The Atypical HUS Website
Welcome!
While Atypical HUS has some similaries to "Regular HUS or Ecoli HUS", Atypical HUS is quite different. We will show the differences between two types of HUS: Typical HUS and Atypical HUS. The information is presented in both common terminology and also in medical terminology
This page is dedicated to all of the children who are battling this disease. So little is known about the disease, and we hope to raise awareness and funds in order to find better treatments.
Atypical HUS
Atypical HUS (Hemolytic Uremic Syndrome) is not caused by an external agent (such as a bacteria, virus etc). Instead, some sort of internal chain of events sets the HUS off, and the syndrome becomes active.
The latest discoveries in Human Genetics have allowed Medical professionels to classify Atypical HUS into 3 major categories. The categories are a) Pneumococcus 2) Genetic (Complement system) and c) Others. It is believed that a majority of the cases are genetic in origin.
As of October 07, there were 4 genes associated with this disorder. They are Factor H, Factor I, Factor B and MCP. The first three genes are responsible for producing proteins that originate in the liver. The fourth, MCP, is not systhesized in the liver, but rather is a protecitve coating that lines the kidneys.
All four factors cause a problem in the complement system, which ends up damaging the endothelium. However, the first three proteins are 'free floating", and therefore the origin can be thought of as originating in the complement system. To be more specific, a dysfunctional level of a factor H, I or B protein fails to shut off the complement system (part of the immune system). This process is not evident until a "trigger" mechanism such as infection, cold, flu sets off a cascasding chain of events. Complement cascade then proceeds to injure microscopic blood vessels, (Microangeaopathic Hemolytic anemia), and thrombosis (clots) occur. The kidney is the organ that seems especially suseptible to these problems.
There are also a variety of other unknown causes, (ie ideopathic Atypical HUs).
In either case, Atypical HUS patients have many of the the same symptons as Typical HUS Patients.
However, there are quite a number of differences. While the Typical form of the disease may start off more severe, the Atypical form is subject to longer lingering effects, and is much more likely to become a chronic problem. Recurrance is much more common with the Atypical form of the disease.
Frequency of Atypical HUS
Atypical HUS is quite rare. The number of cases in the world is unknown. It does not seem to have any barriers geographically. In the United States, there is believed to be between 300 - 600 cases. Most cases occur in young children, although some adults may suddenly get hit with the disease. The disease is not contagious, therefore, there is no required reporting.
The email address below is for the Director of the Foundation, who is also a parent of a child afflicted by Atypical HUS.
