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Progeria

General informations
Progeria is an extremely rare disease
that makes children look like oldies. Progeria
is a noun used to describe many diseases
that cause premature aging, but is usually
used to describe the Hutchinson-Gliford
progeria syndrome (HGPS ). Those born with
progeria typically live from their mid-teens to
early twenties.
Cause
Is a genetic condition caused by a mutation that affect the LMNA gene which
codes for a structural protein called prelamin A. The prelamin A suffers some
transformation and turns into a protein named Lamin A, which along with the proteins
called Lamin B and Lamin C makes up he nuclear lamina. Nuclear lamina is an important
component of the nuclear envelope which provides structural support to the nucleus.
The nuclear lamina of the people with progeria provides less support and takes
abnormal shapes which makes cellular division hard to take place. This disease is rarely
inherited because people with progeria usually do not live enough to reproduce
children.
Symptoms
People who are born with progeria have small bodies, growth delay, small faces
with a head bigger than the normal proportion for their bodies, and the aspect of an old
person.
Along with those symptoms, people with progeria may manifest hair loss, heart
disease, stroke, hip dislocation, muscular sclerosis and many others.
People who dies from progeria, do not die because of the disease itself, but
because of the heart conditions that appears as they age.
Treatment
Unfortunately there is no cure for progeria , but occupational and physical
therapy can help those people to keep moving.
Hearth health is critical for people with progeria, so the doctor may prescribe
some medicines to prevent heart failure.
Mental health is very important for those people, many of them are anxious or
depressive due to the fact that is hard for them to establish interactions with other
people. Most of the people with progeria are marginalized and considered to be freaks
due to their appearance.
Progeria was a source of
inspiration for some movies
including “The curious case of
Benjamin Button” form 2008.
Even if the movie is a little
unrealistic because people with
progeria do not get younger as
they age, this movie describes
exactly the life of a kid with
progeria and the hardships of
his life.

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