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Atypical Haemolytic Uraemic Syndrome

What is Atypical Haemolytic Uraemic Syndrome (aHUS) & Its Treatment?

By December 30, 2024No Comments

Atypical haemolytic uraemic syndrome (aHUS) is a very rare and life-threatening kidney condition. aHUS is a sort of condition in which small blood clots block the tiny blood vessels in the kidneys. This causes damage to the kidney’s filters and affects their capability to work properly.

The condition occurs because of abnormalities in the complement system. Complement is a part of our immune system that is crucial in combating infection. It does this by covering both foreign cells (such as viruses and bacteria) and our body’s own cells (including kidney cells), with special proteins called C3 and C4. These proteins help the immune system detect and respond to threats.

The body kills bacteria and viruses by damaging their cell walls. Our cells are generally protected from these proteins by special “protector proteins. These protector proteins keep the system under control. In aHUS, there are faults in the complement system which means the ‘protector proteins’ don’t work properly. As a result, clots form within the blood vessels within the filters of the kidney causing anemia, a reduction in platelets (a type of blood cell), and kidney failure.

Causes of Atypical Haemolytic Uraemic Syndrome (aHUS):
In most cases, aHUS occurs due to genetic fault, which is present at birth. But the condition can begin to affect the patient at any age because it is usually triggered by something such as:
  • pregnancy
  • a viral infection
  • certain medicines

If this rare condition is not treated promptly, it can be life-threatening and will generally cause end-stage kidney failure.

How does aHUS Differ from Typical HUS?

STEC-HUS, also known as typical haemolytic uraemic syndrome (HUS). It is a disease generally caused by a bacterial infection. This infection often comes from a gastrointestinal bug, like E. coli O157.
 
The main problem in STEC-HUS is caused by a substance called Shiga toxin. This toxin is produced by the E. coli bacteria. For this reason, the condition is also called STEC (Shiga-toxin producing E. coli)-HUS.
 
STEC-HUS can cause sudden kidney failure, which can be serious. However, in most cases, people make a full recovery. There are usually no long-term health problems, and the illness does not come back.
 
Symptoms of Atypical Haemolytic Uraemic Syndrome (aHUS):
The early signs of aHUS can feel like a persistent illness that doesn’t go away. Common symptoms include:
  • fatigue
  • weakness
  • reduced urine output
  • loss of appetite
  • swelling in the body
In case this condition is left untreated, it can lead to kidney failure and certain other complications.
Diagnosis of aHUS:
To diagnose HUS, doctors may suggest urgent blood tests. These tests check for anemia (low red blood cells), low platelets, and signs of kidney failure.
To confirm aHUS, additional tests are required. These tests rule out other probable types of HUS, such as STEC-HUS or other causes.
Complement system studies and genetic testing may also be done. These tests help identify problems in the complement system, which can make aHUS more likely.

Treatment of Atypical Haemolytic Uraemic Syndrome (aHUS):

Until recently, most patients with atypical hemolytic uremic syndrome (aHUS) had to spend many years on dialysis. For many, a kidney transplant was not an option. This is because the disease often comes back in the new kidney.
Now, there is a new therapeutic option known as eculizumab (Soliris). It helps control the complement system, which is overactive in aHUS.
Soliris (Eculizumab) can prevent kidney failure in patients who recently developed aHUS. It can also stop the disease from coming back in transplanted kidneys. This gives new hope to patients with aHUS.
Other Therapeutic Options:
Patients with atypical Hemolytic Uremic Syndrome (aHUS) may also need additional treatments. These may include:
  • dialysis for a short period (but some individuals may still need to remain on it in the longer term)
  • plasma exchange – where faulty proteins that contain plasma are removed from the body and replaced with new non-faulty proteins that contain plasma. Plasma is basically the main ingredient of blood. This clear, straw-colored liquid holds platelets, RBCs, and WBCs in suspension. Plasma exchange, like dialysis, usually takes a few hours to complete. A line will need to be inserted into one of your larger blood vessels for the procedure to take place.

Soliris (Eculizumab) can be made available in India through trusted importers. It is primarily used to treat rare conditions like aHUS and PNH. Indian Pharma Network (IPN) is a trusted importer of Soliris, ensuring access to genuine medicine.

You can buy Soliris online in India through authorized suppliers like the Indian Pharma Network. Always buy this therapeutic drug from verified sources that comply with legal and medical regulations to avoid counterfeit products.

Yes, Soliris can be made available in SAARC countries, including India, Nepal, Bangladesh, Bhutan, Sri Lanka, Maldives, Afghanistan, and Pakistan. It can be accessed through trusted suppliers like Indian Pharma Network (IPN) for patients needing specialized care.

Indian Pharma Network (IPN) is a leading importer of Soliris in India. We help patients readily access this life-saving therapeutic drug through a reliable and transparent process. We offer quick and safe delivery.

The price of Soliris (eculizumab) in India varies depending on the dosage, brand, supplier, and treatment duration. It is a high-cost imported medicine. Kindly Call/WhatsApp: +91 9310090915 to consult with us for competitive pricing and assistance programs.

Nitin Goswami

Nitin Goswami

Nitin Goswami joined us as an Editor in 2020. He covers all the updates in the field of Pharmaceutical, Business Healthcare, Health News, Medical News, and Pharma News.

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