Progeria

Luca Marn Fabin1 Bachillerato, 2011-2012I.E. V. Caada Blanch London

Progeria

1. What is progeria?

Progeria is a disorder that affects young children and it is also known as the Hutchinson Gilford syndrome.

PROGERIA

Progeria affects children and gives them an old persons appearance. The classic type of Progeria (there are different forms) is the Hutchinson-Gilford Progeria Syndrome (HGPS).

2. How prevalent is Progeria?At present there are 53 known cases of Progeria around the world and only 2 in the UK

3. What are the characteristics of Progeria?Signs of Progeria may include:

Growth failureLoss of body fatLoss of hairSkin starts to look agedStiffness in the jointsHip dislocationGeneralized atherosclerosis (cardio and heart disease) Stroke

4. What causes Progeria?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

There's no cure for Progeria.

Regular monitoring for cardiovascular disease may help with managing the child's condition.

Treatment

Certain therapies may help ease or delay some of the signs and symptoms. They include: Low-dose aspirin. A daily dose may help prevent heart attacks and strokes.Other medications. Depending on the child's 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 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.

Other similar syndromes

There are other similar syndromes that run in families, e.g.: The Wiedemann-Rautenstrauch syndrome, also known as neonatal progeroid syndrome, when aging begins in the womb and The Werner syndrome, which begins in adolescence or early adulthood.

These inherited progeroid syndromes also cause rapid aging and shortened life span.

SOURCESwww.medicalnewstoday.comhttp://healthmad.com/conditions-and-diseases/progeria-the-80-year-old-children/Hayleys page.www.mayoclinic.comGoogle images.www.youtube.com

Progeria