Hutchinson-Gilford progeria syndrome (HGPS)


Hutchinson-Gilford progeria syndrome (HGPS), also called progeria and premature aging syndrome, is an extremely rare genetic disorder that causes premature aging shortly after birth. Although patients with HGPS have normal intelligence and motor control skills, they have distinct physical features that make them appear older than they really are. This is because a random genetic mutation causes cells in their bodies to die earlier than normal.
Symptoms, such as slowed growth, hair loss, and loose, aged-looking skin, typically develop when the child is six months to two years old. In addition, children with HGPS typically develop a condition called atherosclerosis, which occurs when the walls of the arteries become thick and hard.
There are several different forms of progeria, but the classic and most common type is HGPS. This disorder was named after two physicians from England, Dr. Jonathan Hutchinson, who first described it in 1886, and Dr. Hastings Gilford, who first described it in 1897.
It is estimated that HGPS occurs in about one out of eight million births. However, some researchers believe the incidence might be closer to one out of four million births because many cases may be undiagnosed or misdiagnosed as other conditions, such as Werner's syndrome (a very rare, autosomal recessive disorder also characterized by premature aging). Only about 100 cases worldwide have been reported in the medical literature.
There is currently no cure for HGPS, and a person's prognosis is generally very poor. People with HGPS may live to be 7-30 years old. On average, most people die at age 13. About 90 percent of children with HGPS die from complications related to atherosclerosis.

Related Terms

Atherosclerosis, farnesyltransferase inhibitors, FTI, HGPS, high-calorie dietary supplements, Hutchinson-Gilford progeria syndrome, Hutchinson-Gilford syndrome, lamin A, LMNA, premature aging, premature aging syndrome, progeria, progeria of childhood.