What is Hunter’s syndrome?

What is Hunter’s Syndrome?

Hunter’s Syndrome is a  rare severe genetic disorder that affects only the male. It is also known as MPS II. Mucopolysaccharide refers to long chains of sugar and carbohydrates that occur within the cells and help build bone, cartilage, tendons, corneas, skin and other connective tissue. Under this condition, the nutrients are not broken down completely. Consequently, this causes harmful accumulation of GAGS thereby causing a chronic progressive disease.

Hunter’s syndrome affects 1 in 100,000 to 1 in 150,000 males, on an average.

Hunter's Syndrome
Source: Mahomet Citizen

Signs and symptoms

Not everyone is affected in the same way. Symptoms of this condition are not very clear or obvious at the time of birth, until eighteen months.

Hunter's Syndrome
Source: Trey Purcell

A child suffering from Hunter’s Syndrome will usually have:

-Slow growth

-Thick tough skin

-Thick lips

-Large tongue

-Prominent forehead

-Short or broad hands with curled fingers

In the beginning, the condition can be severe with the symptoms like Aggressive behaviour, Hyperactivity, intellectual disability, jerky body movements and degrading mental functions. However, in numerous cases, there may be mild or no mental deficiency.

Children with MPS II grow steadily until about age 5, and then their growth slows and they develop short stature.

The joint problem, numbness, weakness, hearing problem, increased hair growth, having a frequent cold, bowel problems, weak muscles, bone thinking, trouble talking or thinking are also few problems with a child suffering from Hunter’s Syndrome faces.

Treatment

Enzyme replacement therapy can help slow down the symptoms for a male child if the condition is not severe. It helps by replacing the protein their body does not produce. It also helps improve hair, facial features, muscle movement, breathing and walking.

Bone marrow or umbilical cord transplant can also be done if suggested by the doctor.
Medication and surgery can help with some of the complications and physical therapy can solve movement and joint problems.

Hunter's Syndrome
Source: Trey Purcell

Edited by Preetika Dubey 

 

Ashi Bajpai

Content writer

19, Gallivanter, Belives in equity and not equality. Love humans, Food, Allegories and Dogs. Currently pursuing BBA from BSSS. Believe in yourself and BE YOU. The world will adjust.

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