Other Names for this Disease
- Lentiginosis, perioral
- Periorificial lentiginosis syndrome
- Peutz Jeghers polyposis
- Polyposis, hamartomatous intestinal
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Peutz-Jeghers syndrome (PJS) is inherited in an autosomal dominant manner. This means that to be affected, a person only needs a change (mutation) in one copy of the responsible gene in each cell. In some cases, an affected person inherits the mutation from an affected parent. Other cases may result from new (de novo) mutations in the gene. These cases occur in people with no history of the disorder in their family. A person with PJS has a 50% chance with each pregnancy of passing along the altered gene to his or her child.
Last updated: 3/15/2014
- Thomas J McGarrity, MD, Christopher I Amos, PhD, Marsha L Frazier, PhD, and Chongjuan Wei, PhD. Peutz-Jeghers Syndrome. GeneReviews. July 2013; http://www.ncbi.nlm.nih.gov/books/NBK1266/.