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Progeria
Hutchinson-Gilford progeria syndrome; HGPS
Progeria is a rare genetic condition that produces rapid aging in children.
Causes
Progeria is a rare condition. It is remarkable because its symptoms strongly resemble normal human aging, but it occurs in young children. In most cases, it is not passed down through families. It is rarely seen in more than 1 child in a family.
Symptoms
Symptoms include:
- Growth failure during the first year of life
- Narrow, shrunken or wrinkled face
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Baldness
Baldness
Partial or complete loss of hair is called alopecia.
ImageRead Article Now Book Mark Article - Loss of eyebrows and eyelashes
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Short stature
Short stature
A child who has short stature is much shorter than children who are the same age and sex. Your health care provider will go over your child's growth ...
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Large head for size of face (
macrocephaly
)
Macrocephaly
Increased head circumference is when the measured distance around the widest part of the skull is larger than expected for the child's age and backgr...
ImageRead Article Now Book Mark Article - Open soft spot (fontanelle)
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Small jaw (
micrognathia
)
Micrognathia
Micrognathia is a term for a lower jaw that is smaller than normal.
ImageRead Article Now Book Mark Article - Dry, scaly, thin skin
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Limited range of motion
Limited range of motion
Limited range of motion is a term meaning that a joint or body part cannot move through its normal range of motion.
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Teeth - delayed or absent formation
Teeth - delayed or absent formation
When a person's teeth grow in, they may be delayed or not occur at all.
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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. Aspirin and statin medicines 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. People with the condition most often only live to their teenage years (average lifespan of 13 years). However, some can live into their early 20s. The cause of death is very often related to the heart or a stroke .
Stroke
A stroke occurs when blood flow to a part of the brain stops. A stroke is sometimes called a "brain attack. " If blood flow is cut off for longer th...
Possible Complications
Complications may include:
-
Heart attack (
myocardial infarction)
Myocardial infarction)
Most heart attacks are caused by a blood clot that blocks one of the coronary arteries. The coronary arteries bring blood and oxygen to the heart. ...
ImageRead Article Now Book Mark Article - Stroke
When to Contact a Medical Professional
Call your provider if your child does not appear to be growing or developing normally.
References
Gordon LB, Brown WT, Collins FS. Hutchinson-Gilford progeria syndrome. Gene Reviews. Seattle, WA: University of Washington: 2015:1. PMID: 20301300 www.ncbi.nlm.nih.gov/pubmed/20301300 . Accessed August 1, 2015.
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Coronary artery blockage - illustration
Atherosclerosis is a common disorder of the arteries. Fat, cholesterol, and other substances collect in the walls of arteries. Larger accumulations are called atheromas or plaque and can damage artery walls and block blood flow. Severely restricted blood flow in the heart muscle leads to symptoms such as chest pain.
Coronary artery blockage
illustration
-
Coronary artery blockage - illustration
Atherosclerosis is a common disorder of the arteries. Fat, cholesterol, and other substances collect in the walls of arteries. Larger accumulations are called atheromas or plaque and can damage artery walls and block blood flow. Severely restricted blood flow in the heart muscle leads to symptoms such as chest pain.
Coronary artery blockage
illustration
Review Date: 8/1/2015
Reviewed By: Chad Haldeman-Englert, MD, FACMG, Fullerton Genetics Center, Asheville, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
