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= RESEARCH = = QUESTION: = || What causes progeria? || The Fire Curse and other true medical mysteries, David Lee Drotar, Walker, 1994, Progeria, Mayo Clinic Staff, MFMER, Jan. 24, 2009, [|http://www.mayoclinic.com/health/progeria/DS00936] || (include the words or phrases from keyword searches) || Cause, progeria || Believed to be passed on in an autosomal dominant new mutation fashion. The disorder is transmitted to children by autosomal dominant inheritance. Science has not found the piece of DNA that causes premature aging. Some scientists think that the DNA premature aging victims receive from their parents is unhealthy. However, some think that an unk n own agent-possibly a drug radiation or a pesticide damages the DNA long before the child is born (mutation). In 2003, researchers announced the discovery of a single gene mutation responsible for Hutchinson-Gilford progeria syndrome. The gene is known as lamin A (LMNA), which makes a protein necessary to holding the center (nucleus) of a cell together. Researchers believe the genetic mutation renders cells unstable, which appears to lead to progeria's characteristic aging process. Unlike many genetic mutations, Hutchinson-Gilford progeria isn't passed down in families. Rather, the gene change is a chance occurrence that researchers believe affects a single sperm or egg just before conception. Neither parent is a carrier, so the mutations in the children's genes are new (de novo). Ninety percent of children with progeria have a mutation on the gene that encodes the protein lamin A. Progeria usually occurs sporadically, and is not seen in siblings of affected children, although -- in very rare circumstances -- more than one child in a family can have progeria. It was recently discovered that progeria develops from a mutated gene that contains the information for creating a protein called lamin A. The resulting abnormal protein affects the structure of a cell's nucleus, so in children with progeria, the nucleus becomes unstable, which leads to premature aging. In 2003, NIH-funded researchers discovered that Hutchinson-Gilford progeria is caused by a tiny mutation in a single gene, known as lamin A (LMNA). In laboratory tests involving cells taken from progeria patients, researchers found that the mutation responsible for Hutchinson-Gilford progeria causes the LMNA gene to produce an abnormal form of the lamin A protein which destabilizes the patient’s cells. || = QUESTION: = || What are the symptoms of progeria? || The Fire Curse and other true medical mysteries, David Lee Drotar, Walker, 1994, Progeria, Mayo Clinic Staff, MFMER, Jan. 24, 2009, [|http://www.mayoclinic.com/health/progeria/DS00936] || (include the words or phrases from keyword searches) || Symptoms, progeria || Most patients appear normal at birth. Signs and symptoms usually begin to develop within the first one to two years of life. Symptoms: Changes in skin, failure to gain weight, accelerated aging, hair loss, prominent scalp veins, absence of fat under the skin, scleroderma (thickening of the skin), pinched nose, small face and jaw relative to head size, delayed tooth formation, high pitched voice, impaired or absence of sexual development, stiffening of various joins, bone structure abnormalities. Skin is usually thin, dry, and wrinkled. Exhibits color (pigmentation) changes, which presents clinically as yellow-brownish spots, and the eyes are prominent. Patients with progeria die from an infection, heart attack, or kidney or live r failure. · Hair loss (alopecia), including eyelashes and eyebrows · Hardening and tightening of skin on trunk and extremities (scleroderma) · Loose, aged-looking skin · Head too large for face · Prominent scalp veins · Prominent eyes · Small lower jaw (micrognathia) · High-pitched voice · Delayed and abnormal tooth formation · Loss of body fat and muscle · Stiff joints · Hip dislocation · Baldness · Teeth- delayed or absent formation Patients usually do not grow taller than 3.7ft (1.15m) or weigh over 40lb (15kg). Patients with progeria do not usually exhibit mental impairment.
 * Citation** (title, author, publication, date, URL, etc.) || The Gale Encyclopedia of Genetic Disorders, Laith Farid Gulli and Nicole Mallory, 2002
 * Citation** (title, author, publication, date, URL, etc.) || The Gale Encyclopedia of Genetic Disorders, Laith Farid Gulli and Nicole Mallory, 2002
 * ** Subject of notes **
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 * Try to find a short effective quote || Progeria is a genetic disease.
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 * = RESEARCH =
 * Citation** (title, author, publication, date, URL, etc.) || The Gale Encyclopedia of Genetic Disorders, Laith Farid Gulli and Nicole Mallory, 2002
 * Citation** (title, author, publication, date, URL, etc.) || The Gale Encyclopedia of Genetic Disorders, Laith Farid Gulli and Nicole Mallory, 2002
 * ** Subject of notes **
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 * Try to find a short effective quote || Progeria syndrome is an extremely rare genetic disorder of unknown origin that manifests as premature aging in children.

Arteriosclerosis (hardening of the arteries) is the lead cause of death for progeria. ||  || = QUESTION: = || How can progeria be cured? || The Fire Curse and other true medical mysteries, David Lee Drotar, Walker, 1994, Progeria, Mayo Clinic Staff, MFMER, Jan. 24, 2009, [|http://www.mayoclinic.com/health/progeria/DS00936] || (include the words or phrases from keyword searches) || cure treatment progeria || Notes <span style="font-size: 10pt; font-family: Symbol; msobidifontsize: 16.0pt; msofareastfontfamily: Symbol; msobidifontfamily: Symbol; mso-list: Ignore; msolist: Ignore;">· Low-dose aspirin. A daily dose may help prevent heart attacks and stroke. <span style="font-size: 10pt; font-family: Symbol; msobidifontsize: 16.0pt; msofareastfontfamily: Symbol; msobidifontfamily: Symbol; mso-list: Ignore; msolist: Ignore;">· Physical and occupational therapy. These may help with joint stiffness and hip problems, and may allow your child to remain active. <span style="font-size: 10pt; font-family: Symbol; msobidifontsize: 16.0pt; msofareastfontfamily: Symbol; msobidifontfamily: Symbol; mso-list: Ignore; msolist: Ignore;">· High-calorie dietary supplements. Including extra calories in your child's daily diet may help prevent weight loss and ensure adequate nutrition. <span style="font-size: 10pt; font-family: Symbol; msobidifontsize: 16.0pt; msofareastfontfamily: Symbol; msobidifontfamily: Symbol; mso-list: Ignore; msolist: Ignore;">· Feeding tube. Infants who feed poorly may benefit from a feeding tube and a syringe. You can use the syringe to push pumped breast milk or formula through the tube to make it easier for your child to feed. <span style="font-size: 10pt; font-family: Symbol; msobidifontsize: 16.0pt; msofareastfontfamily: Symbol; msobidifontfamily: Symbol; mso-list: Ignore; msolist: Ignore;">· Extraction of primary teeth. Your child's permanent teeth may start coming in before his or her baby teeth fall out. Extraction may help prevent problems associated with the delayed loss of baby teeth, including overcrowding and developing a second row of teeth when permanent teeth come in. <span style="line-height: 125%; font-family: 'Times New Roman','serif'; mso-fareast-font-family: Batang; mso-bidi-font-family: Arial; mso-bidi-font-size: 16.0pt; mso-font-kerning: 16.0pt; mso-fareast-language: EN-US;">New drugs known as farnesyltransferase inhibitors (FTIs), which were developed for treating cancer, show promise in laboratory studies in correcting the cell defects that cause progeria. FTIs still need to be tested in clinical studies for treatment of progeria Treatment is symptomatic and aimed at providing psychological support. The combination treatment of nutritional therapy and growth hormone supplementation demonstrated an increase in growth of Progeria patients, an increase in growth factors within the blood, and a decrease in the patient’s basal metabolic rate. In one of the recent studies, published in //Nature Medicine//, researchers at the [|National Cancer Institute (NCI)]found that cells affected by this condition could be made healthy again by correcting defects in the lamin A protein. While there is no treatment for progeria, according to the [|Progeria Research Foundation], there are steps parents and doctors can take to improve a child's quality of life. For example, children with progeria often have poor appetites, and certain nutritional supplements, such as Pediasure and Boost, can help them get the nutrition and calories they need. This, in turn, can improve their mood, energy levels and skin and hair health. Likewise, physical or occupational therapy two to three times a week can help children stay active. Additionally, daily low-dose aspirin therapy may be recommended to prevent heart disease, as it is in at-risk adults. Some children with progeria undergo coronary artery bypass surgery and/or angioplasty in attempts to ease the life-threatening cardiovascular complications caused by progressive atherosclerosis. || = RESEARCH = = QUESTION: = || How can you prevent getting progeria? || [] || (include the words or phrases from keyword searches) || prevention progeria || = RESEARCH = = QUESTION: = || What is the life like for a child with progeria? || (include the words or phrases from keyword searches) || Life for progeria children || "I just wish people would stop staring!" Jesper said. He and his friends just want to be as normal as possible. "I am not that much different from others, I am still human," Jesper said to Caroline in a private conversation, "yet i sometimes feel like an animal in a zoo people come to look at. Animals at zoos are locked in cages, I'm locked in my body." "In Boston with other progeria kids I just feel normal!" Jesper countinues saying. ||
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 * = RESEARCH =
 * = RESEARCH =
 * **Citation** (title, author, publication, date, URL, etc.) || The Gale Encyclopedia of Genetic Disorders, Laith Farid Gulli and Nicole Mallory, 2002
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 * Try to find a short effective quote || There is no cure for progeria.
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 * Images/video to support this || its remarkable how progeria patients finally have hope to be cured now. ||
 * Citation** (title, author, publication, date, URL, etc.) ||
 * Citation** (title, author, publication, date, URL, etc.) ||
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 * Try to find a short effective quote || there currently is no treatment or cure for the underlying condition ||
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 * Citation** (title, author, publication, date, URL, etc.) || Jesper Sorensen, Jette Sorensen ||
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 * Try to find a short effective quote || Jesper and his progeria friends all hate when people stare at them. Most wish people would just come up and talk to them like they where normal people.
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 * Images/video to support this || I think what they say is very true. As a cousin to Jesper I have seen how people look at him.

Haley another progeria kid has some advise for how to deal with people staring at them: __ [] __ ||
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