What are symptoms of aHUS?

Often, people with aHUS will report a vague feeling of illness, with non-specific symptoms that may include paleness, nausea, vomiting, fatigue, drowsiness, high blood pressure, and swelling. There are three hallmark symptoms that define aHUS: hemolytic anemia, thrombocytopenia, and kidney failure.

What causes aHUS disease?

What causes aHUS? In most cases, aHUS is related to gene mutations, coupled with a triggering event. Just having a gene mutation alone usually will not cause the disease. You need a gene mutation and a triggering event for a flare-up to start.

What is the difference between HUS and aHUS?

Typical HUS (ie, STEC-HUS) follows a gastrointestinal infection with STEC, whereas aHUS is associated primarily with mutations or autoantibodies leading to dysregulated complement activation.

Is there a cure for aHUS?

Atypical hemolytic uremic syndrome (aHUS) is a disease that causes blood clots in small blood vessels in your kidneys and other organs. These clots keep blood from getting to your kidneys, which can lead to serious medical problems, including kidney failure. There’s no cure, but treatment can help manage the condition.

Is aHUS an autoimmune disease?

Atypical hemolytic uremic syndrome (aHUS) can co-exist with autoimmune disorders, complicating the diagnosis of the rare blood-clotting disease, a case report shows. The findings highlight the need for genetic testing to diagnose aHUS in complex cases, the researchers said.

How long can you live with aHUS?

Dialysis. Patients with aHUS who have ESRD are generally consigned to lifelong dialysis, which carries a 5-year survival rate of 34–38%, with infections accounting for 14% of deaths. These patients also remain at ongoing risk of non-kidney systemic complications of the disease.

Is aHUS a blood disorder?

Atypical hemolytic uremic syndrome (aHUS) is an extremely rare disease characterized by low levels of circulating red blood cells due to their destruction (hemolytic anemia), low platelet count (thrombocytopenia) due to their consumption and inability of the kidneys to process waste products from the blood and excrete …

How common is atypical HUS?

The incidence of atypical hemolytic-uremic syndrome is estimated to be 1 in 500,000 people per year in the United States. The atypical form is probably about 10 times less common than the typical form.

How do you diagnose atypical HUS?

Diagnosing aHUS is complicated by the fact that it is more difficult to establish without a family history of the disorder. The diagnostic criteria associated with aHUS are hemolytic anemia (anemia in the presence of broken red blood cells), low platelet count (thrombocytopenia) and kidney dysfunction.

How rare is atypical hemolytic uremic syndrome?

What are the main causes of the hemolytic uremic syndrome?

Hemolytic uremic syndrome (HUS) is a complex condition where an immune reaction, most commonly after a gastrointestinal tract infection, causes low red blood cell levels, low platelet levels, and kidney injury. Infections of the gastrointestinal tract (your stomach and intestines) are the most common cause of this syndrome.

How do I treat atypical HUS?

This uncommon type of HUS is generally treated with plasma exchange. Your doctor might also recommend a medication – eculizumab (Soliris) – that can prevent the continued destruction of healthy cells. To prevent serious infection, you or your child might have a meningococcal vaccine before receiving this medication.

What is aHUS disease?

Atypical hemolytic uremic syndrome (aHUS) is an extremely rare disease characterized by low levels of circulating red blood cells due to their destruction ( hemolytic anemia ), low platelet count (thrombocytopenia) due to their consumption and inability of the kidneys to process waste products from the blood and excrete them into the urine (acute