Dr. Chris Amos
Juvenile polyposis (JP) is another
condition associated with the development of polyps in the intestinal
tract. The polyps are usually benign and develop during the first
or second decade of life. JP can be
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distinguished from Peutz-Jeghers syndrome (PJS)
and familial adenomatous polyposis (FAP) by the pathology of the
polyps and the number, location and age of onset of the polyps,
and by the presence of other features associated with PJS and FAP.
Affected individuals have numerous polyps in the colon and small
intestine, but they do not have the freckling or other hyperpigmentation
that is seen in individuals with Peutz-Jeghers syndrome. There are
several causes of JP, but 2 genetic forms that have been recognized
have been attributed to mutations in the genes, SMAD4 and PTEN.
People with JP caused by SMAD4 mutations have a higher risk for
colon and pancreatic cancers. People with JP caused by alterations
in the PTEN are rarer, but may have a higher risk for thyroid and
breast cancers. Other causes for JP have not yet been identified.
Sometimes families with JP may also have a family history of heart
defects. Dr. James Howe at the University of Iowa College of Medicine
is studying JP, and you may contact him at (319)356-7945 for more
information about his study.
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