What Is The Overall Prognosis For Hunter Syndrome?

What is Hunter Syndrome?

Hunter Syndrome is an extremely rare inherited medical condition in which different vital organs of the body and the physical stature of an individual is affected.(1, 4)

Hunter Syndrome is caused when a defective gene results in malfunction or in some cases complete absence of enzyme iduronate 2-sulfatase.(2) This enzyme is necessary for breakdown of complex molecular structures because of which various metabolic processes and vital organs of the body function. Malfunction of this enzyme results in build-up of these molecular structures, which over time start to inflict heavy damage to the various organ systems of the body.

The organs which are most affected by Hunter Syndrome are brain, bones and other vital organs of the body. This leads to significant change of facial expression, growth retardation, organ failure, and physical capabilities of the child. Hunter Syndrome is commonly seen in boys and very rarely in girls.(1) Hunter Syndrome is a part of a group of inherited metabolic conditions named as mucopolysaccharidosis.(1)

As of now, there is no cure for Hunter Syndrome and treatment generally focuses on preventing further worsening of this disease and improving the quality of life of the patient.(3, 4) Hunter Syndrome is an X-linked recessive disorder in which the gene responsible for Hunter Syndrome is inherited from the mother. Detailed below is the overall prognosis or life expectancy of a child with Hunter Syndrome.

What is the Overall Prognosis for Hunter Syndrome?

When it comes to Hunter Syndrome, there are basically two classes of this condition. One is the milder form of Hunter Syndrome, with very few if any symptoms and the other one is the most severe form of Hunter Syndrome with significant symptoms.(1) Both the types of Hunter Syndrome affect different organ systems of the body and tissue structures.

However, the ones with the most severe form tend to have a rapid decline in intellectual functions as Hunter Syndrome progresses.(1) Hunter Syndrome progresses at a much faster rate in these children than the ones with the mild form of this disorder. There is significant loss of whatever growth and development they have achieved within a short span of time.

The symptoms of Hunter Syndrome can be observed starting age 3 and by the time the child has reached 8th year of life, there is significant decline in their overall health status. All in all, the overall prognosis of children with severe form of Hunter Syndrome is about 10-20 years. Children with milder forms of this condition also have limited chances of surviving past the third decade of their life, although throughout their life they tend to have normal intelligence unlike in children with severe form of this disorder.(1)

Cardiovascular and respiratory complications are the primary cause as to why these children succumb to this condition irrespective of the type of Hunter Syndrome they have.

Conclusion

In conclusion, overall Hunter Syndrome doesn’t have a good prognosis. Hunter Syndrome is a rare inherited medical condition in which an enzyme that breaks down complex molecular structures needed for metabolic processes and smooth functioning of various vital organs of the body is either missing or dysfunctional. Hunter Syndrome is an autosomal dominant condition and the defective gene is inherited from the mother.

There are basically two forms of Hunter Syndrome of which one is mild and the other one is severe. The overall prognosis of both forms of Hunter Syndrome is rather poor. However, children with severe form of Hunter Syndrome tend to have a rapid decline in their health and eventually succumb to this condition by the time they are 10 years of age. Children with milder forms of Hunter Syndrome tend to live well into their adulthood; however, even they have a short lifespan due to Hunter Syndrome. There is no cure for Hunter Syndrome and thus children with this condition need a lot of support and help from the family to cope up with the illness and its inherent complications to improve quality of life.

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