What is Progeria?

also known as Hutchinson-Gilford Progeria Syndrome (HGPS)

a rare, fatal genetic disorder that is characterized by an appearance of

accelerated aging in children.

Only 1 out of every 8 million people will get this disease.

For every one year that a child lives with progeria, it is the equivalent of living
10 years for the average normal human.

Although progeria is a genetic disorder, it is not usually passed down in

families since it is a specific mutation.

It is 100% fatal.

The first sign of progeria is scleroderma which is a skin condition which

indicates incapacity to thrive.

Progeria does not affect the normal motor and mental development of
children.

Diagnosis

The usual test for this condition is a genetic test that verifies LMNA
Mutation which will affirm the condition.

How are Progeria and ageing similar?

Children who suffer from Progeria are genetically susceptible to premature, progressive heart
disease. Nearly all Progeria patients die from heart disease. Heart disease is also one of the leading
causes of death globally. Children with Progeria commonly experience cardiovascular events, such as
hypertension (high blood pressure), stroke,angina, enlarged heart and heart failure - conditions
linked to aging.

What causes Progeria?

90% of children with Progeria have a mutation on the gene that encodes Lamin A, a protein that holds the
nucleus of the cell together. It is believed that the defective Lamin A protein makes the nucleus unstable.
This instability seems to lead to the process of premature aging among Progeria patients.