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.
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.
Usually there are no signs and symptoms of Krabbe disease appear during the first few months of life. They begin gradually and then progressively get worsen.
The condition occurs when a person inherits two copies of an altered gene, one copy from each parent. A gene gives a kind of blueprint for producing proteins. But when there is an error in this blueprint, the protein product may not work properly. In this condition, two mutated copies of a particular gene lead to little or no production of an enzyme called galactocerebrosidase.
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Read more on Krabbe Disease Causes and Risks.
Krabbe disease is a rare, inherited degenerative disorder of the central and peripheral nervous systems that destroys the myelin, a protective coating of nerve cells inside the brain and throughout the nervous system.read more
Prognosis of Krabbe disease may be significantly better for children who receive umbilical cord blood stem cells before the onset of the disease or during the early phase of bone marrow transplantation.read more