Progeria which is called as Hutchinson-gilford progeria syndrome is a genetic disorder, characterized by rapid aging of children that begin in their first two years.
Children afflicted with progeria appear normal at birth. But after a year, the symptoms of progeria appear in the form of hair loss and slow growth. Children with progeria die very young and live at an average for 13 years and a maximum about 20 years.
Motor and intelligence development are normal for children with progeria, but the growth in height and weight tend to be lower in comparison to other children.
Most of the children with progeria die of strokes or cardiac problems. There is no known cure for progeria.
- Below average growth of height and weight
- Narrowed face with beak like nose
- Loss of hair from scalp, eye brows and eye lashes
- The body skin tends to be tight and hard
- Disproportionately large head in relation to the face
- Thin lips
- Veins visible through the skin
- Eyes are distinct and prominent
- Smaller lower jaw when compared to rest of face
- High pitched voice
- Abnormal and delayed tooth formation
- Diminished muscle and fat content
- Stiff joints
- There may be dislocated hip
- Resistant to insulin
- Irregular heart beat
Causes of progeria
- Mutation of a single gene called lamin A which produces a protein essential for holding the nucleus, makes the cells unstable, leading to symptomatic aging.
- Mutation of the gene usually occurs at the time of conception, when a single egg or sperm get affected by mutation
- A similar syndrome called Wiedemann-Rautenstrauch has genetic basis and tends to run down in families. Wiedemann-Rautenstrauch syndrome affected fetuses tend to age which causes rapid aging and a short life span.
- There is no cure except continuous monitoring of cardiovascular systems of affected children.
- Bypass surgery, or angioplasty or dilation of cardiac arteries can delay the progression of disease.
- Daily low dose of aspirin prevent strokes and cardiac arrests
- Depending on the condition of affected child, statins for cholesterol, anticoagulants for blood clots and growth hormone for putting weight and height may be prescribed.
- Physical therapy for joint pain and stiffness can make stay active.
- Rapid aging bring early adult teeth needing the extraction of baby teeth.
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