Lucía Marín Fabián1º Bachillerato, 2011-2012I.E. “V. Cañada Blanch” London
Progeria is a disorder that affects young children and it isalso known as the Hutchinson Gilford syndrome.
Progeria affects children and gives them an old person’sappearance. The classic type of Progeria (there aredifferent forms) is the Hutchinson-Gilford Progeria Syndrome(HGPS).
At present there are 53 known cases of Progeria around the world and only 2 in the UK
Signs of Progeria may include: Growth failure Loss of body fat Loss of hair Skin starts to look aged Stiffness in the joints Hip dislocation Generalized atherosclerosis (cardio and heart disease) Stroke
• “90% of children with Progeria have a mutation on the gene that encodes Lamin A, a protein that holds the nucleus of the cell together. It is believed that the defective Lamin A protein makes the nucleus unstable. This instability seems to lead to the process of premature aging among Progeria patients”.• Progeria appears to occur without cause - it is not seen in siblings of affected children. In extremely rare cases more than one child in the same family may have the condition
Treatment• Theres no cure for Progeria.• Regular monitoring for cardiovascular disease may help with managing the childs condition.
Certain therapies may help ease or delay some of the signs andsymptoms. They include:• Low-dose aspirin. A daily dose may help prevent heart attacks and strokes.• Other medications. Depending on the childs condition, the doctor may prescribe other medications, such as statins to lower cholesterol or anticoagulants to help prevent blood clots. The use of growth hormone may help increase height and weight.• Physical and occupational therapy. These may help with joint stiffness and hip problems and may allow the child to remain active.• Extraction of primary teeth. Your childs 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.