Hutchinson-Gilford Progeria Syndrome, otherwise known as "Progeria" or "HGPS", is a very rare and fatal genetic disorder characterized by the onset of accelerated aging in young children. The rate of aging is accelerated up to seven times compared to that of a normal life in the first 13 years of life. Progeria comes from the Greek word (πρό), “pro” meaning premature and (γῆρας), “gerias” meaning old age. Although there are several forms of progeria, the most severe form of progeria is formally known as Hutchinson-Gilford progeria syndrome, which was named after doctors in England: in 1886, Dr. Jonathan Hutchinson who described the syndrome, and Dr. Hastings Gilford who discovered it independently in 1904 (Jameson).Kittel 2It is estimated that 1 in 4 million newborns is affected by this syndrome worldwide. It starts with children suffering from scleroderma, which causes the skin to appear scaly and thin. Within the first year, a slowdown in the rate of growth and weight gain is observed, physical development becomes stagnant, at two or three years the hair begins to fall out and they lose subcutaneous fat. The intelligence and emotional development of children affected by progeria are comparable to those of children of the same age but not affected by the syndrome (Hennekam, 2006 pp. 2603-2624). Development of Classical Progeria Progeria is hypothesized to be a genetic mutation that occurs at or shortly after conception. During pregnancy the mother has no abnormalities or complications that indicate a possible problem with the pregnancy. Newborns appear normal and on average have a slightly lighter birth weight and...... middle of paper ......r Progeria. Monitoring for cardiovascular disease can help manage heart conditions that occur later in your child's life. Some children may undergo coronary artery bypass surgery to dilate the heart arteries (angioplasty) to reduce the risk of cardiovascular disease. Physical and occupational therapy will relieve stiff joints and allow for greater flexibility and allow children to maintain a healthy level of play time with friends or even certain sports. Regular dental visits are important, and extracting baby teeth will help prevent problems associated with HGPS. Eating good calorie storage and promoting smaller meals frequently throughout the day will help with energy and growth. Hydration is very important when dealing with a child with Progeria; Due to thin, dry skin, children with progeria are more prone to dehydration.
tags