneys become inflamed and incur damage from blood clots due to red blood cell and platelet breakdown. An individual affected by hemolytic uremic syndrome may experience symptoms such as diarrhea, bloody stools, lethargy, fatigue, dehydration, uremia, easy bruising, vomiting, pale skin, abdominal pain, irritability, fever, hematuria, confusion, swollen face, swollen limbs, and seizures. Diagnosis of hemolytic uremic syndrome is made with a physical examination, blood testing, urine test, and stool sample test. Treatment for hemolytic uremic syndrome may include fluid replacement, blood transfusion, platelet transfusion, or plasma exchange.
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E. Coli Infection
Children younger than five years old who develop hemolytic uremic syndrome have it because of an infection by a bacteria called E. coli. E. coli refers to a family of bacteria that lives inside of the intestinal tract of healthy animals and humans that rarely ever causes a problem. However, some strains of the E. coli bacteria are known to cause loose, watery stools and produce a toxic substance called the Shiga toxin. The strains of E. coli that make this toxin are referred to as Shiga toxin-producing E. coli (STEC). An individual who has contracted an infection due to STEC can experience the invasion of the bloodstream by the Shiga toxin, which can then move around the body and cause damage to the small blood vessels. This blood vessel damage is what can lead to the development of hemolytic uremic syndrome. An individual may contract a Shiga toxin-producing E. coli infection after they consume undercooked, spoiled, or poorly processed foods. An individual may also contract a Shiga toxin-producing E. coli infection after being exposed to contaminated water.
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Hemolytic uremic syndrome can develop following other infections besides those caused by the E. coli bacteria. The virus that causes influenza can induce hemolytic uremic syndrome as well as the human immunodeficiency virus that leads to acquired immunodeficiency syndrome. An infection by herpesvirus 8, a virus commonly found in acquired immunodeficiency syndrome (AIDS) patients, can also induce hemolytic uremic syndrome. Infections caused by the colonization of the pneumococcal bacteria in the body have also been implicated in the development of hemolytic uremic syndrome in some individuals. The pneumococcal bacteria is what causes a patient to develop pneumonia, and hemolytic uremic syndrome is a rare complication of it. Approximately forty percent of all atypical hemolytic uremic syndrome cases are caused by the streptococcal pneumoniae bacteria. Around five percent of all cases of hemolytic uremic syndrome in children in the United States are the result of infections other than E. coli bacteria.
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In rare cases, an individual may develop hemolytic uremic syndrome as an adverse effect of certain medications. These medications are those used to treat certain types of cancer, like chemotherapy drugs. These drugs can damage the lining of small blood vessels and stop the synthesis of a molecule called prostacyclin, which is responsible for the inhibition of platelet activation. Because inappropriate platelet activation is typically mediated by prostacyclin in the body, drugs that stop the synthesis of prostacyclin can allow blood clots to develop in the bloodstream easier. Immunosuppressants are used to suppress the immune system in individuals who have an autoimmune disease or who have received an organ transplant. These types of medications are also known to cause rare cases of atypical hemolytic uremic syndrome. Other medications used to stop platelets from activating or sticking together include ticlopidine and quinine are known to induce hemolytic uremic syndrome, as well as some birth control pills.
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Complication Of Cancer Or Autoimmune Conditions
An uncommon cause of some cases of hemolytic uremic syndrome is when it is a complication of cancer or certain autoimmune conditions. The most common form of cancer known to induce hemolytic uremic syndrome is called mucin-producing adenocarcinoma. A mucin-producing adenocarcinoma is a type of cancer that occurs in the epithelial cells that line internal organs and the skin. This type of cancer produces heavily glycosylated proteins known to induce hemolytic uremic syndrome. The most common autoimmune conditions known to cause hemolytic uremic syndrome are collagen vascular disorders like systemic lupus erythematosus, antiphospholipid syndrome, and thrombotic thrombocytopenic purpura. Autoimmune conditions cause the production of autoantibodies that inappropriately attack certain proteins in the body encoded by complement genes. Between six and ten percent of cases of autoantibody precipitated hemolytic uremic syndrome are the result of the production of autoantibodies against Factor H. Hemolytic uremic syndrome caused by autoantibodies against other complement proteins have also been reported at a less prevalent rate.
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A rare type of hemolytic uremic syndrome referred to as atypical hemolytic uremic syndrome can affect an individual who has inherited a certain type of genetic mutation from their parents. Not everyone who has the gene from their parents develops atypical hemolytic uremic syndrome. However, a chronic health condition, certain infection, or the use of certain medications can be a trigger for the mutated gene to become activated and induce hemolytic uremic syndrome. Only three percent of all cases of hemolytic uremic syndrome are the result of inherited genetic mutations and are referred to as familial hemolytic uremic syndrome. Children affected by this form of hemolytic uremic syndrome inherit the genetic mutation in an autosomal recessive fashion, and adults who are affected inherit the mutation in an autosomal dominant fashion. The genetic mutation that causes familial hemolytic uremic syndrome is thought to be an abnormality in an individual's complement regulatory proteins. These complement regulatory proteins include factor H, factor I, C3, factor B, and CD46.