Hemolytic-Uremic Syndrome

Hemolytic-Uremic Syndrome: Description, Causes and Risk Factors: Hemolytic-Uremic SyndromeHemolytic-uremic syndrome (HUS) is a disorder that usually occurs when an infection in the digestive system produces toxic substances that destroy red blood cells. It often affects the kidneys. ICD-9-CM: 283.11. Abbreviation: HUS. This 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, as well as non-gastrointestinal infections. While the cause of HUS has not been established with certainty, researchers believe that an abnormal inflammation reaction is stimulated in the blood stream causing platelets to sludge in small blood vessels and form blood clots where they are not supposed to. This uses up the platelets and causes a shortage of them in the rest of the body. This abnormal reaction may be caused by a yet unidentified chemical that is produced in blood plasma (the fluid or non-cellular part of the bloodstream). HUS is most common in children. It was once very rare, but it is increasing in children. It is the most common cause of acute kidney failure in children. Other risk factors for HUS are unknown, although some cases are due to a familial form of the disease. HUS may occur with a variety of other diseases and infections, and after taking certain medications such as mitomycin C or ticlopidine. Hemolytic-Uremic Syndrome is more complicated in adults. It is similar to another disease called thrombotic thrombocytopenic purpura (TTP). Symptoms: HUS often begins with vomiting and diarrhea, which may be bloody. Within a week, the person may become weak and irritable. Persons with this condition may urinate far less than normal. Urine output may almost stop. Other Symptoms: Blood in the stools.
  • Vomiting and diarrhea.
  • Fever.
  • Irritability.
  • Lethargy.
  • Vomiting and diarrhea.
Later symptoms: Bruising.
  • Decreased consciousness.
  • Low urine output.
  • Paleness.
  • Seizures, rare.
  • Skin rash that looks like fine red spots (petechiae).
  • Yellow skin (jaundice).
Diagnosis: The healthcare provider will have a suspicion of the disease based on the history and physical examination. The health care provider will perform a physical exam. This may show: Liver or spleen swelling.
  • Nervous system changes.
Laboratory tests may include: Blood clotting tests (PT and PTT).
  • Chem-20 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.
  • Urinalysis may reveal blood and protein in the urine.
  • Urine protein test can be used to show the specific 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 Options: HUS in children tends to be self-limiting, and supportive care is often all that is needed. This may include intravenous fluids for rehydration and rebalancing of electrolytes like sodium and potassium, which can be lost with the diarrhea. Blood transfusion are only used for the most severe cases of anemia in which the hemoglobin falls below 6 or 7 g/dL (depending on age, the normal value is 11-16). Kidney failure can be managed expectantly (by observation and supportive care), and dialysis is not often required. Adults with Hemolytic-Uremic Syndrome tend to become more ill and need more aggressive therapy than children with the condition. In addition to the supportive care discussed above, plasmapheresis or plasma exchange may be required. Since it is thought there is an abnormal chemical in the plasma stimulating the abnormal clot formation, removing the plasma and replacing it with donor plasma is helpful in treating adult HUS. Temporary dialysis may be needed while awaiting recovery of the kidneys from the illness. Some people may have the liquid portion of their blood (plasma) removed and replaced with fresh (donated) plasma, or the plasma is filtered to remove antibodies from the blood. The vast majority of cases of Hemolytic-Uremic Syndrome are caused by poor food handling, and proper technique in the kitchen is the key to prevention. DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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