Hello all steemian friends!!
In the Health series we have come up with a new topic, Progeria!!

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Look Closely At The Picture!.....What do you See???

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A thin old man with bald head, wrinkled cheeks Who is wearing old type of glasses???..is it??
If you are thinking like that, you are wrong . He is not an old man, he is a Child with a Genetic disease known as Progeria. If you have watched the movies 'Life According to Sam', 'Paa' or 'The Curious Case of Benjamin Button' , then you probably know what this condition is!

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What is Progeria ???

Greek, Pro= before, Geras= Old Age
Progeria, also known as Hutchinson-Gliford Syndrome is a genetic disorder in which a child Ages rapidly and looks like an old person! This was first discovered by Jonathon Hutchinson in 1886, later it was recorded by Hastings Gliford in 1897.
1 in 8million children will have progeria. According to Progeria Research Foundation there are currently 148 children living with progeria in the world. In this condition children usually live only upto 14 yrs.

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Genetics!...What causes this disease???

The genetic defect Lies in the Gene LMNA. In normal individual, the LMNA gene produces Lamin-A protein which is a essential constituent of nuclear membrane, it stabilizes the nuclear membrane.
But in progeria, due to Mutation of LMNA gene abnormal Lamin-a protein is produced. Due to Which nuclear membrane becomes unstable and infoldings appear in it (see in the figure) . These cells with abnormal nuclear membrane die prematurely, and remember our body is nothing but a group of these cells. So eventually children die in early life. The mean age that children live is only 14 years.
Despite Being a genetic disorder there is very very little chance of inheritance to children, almost all patient of progeria have no family history of it.

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What symptoms will the Children Have??

• Symptoms start to appear after 1 to 2 yrs of life
• They have slow bone and muscle growth, so they are small with big head, big eyes, small face and small jaw,
• Delayed teeth development , high pitched voice
• Alopecia- loss of hair, they have bald head and veins are prominent in the head, even eyelashes are not there
• Very little fatty tissue and thin skin - so they have wrinkles all over the body , that is the reason they look very old.
• They have fragile bone,stiff joints and there is high chance of fracture
• High risk of Athereosclerosis and thereby cardiac diseases, Most of the children with this disease die because of Heart attack

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What about their brain?? Does their Brain also ages Rapidly as the rest of the body??

The answer is 'No'. They have normal brain development and have IQ similar to a normal child of same age. Although they look old from outside they are child mentally. We can identify it by their child like Behaviour.

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Is it Curable??? Is there any treatment Available??

'No'. It is not curable, Yet. But various researches are going on . Treatment available are symptomatic only & these are-

• Since they have high chance of atherosclerosis and heart disease- low dose Aspirin, Statins Group of Drugs, Nitrates and anticoagulants can be given,
• They should be given enough water to prevent dehydration and food should be given as small frequent meals,
• Physiotherapy - Regular physical exercise improves Musculoskeletal growth and also helps preventing atherosclerosis
• Psychotherapy- Since the children look different from their friends, They my feel depressed. They and their family should be counselled about the condition properly.

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You can live a Happy live despite being diagnosed with Progeria!

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Image and Contents credits- [1], [2], [3], [4],[5], [6]

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Thats it for today!! Stay tuned for more of these Health, medicine and science topics!

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