The Atypical HUS Foundation


courtesy of Bill Biermann

In most cases, Atypical HUS does not begin with a violent illness. Instead, the child starts off ill, fatigue, irritable, and lethargic to a point where hospitalization is needed. Supportive treatment is absolutely necessary in order to prevent further damage.
Typical HUS cases begin rather violently, with a severe bout of gastroenteritis that may be accompanied by bloody diarrhea.
Thereafter, symptoms of both typical and Atypical HUS may be similar. HUS causes clotting, and therefore results in vascular enlargement. Clotting tends to affect the kidneys, and may result in acute kidney failure, requiring dialysis or kidney tranplants.
A wide variety of symptoms can occur. Life threatening intestinal problems may occur. Neurological problems such as seizures, blindness and coma could develop. Profound intestinal or neurological diseases are indicative of a more severe HUS, and have poor prognosis. Nearly 5% of children will die during the acute phase of the disease as a result of renal failure or multiple organ complications. However, one the acute phase of the disease passes, most patients will recover eventually in full.
However, a small percentage of HUS patients are diagnosed with Atypical HUS. This group is especially proned to recurrences of the disease and are much more likely to develop chronic renal failure and other complications such as chronic high blood pressure. Atypical patients can have HUS episodes set off by routine colds and infections.

The initial symptoms may come gradually, and may differ from case to case. In most cases, Atypical HUS does not begin with a violent illness. Instead, the child starts off ill, fatigue, irritable, and lethargic often with fever. Usually, loss of appetite also occurs, Hospitalization usually occurs at this point.

Once hospitalized, the disease is usually identified through a routine blood exam. The blood exam will show:
1) Reduced platelet counts
2) Reduced Red Blood cell counts
3) Shattered red blood cells (this is called Shistocytes)

Children with Atypical HUS are much more likely to develop complications. These complications may be:
• Recurrance
• Kidney failure
• Seizures/Neurological problems
• No response to supportive treatments
• Return of HUS even after s kidney transplant occurs
• Severe Hypertension (high blood pressure)

To understand why these symptoms occur, we first must understand more about the disease.

To understand atypical HUS you first have to understand the role of blood and vascular system as well as the kidneys and how they work together.

  • The average adult has about five liters of blood, the average child less, living inside of their body, coursing through their vessels, delivering essential elements, and removing harmful wastes.
  • Blood is the fluid of life and growth, transporting oxygen from the lungs to body tissue and carbon dioxide from body tissue to the lungs, transporting nourishment from digestion and hormones from glands throughout the body. Blood is the fluid of health, transporting disease fighting substances to the tissue and waste to the kidneys.
  • Red blood cells and white blood cells are responsible for nourishing and cleansing the body. Since the cells are alive, they too need nourishment. Vitamins and minerals keep the blood healthy. The blood cells have a definite life cycle, just as all living organisms do.
  • Approximately 55 percent of blood is plasma, a straw-colored clear liquid. The liquid plasma carries the solid cells and the platelets which help blood clot. Without blood platelets, you would bleed to death.
  • The kidneys play an important roll in making sure we have enough red blood cells and in removing toxins, waste products of metabolism, from the body.
  • Factor H, is a protein found in the plasma which protects the endothelium tissue located in the lining of your blood vessels of the kidney. A factor H deficiency can resulting in damage to the endothelium. This triggers a cascade of events that result in platelets aggregating in the endothelium, clots which block arterioles and capillaries of the kidney. The platelets clump together (aggregate) and cause blood clotting and low platelet counts also called consumptive thrombocytopenia.



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