Never judge a book by its cover. Don’t assume a person with wrinkly skin, age spots, or balding scalps are old. They could be struggling with an extremely rare disease known as Hutchinson-Gilford Progeria. One summer I was extremely bored. I went on youtube clicking on random videos until I ended up on a documentary on Progeria. As I watched, I became so intrigued.
Hutchinson-Gilford Progeria Syndrome ("Progeria", or "HGPS") is a rare, terminal genetic disease characterized by accelerated aging in children. Its name is derived from the Greek and means "prematurely old." And is named after the doctors who first described it in England; in 1886 by Dr. Jonathan Hutchinson and in 1897 by Dr. Hastings Gilford.
Many children with progeria are normal healthy-looking babies, however, within the first year of their life, the symptoms begin to appear. The symptoms of progeria closely resemble the normal human aging process. Progeria ages kids about 8 times faster than regular. Signs of Progeria include slow growth, failure to gain weight, loss of body fat , loss of hair, wrinkled skin, age spots, fragile bones, loss of hearing, and stiffness of joints.
As children get older, they suffer from osteoporosis, generalized atherosclerosis, cardiovascular (heart) disease and stroke. The disease affects aging however, the brain stays intact. As their body ages, their minds get left behind. Progeria is a genetic disease that is dominant. Progeria is a genetic mutation in the gene called LMNA. The lamin A protein holds the nucleus of a cell together. The abnormal lamin A protein that causes Progeria is called progerin. Progerin makes the nucleus unstable. That cellular instability leads to the process of premature aging and diseas...
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Unfortunately, since there is no cure, the end result is ultimately death. The average lifespan of a child living with progeria is about thirteen however the oldest person has ever lived with this disease is thirty-two. Some children die as young as five or six. Since their bodies age about eight times faster, a thirteen year old would be living inside a body equivalent to that of somebody who is one hundred and four years old.
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Humans undergo several stages during their lifetime including growth, development, reproduction and senescence. Senescence is defined as the deteriorative biological changes that organisms experience as they age eventually leading to death. These changes include low metabolism, a weak immune system, memory loss, poor vision and loss of hearing. Senescence begins in humans during their post-reproductive years. However, gerontology research has shown that individuals who reproduce late have longer life spans compared to individuals who reproduce early. Nonetheless, it does not indicate that senescence is inevitable. All organisms experience senescence, but at different rates and time. Many genetic diseases such as Alzheimer’s disease and Huntington’s disease are prevalent in older individuals and the symptoms being to appear in middle adulthood. The causes of genetic diseases and disabilities in older individuals are explained by three evolutionary theories: antagonistic pleiotropy theory, mutation accumulation theory and disposable soma theory. These theories suggest that favorable natural selection and heavy allocation of resources for somatic maintenance during the reproductive period decreases the chances of genetic diseases in younger individuals.
...r Progeria. Monitory for cardiovascular disease may help with managing the heart conditions that occur later in the child’s life. Some children may have coronary artery bypass surgery of dilation of cardiac arteries (angioplasty) to reduce the onset of cardiovascular disease. Physical and occupation therapy will ease stiff joints and allow more flexibility and allows children to maintain a healthy level of playtime with friends or even some sports. Regular dental visits are important and having the milk teeth extracted will help prevent problems associated with HGPS. Good nutrition for caloric building and promoting smaller meals frequently through out the day will help with energy and growth. Hydration is very important when dealing with a child suffering from Progeria; due to the thin dry skin children with progeria are more prone to become dehydrated.
It is noteworthy to mention that there are numerous diseases associated with rapid ageing and progeria like symptoms. Cockayne, Lison, Werner’s, and Wiedemann-Rautenstrauch Syndromes are amongst these diseases. The shortened term progeria can be used to address any of these disorders but is most often specifically associated with HGPS. This distinct disease was named after Jonathan Hutchinson and Hastings Gilford who each independently described it in 1886 and 1897 respectively. Thankfully, this alarming syndrome is so rare that it only affects about 1 in every 4 million children born.
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Aging occurs in every species. Over time a change occurs on a cellular level in a person’s body, which causes degenerative effects on the brain, muscles, organs, bones, hormones, and DNA. In 1991, the book Evolutionary Biology of Aging, offered the following definition of aging: a persistent decline in the age-specific fitness components of an organism due to internal physiological deterioration.1 Aging affects the body physically and mentally. Many people dread getting older due to the numerous changes the body goes through. The geriatric population experiences many pains and is inflicted with various diseases. There are a few who are lucky enough to not get diagnosed with a life altering disease, such as Alzheimer’s, type II diabetes, high blood pressure, macular degeneration, or some form of cancer. Studies have shown that genetics play a vital role in the aging process.
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Wrinkles are not unavoidable; they are not a necessary part of aging. Some look to wrinkles as a sign of aged wisdom, but most men and women do not look forward to them. In fact, they are considered an unwelcome and distressful part of the aging process. For many years now, Botox has been available to treat these distasteful telling signs of our years on earth. Today, more people than ever are not only using Botox to treat wrinkles but to prevent them as well.
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This is a genetic condition that is characterized by the dramatic, rapid appearance of aging beginning in childhood. Children with this condition more often have prominent eyes, thin lips, a thin nose, and protruding ears. Alopecia is also common and so is aged looking skin and joint abnormalities. Hardening of the arteries (arteriosclerosis) is also common. This increases the chances of having a heart attack or a stroke. This condition is rare and is reported to occur in 1 in 4 million newborns worldwide. This condition is diagnosed by genetic testing along with other physical examinations. This condition is caused by a mutation in the LMNA gene. The LMNA provides instructions for making proteins called lamin. This condition results in the production of an abnormal version of the lamin A protein. Because of this mutated protein, the nuclear envelope is unstable and the nucleus becomes progressively damaged. The average life expectancy for someone with this disease is approximately 13 years old. There is not a known cure for this disease, but medications and therapy can help alleviate
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