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Progeria

Progeria (also known as Hutchinson–Gilford Progeria Syndrome, Hutchinson–Gilford syndrome and Progeria syndrome) is an extremely rare genetic condition wherein symptoms resembling aspects of ageing are manifested at an early age. The word Progeria comes from the Greek words "pro" means "before" and "géras" means "old age".
The earliest symptoms include failure to thrive and a localized scleroderma-like skin condition. As a child ages past infancy, additional conditions become apparent. Limited growth, alopecia, and a distinctive appearance (small face and jaw, pinched nose) are all characteristics of progeria. People diagnosed with this disorder usually have small, fragile bodies, like those of elderly people. Later, the condition causes wrinkled skin, atherosclerosis, kidney failure, loss of eyesight, hair loss and cardiovascular problems. It is not transferred by the offspring.

A child with this condition show signs of symptoms usually around 18–24 months. After being born a healthy looking baby, their height and weight suddenly fall below average for their age. Individuals generally retain normal mental and motor development. There are many signs and symptoms of this progressive disease, and they tend to get worse as the child ages. The facial appearance is usually wrinkled, with a larger head in relation to their body, with a narrow face and a beak nose. The child experiences full-body alopecia. Scleroderma, a hardening and tightening of the skin on trunk and extremities of the body, is also prevalent. Since they experience hair loss, prominent scalp veins are noticeable, as well as prominent eyes. Musculoskeletal degeneration causes loss of body fat and muscle, stiff joints, hip dislocations, and other symptoms generally absent in the non-elderly population. The average life expectancy for a child with progeria is about 13, but some with the disease die younger and some live 20 years or longer. Heart problems or strokes are the eventual cause of death in most children with progeria. There's no cure for this condition, but ongoing research shows some promise for treatment.

No treatments have been proven effective. Most treatment focuses on reducing complications (such as cardiovascular disease) with heart bypass surgery or low-dose aspirin. Children may also benefit from a high-calorie diet. There's no cure for progeria. Regular monitoring for cardiovascular disease may help with managing your child's condition. Some children undergo coronary artery bypass surgery or dilation of cardiac arteries (angioplasty) to slow the progression of cardiovascular disease.

Certain therapies may ease or delay some of the signs and symptoms including low dose aspirin to prevent heart attacks and strokes, other medications according to the symptoms present, physiotherapy to help with joint stiffness and hip problems and extraction of primary teeth to prevent overcrowding as second row of teeth starts coming in early. Some anti-cancerous drugs like farnesyltransferase inhibitor may help in correcting cell defects but it is still in trial stage.

Dietary help:
  • Make sure your child drinks plenty of water. Dehydration can be more serious in children with progeria. Be sure your child gets enough to drink, especially during an illness or in hot weather.

  • Provide small frequent meals. Because nutrition and growth can be an issue for children with progeria, giving your child smaller meals more often may help to increase his or her caloric intake.

  • Regular physical activity is essential to keep your child active. Check with your child's doctor to learn which activities are right for your child.

  • Get cushioned shoes or shoe inserts for your child. The loss of body fat in the feet can cause discomfort.

  • Make sure your child is up to date on childhood immunizations. A child with progeria isn't at increased risk of infection, but like all children is at risk if exposed to infectious diseases.

  • Provide learning opportunities. Progeria won't affect your child's intellect, so he or she can attend school at an age-appropriate level.
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