Peutz-Jeghers syndrome


Also found in: Dictionary, Thesaurus, Acronyms, Encyclopedia, Wikipedia.
Related to Peutz-Jeghers syndrome: Lynch syndrome, Turcot syndrome

Peutz-Jeghers syndrome

 [pertz ya´gerz]
familial gastrointestinal polyposis, especially in the small bowel, associated with mucocutaneous pigmentation.

Peutz-Je·ghers syn·drome

(pŭtz jā'gĕrz), [MIM*175200]
generalized hamartomatous multiple polyposis of the intestinal tract, consistently involving the jejunum, associated with melanin spots of the lips, buccal mucosa, and fingers; autosomal dominant inheritance, caused by mutation in the serine/threonine kinase gene (STK11) on chromosome 19p.

Peutz-Jeghers syndrome

[poits′ jeg′ərz]
Etymology: J.L.A. Peutz, Dutch physician, 1886-1957; Harold J. Jeghers, American physician, b. 1904
an inherited disorder transmitted as an autosomal-dominant trait, characterized by multiple intestinal polyps and abnormal mucocutaneous pigmentation, usually over the lips and buccal mucosa. If obstruction or bleeding occurs, surgical removal of the polyps may be indicated.
enlarge picture
Peutz-Jeghers syndrome

Peutz-Jeghers syndrome

Surgery An AD condition characterized by brownish perioral and oral macules developed in infancy, accompanied by premalignant intestinal polyps causing abdominal cramping, intussusception, chronic bleeding, anemia due to chronic blood loss

Peutz-Jeg·hers syn·drome

(PJS) (pŭts jā'gĕrz sindrōm)
Generalized hamartomatous multiple polyposis of the intestinal tract, consistently involving the jejunum, associated with melanin spots of the lips, buccal mucosa, and fingers. Also called Jeghers-Peutz syndrome

Peutz-Jeghers syndrome

A rare AUTOSOMAL DOMINANT genetic disorder in which large numbers of small polyps grow on the lining of the intestine, and small, flat, brown spots appear on the lips and in the mouth. Unlike MULTIPLE POLYPOSIS this condition rarely progresses to cancer. Complications are uncommon. (John Law Augustine Peutz, 1886–1957, Dutch physician, and Harald Jos Jeghers, American physician, b. 1904).

Jeghers,

Harold Joseph, U.S. physician, 1904-1990.
Jeghers-Peutz syndrome - Synonym(s): Peutz-Jeghers syndrome
Peutz-Jeghers syndrome - see under Peutz

Peutz,

Johnnes Laurentius Augustinus, Dutch physician, 1886-1957.
Jeghers-Peutz syndrome - Synonym(s): Peutz-Jeghers syndrome
Peutz syndrome - Synonym(s): Peutz-Jeghers syndrome
Peutz-Jeghers syndrome - generalized hamartomatous multiple polyposis of the intestinal tract, consistently involving the jejunum, associated with melanin spots of the lips, buccal mucosa, and fingers. Synonym(s): Jeghers-Peutz syndrome; Peutz syndrome

Peutz-Jeg·hers syn·drome

(pŭts jā'gĕrz sindrōm) [MIM*175200]
Inherited intestinal polyposis syndrome in which polyps are hamartomatous and thus do not transform to malignancy. Small macular pigmentations may appear around mouth.
References in periodicals archive ?
Patients with Peutz-Jeghers syndrome develop numerous histologically characteristic polyps throughout the gastrointestinal tract.
Patients with Peutz-Jeghers syndrome have a 40% risk of colorectal cancer, a 50% risk of breast cancer, and elevated risks of pancreatic, stomach, small intestinal, esophageal, ovarian, lung, cervical, uterine, and testicular cancers.
Treatment of Peutz-Jeghers syndrome involves endoscopic resection of any large polyps.
12, 13) In the Peutz-Jeghers syndrome perioral buccal lentiginosis is accompanied by polyposis of the intestine.
In Peutz-Jeghers syndrome, Key diagnostic features are hamartomatous polyps and mucosal hyperpigmentation.
Peutz-Jeghers syndrome (PJS) is an autosomal, dominantly inherited disease characterized by hamartomatous polyps of the gastrointestinal tract and pigmented macules of the lips and buccal mucosa.
57,89) Individuals with a strong family history of pancreatic cancer (at least 3 close relatives with pancreatic cancer) or with the Peutz-Jeghers syndrome were screened by using a combination of EUS and computed tomography, and in some cases, endoscopic retrograde cholangiopancreatography.
Increased risk for cancer in patients with the Peutz-Jeghers syndrome.
Increased risk of cancer in the Peutz-Jeghers syndrome.
INTRODUCTION: Peutz-Jeghers polyps are uncommon hamartomatous polyps that occur as a part of a rare autosomal dominant Peutz-Jeghers syndrome.
3) Diagnostic Criteria for Peutz-Jeghers Syndrome (PJS) include (1) Three or more histologically confirmed Peutz-Jeghers polyps, or (2) Any number of Peutz-Jeghers polyps with a family history of PJS, or (3) Characteristic prominent mucocutaneous pigmentation with a family history of PJS, or (4) Any number of Peutz-Jeghers polyps and characteristic prominent mucocutaneous pigmentation.
As Peutz-Jeghers Syndrome is rare in India, a case report has been sent for publication.