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Peutz-Jeghers Syndrome

more about Peutz-Jeghers Syndrome

  • Peutz-Jeghers Syndrome is an inherited condition that leads to formation of multiple polyps throughout the intestinal tract.  The patient usually will also have dark patches on the lips, on their skin, and/or in the mouth.  There is no specific treatment for this disease.
  • Peutz-Jeghers Syndrome is quite rare.  It is an inherited disease that causes the development of multiple polyps throughout the intestinal tract.  Polyps are growths that develop within the intestines.  Many people will have a few polyps that develop in the colon, especially as they get older.  People with this disease, however, have many, many polyps (sometimes hundreds of polyps) and the polyps develop at an earlier age.  Also, they have polyps throughout their stomach, small intestines, and large intestines.  The polyps that develop in this disease are benign and are not pre-cancerous.  Another characteristic of this disease is dark patches in various parts of the body.  Though the polyps are not pre-cancerous, people with this disease have a very high risk of developing certain types of cancers and need to be followed closely to make sure that any abnormalities are picked up early.  There is no specific treatment.  Any complications that may arise will need to be treated accordingly.

  • There are no specific symptoms.
  • Sometimes, the characteristic dark patches can guide the doctor towards the diagnosis.
  • These dark patches are usually found on the lips, inside the mouth, and on the skin in various parts of the body.
  • If the polyps become very big, then they can cause intestinal bleeding, Intussusception, or Intestinal Blockage.  Otherwise, they may not cause any symptoms.

  • This is an inherited disease, but the culprit gene is still under investigation.

  • Diagnosis is usually made when the characteristic rash is noted or when multiple polyps are found during an endoscopy that is being done for some other reason.
  • Sometimes, the polyps may also be found during surgery that is being done for some unrelated problem or because of a complication of the disease.
  • This condition has a very strong pattern of inheritance.  Therefore, all close relatives of people with this disease need to be tested or checked out for the presence of this problem.  Though the disease cannot be treated, at least close monitoring by the doctor may help prevent some of the complications of this disease.

  • It is not known at the present time what the risk factors are, other than possessing the gene that causes the disease.

  • There is no specific treatment for this disease.
  • People with this disease have a very high risk of developing cancer.
  • Therefore, all people with this disease need to be checked and screened very carefully and very often for any abnormalities.

  • Seek medical attention as soon as possible.
  • Once the diagnosis is made, other family members should also be told to see their doctors.

  • There is no specific way to prevent this disease.
  • However, cancers associated with this disease can be detected earlier if you routinely visit the doctor.

more about Peutz-Jeghers Syndrome

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