Q

Chromosomal Disorders

What are complications of Hutchinson-Gilford progeria syndrome (HGPS)?

A Answers (1)

  • People with Hutchinson-Gilford progeria syndrome (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.

    • Atherosclerosis (hardening of the arteries): Children with HGPS typically develop a condition called atherosclerosis, which occurs when the walls of the arteries become hard and thick. Atherosclerosis may limit blood flow to the heart, brain, or other parts of the body. When vital organs do not receive enough blood, they can fail. For instance, if blood flow to the heart is blocked, it causes a heart attack. If blood flow to the brain is blocked, it causes a stroke. Most children with HGPS die from cardiovascular abnormalities, including congestive heart failure, heart attacks, and strokes.

    • Malnutrition: Malnutrition is another common complication of HGPS because some infants have difficulty feeding.

    • Osteoporosis: Patients with HGPS have an increased risk of developing osteoporosis, a condition that causes the bones to become weak, brittle, and porous. As a result, children with progeria may have an increased risk of experiencing bone fractures than healthy children.

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How is Hutchinson-Gilford progeria syndrome (HGPS) diagnosed?