Severe Combined Immunodeficiency (SCID)

thuang8 By thuang8, 16th Mar 2012 | Follow this author | RSS Feed | Short URL http://nut.bz/16ck3hyx/
Posted in Wikinut>Health>General Health>Diseases & Infections

SCID: What is it? How do you get it? How to treat it, and how to prevent it.

What is Severe Combined Immunodeficiency?

Severe Combined Immunodeficiency is the result of the human immune system being compromised and not being able defend itself from infections. These infections can be combated by the human immune system in healthy individuals. This disease is commonly regarded as the "bubble-boy" syndrome. Afflicted individuals usually live in highly sterile conditions, because individuals usually do not survive past infancy without treatment.

How is SCID inherited?

Specifically, X-linked severe combined immunodeficiency (XSCID) is the terminology used for SCID that is inherited with mutation on the X chromosome. The mutated gene on the X chromosome is the IL2RG and is involved with creating surface receptor protein (gamma chain) on lymphocyte surfaces. This receptor is important in initiating the body's defenses against infection.

Males are more prone to inherit XSCID because if the X chromosome is a carrier of the mutation the Y chromosome does not have the gene ready to correct the mutation. Whereas, in females if one of the X chromosomes is a carrier and the other X chromosome is not then the healthy gene is able to correct the mutation.

How to treat it and prevent SCID?

SCID will be prominent in newborns due to the number of infections the newborn will contract. The best treatment is a bone marrow transplant in order to allow the afflicted individual to produce a continuous supply of healthy immune cells. The best way to get bone marrow is to allocate it from a sibling.

Gene therapy is an alternative method of replacing the IL2RG gene with a healthy copy. It proved to be successful until some patients having gone through the therapy developed leukemia. Although successfully "curing" SCID, it provided increased susceptibility to cancer. In this case, leukemia. Thus genet therapy has been suspended world wide, although efforts continue to make improvements on the therapy to better understand the treatment to prevent cancers to arise.

DNA sequencing can be used to detect the SCID gene, but if there is not history of the disorder in the family it is not cost effective since the disease itself is so rare.

Tags

Genetic, Genetic Disorder, Genetic Inheritance, Genetic Reasons, Genetics, Scid, Sever Combined Immunodeficiency

Meet the author

author avatar thuang8
I am a student of University of California, Merced. I have also studied at Hong Kong University. My major is Human Biology.

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