Definition: Progeria is a disease that produces rapid aging, beginning in childhood.
Alternative Names: Hutchinson-Gilford syndrome
Causes, incidence, and risk factors: Progeria is a rare condition that is remarkable because its symptoms strongly resemble normal human aging, but occur in young children. Ninety percent of children with progeria have a mutation on the gene that encodes the protein lamin A. Progeria usually occurs without cause. It is not seen in siblings of affected children, although -- in very rare circumstances -- more than one child in a family can have the condition.
Signs and tests: The signs include: - Skin changes similar to that seen in scleroderma (the connective tissue becomes tough and hardened)
- Insulin-resistant diabetes (diabetes that does not respond readily to insulin injections)
Cardiac stress testing may reveal signs of early atherosclerosis of blood vessels. Genetic testing can detect mutations in lamin A that cause progeria.
Treatment: There is presently no treatment for progeria.
Expectations (prognosis): Progeria is associated with a short lifespan. The average patient survives to the early teens. However, some patients can live up to 30 years. The cause of death is usually related to the heart or a stroke as a result of the progressive atherosclerosis.
Calling your health care provider: Call for an appointment with your health care provider if you child does not appear to be growing or developing normally.
Prevention: There is no known prevention.
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