Peutz-Jeghers syndrome natural history, complications, and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2]

Overview

If left untreated, patients with Peutz-Jeghers syndrome may progress to develop rectal bleeding, anemia, intussusception, bowel obstruction, and abdominal pain. Common complications of Peutz-Jeghers syndrome include colon cancer and cachexia.[1] Prognosis is generally good with treatment.

Natural History

If left untreated, patients with Peutz-Jeghers syndrome may progress to develop rectal bleeding, anemia, intussusception, bowel obstruction, and abdominal pain.

Complications

Complications that can develop as a result of Peutz-Jeghers syndrome are:[2]

Prognosis

Prognosis is generally good with treatment. 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%.[4]

References

  1. Zbuk KM, Eng C. Hamartomatous polyposis syndromes. Nat Clin Pract Gastroenterol Hepatol. 2007 Sep. 4(9):492-502.
  2. http://radiopaedia.org/articles/peutz-jeghers-syndrome-2
  3. Butt N, Salih M, Khan MR, Ahmed R, Haider Z, Shah SH (2012). "An incidentally discovered asymptomatic para-aortic paraganglioma with Peutz-Jeghers syndrome". Saudi J Gastroenterol. 18 (6): 388–91. doi:10.4103/1319-3767.103432. PMC 3530995. PMID 23150026.
  4. "eMedicine - Peutz-Jeghers Syndrome : Article by Andrea Duchini, MD". Retrieved 2007-07-21.

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