Peutz-Jeghers syndrome natural history, complications, and prognosis

Peutz-Jeghers syndrome Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Peutz-Jeghers, also known as Hereditary Intestinal Polyposis Syndrome, is an autosomal dominant genetic disease characterized by the development of benign hamartomatous polyps in the gastrointestinal tract.

Prognosis

Almost half of Peutz-Jeghers patients die from cancer by age 57 years, and the cumulative risk of developing a form of cancer associated with Peutz-Jeghers syndrome between ages 15-64 is 93%.

References

de:Peutz-Jeghers-Syndrom it:Sindrome di Peutz-Jeghers nl:Syndroom van Peutz-Jeghers