Peutz-Jeghers syndrome


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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

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 ?
Molecular and clinical characteristics in 46 families affected with Peutz-Jeghers syndrome. Dig Dis Sci 2007;52:1924-1933.
Ovarian sex cord tumor with annular tubules: review of 74 cases including 27 with Peutz-Jeghers syndrome and four with adenoma malignum of the cervix.
Peutz-Jeghers syndrome. In: Hamilton SR, Aaltonen LA, editors.
Table 1: Inherited Predisposition to Pancreatic Cancer Disorder Gene Increased Risk of (Chromosome Location) Pancreatic Cancer fold Hereditary breast and BRCA2(13q12-q13) 4-10 ovarian cancer Familial atypical p16/CDKN2A (9p21) 20-35 multiple-mole melanoma syndrome Strong family history Unknown 14-32 (3 or more relatives with pancreatic cancer) Hereditary pancreatitis PHSS1 (7q35) and 50-90 SPINK1 Peutz-Jeghers syndrome LKB (19p13) 130 Disorder Risk of Pancreatic Cancer by Age 70 (%) Hereditary breast and 5 ovarian cancer Familial atypical 10-17 multiple-mole melanoma syndrome Strong family history 8-16 (3 or more relatives with pancreatic cancer) Hereditary pancreatitis 25-40 Peutz-Jeghers syndrome 30-60
Peutz-Jeghers Syndrome (PJS) is a rare familial autosomal dominant disorder characterized by mucocutaneous pigmentation and gastrointestinal polyps.
* Peutz-Jeghers syndrome. The hallmark skin-related characteristics of this disease include mucocutaneous pigmentation due to melanin deposition.
In patients with Peutz-Jeghers syndrome, pancreatic cancer tends to present in the fourth decade of life.
The risk of small bowel adenocarcinoma is increased in Celiac disease, Crohn's disease, familial adenomatous polyposis (FAP), and Peutz-Jeghers syndrome. Lymphomas are associated with Celiac disease, immunoproliferative small intestinal disease (IPSID) and very rarely to Crohn's disease.
Peutz-Jeghers Syndrome (PJS) is an autosomal dominant genetic condition in which hamartomatous polyps (a benign, noncancerous form) develop in the gastrointestinal tract, including the stomach, small intestine and colon.
These tumors are probably if anything less common than Sertoli-Leydig cell tumors and are characterized in most cases by a pure or predominant tubular pattern although occasional variant morphologies may be seen in tumors that are less well differentiated; the rare lipid-rich type, when it occurs in children, which it typically does, may result in isosexual pseudoprecocity and 4 among the small number of patients in this category have had Peutz-Jeghers syndrome. (55) These tumors of whatever subtype are almost invariably unilateral and stage I and typically have a lobulated, solid, yellow sectioned surface.
Duodenal intussusception due to a giant neuroendocrine carcinoma in a patient with Peutz-Jeghers syndrome: case report and systematic review.