Who is at a Risk of developing Krabbe Disease?

By  ,  Onlymyhealth editorial team
Dec 17, 2014
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Quick Bites

  • Protective coating of nerve gets destroyed.
  • Signs develop in infants usually.
  • The condition can be fatal
  • Gene alteration causes the condition.

What is Krabbe Disease?

An inherited condition that destroys the protective coating of nerve cells present in the brain and in the entire nervous system is known as Krabbe disease. In majority cases, the signs and symptoms of Krabbe diseases develop in infants before 6 months of age and the condition usually causes death by the age 2. When the condition develops in older children and adults, its course varies largely.

Krabbe Disease Risks

Although there has been no cure for Krabbe disease, the treatment is focused on giving some supportive care. However, the stem cell transplants have shown some success in infants who undergo a treatment before the onset of symptoms and in some older children and adults.

Who is at Risk of Developing Krabbe Disease?

The gene mutation which is linked with Krabbe disease only causes the disease when two mutated copies of the gene are inherited. When a disease results from two mutated copies it is called an autosomal recessive disorder. If each parent has one mutated copy of the gene, the child’s risk will be as follows:

Krabbe Disease Risks

  • 25 percent chance of inheriting two mutated copies, resulting into the disease
  • 50 percent chance of inheriting only one mutated copy, which means that the child will carry the mutation but it will not result in the disease itself
  • 25 percent chance of inheriting two normal copies of the gene

Image courtesy: Getty Images

Read more on Krabbe Disease Causes and Risks.

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