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As Sandhoff disease is a recessive disorder, males and females are affected with equal frequency. This disorder is more common in people with non-Jewish descent, unlike Tay-Sachs disease, which is prevalent mainly in individuals with Jewish ancestry.
Wiki User
∙ 13y ago
Wiki User
∙ 13y agois caused by mutations in two different genes that encode subunits that make up a protein called hexosaminidase
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What is the test for Sandhoff disease?
Because Sandhoff disease and Tay-Sachs disease have similar clinical symptoms, distinguishing them requires biochemical analysis. This involves a test to measure enzyme activity of the two hexosaminidase enzymes.
What is the life expectancy for Sandhoff disease?
The prognosis for Sandhoff disease is poor. Affected babies usually do not survive past the age of three and typically, death occurs due to complications associated with respiratory infections.
How is Sandhoff disease different from Tay-Sachs?
In Tay-Sachs disease, a mutation that affects the alpha subunit of the enzyme causes a deficiency in HexA. Sandhoff disease is caused by mutations that affect the beta subunit, rendering both the HexA and HexB enzymes deficient.
What are the symptoms of Sandhoff disease?
The symptoms begin with motor deficits (lack of normal movement) and a characteristic startle reaction to various sounds. Babies with Sandhoff disease progressively deteriorate in terms of motor function, and they often have seizures and myoclonus
What is the treatment for Sandhoff disease?
There is no cure for Sandhoff disease, and treatment is based on lessening the symptoms once they begin. Medication is usually given to reduce seizures, for example, and a feeding tube may be inserted to prevent aspiration of feedings into the lungs.
What are the physical characteristics of a child with Sandhoff?
A typical physical feature of Sandhoff disease is the presence of cherry-red spots in the back of the eyes. Additionally, affected children have an abnormally enlarged head and appear to have a doll-like appearance.
What is the average age of onset for Sandhoff disease?
At birth, infants tend to be without symptoms and usually do not develop them until approximately six months of age.
What is the most important risk factor for kidney disease?
Hypertension
Which characteristics is most closely associated with elevated risk of cardiovascular disease?
Hostility
What are some risk factors of lupus?
Risk factors for getting the disease or risk factors if you already have the disease?
What risk is associated with radiation and chemotherapy treatment for Hodgkin's disease?
The development of a second type of cancer is the most serious risk from radiation and chemotherapy treatment for Hodgkin's disease. In particular, there is a risk of developing leukemia, breast cancer, bone cancer
What's the most serious risk factors for heart disease?
There are many risk factors for heart disease. A family history of the disease should mean that the offspring be a bit more careful with their own cardiac health. Chances will increase with unhealthy living.
