Progeria, also known as Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by an appearance of accelerated aging in kids.
Those kids born with the condition typically live to their mid-teens the to early twenties. The genetic condition is a form of mutation in the gene called LMNA that is rarely inherited as carriers rarely survive longer to reproduce.
Although kids born with such syndrome looks healthy earlier, they soon begin to display characteristics within the first two years of their life.
What is Progeria? Know About Its Prevalence
Progeria is very rare. It is a genetic condition that causes a child's body to age fast. The disease affects both sexes equally and in all races. It affects about 1 in every 4 million births worldwide.
It is estimated that there are between 200-250 children living with the issue.
Children with progeria die of atherosclerosis (heart disease) at an average age of about 14 years.
The disease is sometimes called "Benjamin Button disease" after Scott Fitzgerald's fictional character.
American actor Brad Pitt starred as a man who ages in reverse in the movie " The Curious Case of Benjamin Button" whereas people with progeria age forward and quickly.
Signs and Symptoms
Children with the genetic defect generally develop the first sign during their first few months of life.
Early signs and symptoms include a failure to thrive and a localized scleroderma-like skin issue. The symptoms become more severe and visible as the child ages.
Babies with progeria do not grow or gain weight as normal babies do. Some physical traits include:
- A bigger head
- Large eyes
- A small lower jaw
- Loss of body fat and muscle
- Hair loss, including eyelashes and eyebrows
- A high-pitched voice
- A thin nose with a "beaked" tip
- Wrinkled skin
- Kidney failure
- Poor vision
- Cardiovascular issues
Cause of Progeria
Most kids with progeria have a mutation in the gene that encodes for lamin A. Lamin A is a protein that holds the nucleus of the cell together.
The protein is also known as progerin. The defective protein makes the nucleus unstable that makes cells more likely to die younger leading to the condition called 'Progeria'.
Though parents don't have the issue, their kid may have the mutation. If there is already one child suffering from the cause, there is 2-3% chance that another sibling will have it.
, Treatment DiagnosisMeasures, and Complications
As the signs and symptoms of the condition are pretty clear, it's likely that your child's pediatrician will spot the issue during a routine checkup.
The physician performs some physical examination like hearing test and vision, pulse, blood pressure and monitor height and weight.
Here's a short video you people would definitely enjoy. Some time spent with lovely kids suffering from progeria:
No treatment has yet proven effective for the cause. Most treatment options are basically focused on reducing certain complications in the body.
Though there is no cure, few with progeria exceed 13 years of age. 90% kids with progeria die from complications of heart attack and stroke.
Children with the condition usually develop hardening of the arteries (atherosclerosis).
It is the state in which the walls of the arteries stiffen and thicken; often restricting the passage of blood throughout the body.
Some other health problems associated with aging like cataract, cancer, and arthritis typically do not develop as a part of the course of progeria.
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