ADA

ADA
  • Adenosine Deaminase Deficiency(ADA) is an Autosomal Recessive Disorder that affects the immune system.
    external image Autosomal%20Recessive.jpg
  • ADA deficiency is the first known cause of a condition known as severe combined immunodeficiency (SCID).
  • Without Adenosine deaminase(enzyme), the body is unable to break down toxic substance called deoxyadenosine.
  • This diease is caused by a mutation in a gene on chromosome 20.
  • The gene codes for the enzyme adenosine deaminase (ADA)ada.jpg

Symptoms






  • Susceptible to all kinds of infections
  • Including the skin, respiratory system and gastrointestinal tract
  • Sadly, most babies who are born with this disorder die within a few months
  • Pneumonia
  • Chronic diarrhea
  • Widespread skin rashes
  • Slow growth compared to that of a healthy children
Treatment






  • Agressive antibiotic thearpy for opportunistic infections
  • There are no real cures for ADA deficiency but doctors have tried to restore ADA levels and improve the immune system through various treatments like:
  • Bone marrow transplantaton
  • Transfusion of red blood cells
  • Enzyme replacement therapy
  • Gene Therapy
external image Ashanti.jpg
  • In the video below, Students at UEL are working on an ADA gene,
http://www.youtube.com/watch?v=e1BTDUuat1s

Facts

external image 180px-653px-SEM_Lymphocyte.jpg

  • This is an electron microscope image of a human white blood cell(lymphocyte). In adenosine deaminase deficiency, the white blood cells of the immune system are killed such as the one above.
  • ADA deficiency is very rare. It is estimated to occur in 1 in 200,000 to 1,000,000 newborns worldwide. This disorder is responsible for approx. 15% of SCID cases.
  • In 1990, a young girl with ADA deficiency, named Ashanti de Silva, received the first successful gene therapy from doctors William French Anderson, Michael Biase, and Ken Culver.


cv-8F-BFS: Well, since nobody wants to comment on this one, I will. I think this page is fairly easy to understand, it could have used some more PIZAZZ!!! but over all good work!
cv-8f-ctk: this page has alot of great info, but it is a little boring. it could use a little bit more color...like other than red! ha. other than that, great job!


Bibliography