In a study published in the March issue of The Journal of Pediatrics, researchers found that decreased levels of HDL cholesterol, or "good" cholesterol, may contribute to premature heart disease in children with Hutchinson-Gilford Progeria Syndrome, or Progeria. Additionally, adiponectin, a hormone that regulates the metabolism of fat and sugar, may be linked to the disease process and prove helpful in finding treatments.
Progeria is a rare, fatal genetic condition characterized by the appearance of accelerated aging in children, and affects about one in 4-8 million newborns. Although they are born looking healthy, children with Progeria begin to display characteristics of accelerated aging at around 18-24 months of age. Indicators of the disease include growth failure, loss of body fat and hair, aged-looking skin, stiffness of joints, hip dislocation, and atherosclerosis.
"All children with Progeria die between the ages of 6 and 20 years from heart failure or stroke," said Leslie Gordon, MD, PhD, lead author of the study, Medical Director of the Progeria Research Foundation (PRF), and assistant professor, Tufts University School of Medicine.
"Studying heart disease as it relates to children with Progeria can help us better understand how atherosclerosis will affect the aging population while also helping these precious children."
In the study published in The Journal of Pediatrics, a team of researchers compared cholesterol levels in children with Progeria to children who do not have the disease. The scientists discovered that, compared with children who do not have Progeria, children with Progeria in their mid- and later years have decreased levels of HDL cholesterol - or "good" cholesterol - and adiponectin, a hormone that regulates the metabolism of fat and sugar. Both factors work to remove fat from plaques in arteries, and the lower levels may contribute to accelerated plaque formation. However, LDL - or "bad" cholesterol - and triglyceride levels, which are elevated in adults with heart disease, are usually at normal levels in children with Progeria.
Dr. Gordon - along with family and friends - founded the Progeria Research Foundation in 1999 as the only organization solely dedicated to discovering the cause, treatment and cure for Progeria after her son, now eight-years-old, was diagnosed with the disease. In April 2003, PRF succeeded in the first part of its mission when the Foundation helped to discover the gene that causes Progeria.
http://www.progeriaresearch.org and http://www.amjmed.org/