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Peutz-Jeghers syndrome - Overview

Alternative Names

PJS

Definition of Peutz-Jeghers syndrome:

Peutz-Jeghers syndrome (PJS) is a disorder often passed down through families (inherited) in which the person develops intestinal polyps and is at a significantly higher risk for developing certain cancers.

Causes, incidence, and risk factors:

It is unknown how many people are affected by PJS. However, the National Institutes of Health estimates that it affects about 1 in 25,000 to 300,000 births.

There are two types of PJS:

Familial PJS is due to a mutation in a gene called STK11. The genetic defect is passed down (inherited) through families as an autosomal dominant trait. That means if one of your parents has this type of PJS, you have a 50:50 chance of inheriting the bad gene and having the disease.
Sporadic PJS is not passed down through families and appears unrelated to the STK11 gene mutation.

Digestive system organs

Reviewed last on: 10/15/2009
Todd Eisner, MD, Private practice specializing in Gastroenterology, Boca Raton, FL, Clinical Instructor, Florida Atlantic University School of Medicine. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Goldman L, Ausiello D. Cecil Textbook of Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007.

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