Peutz-Jeghers syndrome


Peutz-Jeghers syndrome
A genetic condition characterized by polyps in the intestines and freckle-like spots on the lips, mouth and fingers. People with Peutz-Jeghers syndrome are at increased risk for cancerous tumors of the colon, rectum, stomach, ovary and pancreas. The polyps may occur in any part of the gastrointestinal tract but polyps in the jejunum (in the small intestine) are a consistent feature of the disease. Intussusception (telescoping of the bowel) and intestinal bleeding are the usual symptoms. Peutz-Jeghers syndrome is inherited as an autosomal dominant trait. Mutations of a gene in chromosome 19p13.3 cause the syndrome. The gene, known variously as STK11, PJS, or LKB1, is serine/threonine protein kinase-11. In 1921 Peutz was the first to recognize the familial association of gastrointestinal polyps and spots. A review by Jeghers et al. in The New England Journal of Medicine in 1949 put the polyps-and-spots syndrome "on the map."

* * *

Peutz-Je·ghers syndrome 'pə(r)ts-'jā-gərz-, 'pǣts- n a familial polyposis inherited as an autosomal dominant trait and characterized by numerous polyps in the stomach, small intestine, and colon and by melanin-containing spots on the skin and mucous membranes esp. of the lips and gums
Peutz 'pǣts J. L. A. (1886-1957)
Dutch physician. Peutz published the first detailed description of Peutz-Jeghers syndrome in 1921. Some of the symptoms had been described by British physician Sir Jonathan Hutchinson in 1896.
Jeghers Harold Joseph (1904-1990)
American physician. Jeghers successively served on the medical faculties of Boston University, Georgetown University, New Jersey College of Medicine and Dentistry, and Tufts University. He also held concurrent positions with several hospitals in Boston, Washington, D.C., and Jersey City, New Jersey. His last position was that of director of St. Vincent Hospital in Worcester, Massachusetts. His topics of research included nutrition, especially vitamin A deficiency, renal function, and skin pigmentation. He published his description of Peutz-Jeghers syndrome with V. A. McKusick and K. H. Katz in 1949.

* * *

a hereditary disorder in which the presence of multiple polyp in the lining of the small intestine (intestinal polyposis) is associated with pigmented areas (similar to freckles) around the lips, on the inside of the mouth, and on the palms and soles. The polyps may bleed, resulting in anaemia, or may cause obstruction of the bowel. Half of those affected develop malignant tumours (not necessarily of the bowel).
J. L. A. Peutz (1886-1957), Dutch physician; H. J. Jeghers (1904- ), US physician

* * *

Peutz-Jeg·hers syndrome (pootzґ jaґgerz) [J.L.A. Peutz, Dutch physician, 1886–1957; Harold Jeghers, American physician, 1904–1990] see under syndrome.

Medical dictionary. 2011.

Look at other dictionaries:

  • Peutz-Jeghers syndrome — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 9905 ICD10 = ICD10|Q|85|8|q|80 ICD9 = ICD9|759.6 ICDO = OMIM = 175200 MedlinePlus = eMedicineSubj = med eMedicineTopic = 1807 MeshID = D010580 Peutz Jeghers, also known as Hereditary… …   Wikipedia

  • Peutz-Jeghers syndrome — a hereditary disorder in which the presence of multiple polyps in the lining of the small intestine (intestinal polyposis) is associated with pigmented areas (similar to freckles) around the lips, on the inside of the mouth, and on the palms and… …   The new mediacal dictionary

  • Peutz-Jeghers syndrome — PJS. A genetic disorder in which polyps form in the intestine and dark spots appear on the mouth and fingers. Having PJS increases the risk of developing gastrointestinal and many other types of cancer. Also called PJS …   English dictionary of cancer terms

  • Syndrome de peutz-jeghers — Le syndrome de Peutz Jeghers est une maladie génétique caractérisée par l association de polypes intestinaux et gastriques avec une pigmentation anormale de la peau et des muqueuses. Les polypes, histologiquement des hamartomes, sont le plus… …   Wikipédia en Français

  • Peutz-Jeghers-Syndrom — Klassifikation nach ICD 10 Q85.8 Sonstige Phakomatosen, anderenorts nicht klassifiziert …   Deutsch Wikipedia

  • Syndrome de Peutz-Jeghers — Le syndrome de Peutz Jeghers est une maladie génétique caractérisée par l association de polypes intestinaux et gastriques avec une pigmentation anormale de la peau et des muqueuses. Les polypes, histologiquement des hamartomes, sont le plus… …   Wikipédia en Français

  • Maladie de Peutz-Jeghers-Touraine — Syndrome de Peutz Jeghers Le syndrome de Peutz Jeghers est une maladie génétique caractérisée par l association de polypes intestinaux et gastriques avec une pigmentation anormale de la peau et des muqueuses. Les polypes, histologiquement des… …   Wikipédia en Français

  • síndrome de Peutz-Jeghers — Eng. Peutz Jeghers syndrome Síndrome de herencia autosómica dominante caracterizado por pigmentación en forma de moteado marrón o negro perioficial generalizada, poliposis gastrointestinal con degeneración maligna, manchas puntiformes amarronadas …   Diccionario de oftalmología

  • Syndrome de bannayan-riley-ruvalcaba — Autre nom Syndrome de Riley Smith Référence MIM …   Wikipédia en Français

  • Syndrome de Bannayan-Riley-Ruvalcaba — Référence MIM 153480 Transmission Dominante Chromosome 10q23.31 Gène PTEN Empreinte parentale …   Wikipédia en Français