Coordinated Health

Conditions

Definition

Progeria is a rare genetic condition that produces rapid aging in children.

Alternative Names

Hutchinson-Gilford progeria syndrome; HGPS

Causes

Progeria is a rare condition that is remarkable because its symptoms strongly resemble normal human aging, but occur in young children. It usually is not passed down through families. Rarely is it seen in more than one child in a family.

Symptoms

  • Growth failure during the first year of life
  • Narrow, shrunken or wrinkled face
  • Baldness
  • Loss of eyebrows and eyelashes
  • Short stature
  • Large head for size of face (macrocephaly)
  • Open soft spot (fontanelle)
  • Small jaw (micrognathia)
  • Dry, scaly, thin skin
  • Limited range of motion
  • Teeth – delayed or absent formation

Exams and Tests

The health care provider will perform a physical exam and order laboratory tests. This may show:

  • Insulin resistance
  • Skin changes similar to that seen in scleroderma (the connective tissue becomes tough and hardened)
  • Generally normal cholesterol and triglyceride levels

Cardiac stress testing may reveal signs of early atherosclerosis of blood vessels.

Genetic testing can detect changes in the gene (LMNA) that causes progeria.

Treatment

There is no specific treatment for progeria, but aspirin and statin medications may be used to protect against a heart attack or stroke.

Support Groups

Progeria Research Foundation, Inc. — www.progeriaresearch.org

Outlook (Prognosis)

Progeria causes early death. Patients usually only live to their teenage years (average lifespan of 13 years). However, some patients can live into their early 20s. The cause of death is usually related to the heart or a stroke.

Possible Complications

  • Heart attack (myocardial infarction)
  • Stroke

When to Contact a Medical Professional

Call for an appointment with your health care provider if your child does not appear to be growing or developing normally.

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