juvenile polyposis syndrome , JPS (Abkürzung)

Chow E, Macrae F: A review of juvenile polyposis syndrome.

The World Health Organization criteria for diagnosis of juvenile polyposis syndrome are one of either:

It is also found mutated in the autosomal dominant disease juvenile polyposis syndrome (JPS).

Juvenile Polyposis Syndrome can occur sporadically in families or be inherited in an autosomal dominant manner.

Individuals with Peutz-Jeghers and juvenile polyposis syndromes are at increased risk of colorectal cancer, as well as extracolonic cancers.

Juvenile polyposis syndrome is a syndrome characterized by the appearance of multiple juvenile polyps in the gastrointestinal tract.

Lifetime risk of developing cancers of the gastro-intestinal tract in people with Juvenile Polyposis Syndrome ranges from 9% to 50%.

While the majority of the polyps found in Juvenile Polyposis Syndrome are non-neoplastic, hamartomatous, self-limiting and benign, there is an increased risk of adenocarcinoma.

The term 'Juvenile' in the title of Juvenile Polyposis Syndrome refers to the histological type of the polyps rather than age of onset.

Two genes associated with Juvenile Polyposis Syndrome are BMPR1A and SMAD4.

Hamartomatous polyps are often found by chance; occurring in syndromes such as Peutz-Jegher Syndrome or Juvenile Polyposis Syndrome.

Juvenile polyposis syndrome: SMAD4/DPC4 and BMPR1A genes.

Clinical Practice Guidelines for the Diagnosis of Colon Cancer in Familial Juvenile Polyposis Syndrome (JPS)

Coburn MC, Pricolo VE, DeLuca FG, et al.: Malignant potential in intestinal juvenile polyposis syndromes.

For example, hereditary colon cancer may be due to familial adenomatous polyposis (FAP), Lynch syndrome, Peutz-Jeghers syndrome, juvenile polyposis syndrome, or other syndromes.

Aretz S, Stienen D, Uhlhaas S, et al.: High proportion of large genomic deletions and a genotype phenotype update in 80 unrelated families with juvenile polyposis syndrome.

BMPR1A, SMAD4 and PTEN are responsible for Juvenile polyposis syndrome, juvenile intestinal polyposis and Cowden's disease.

Familial adenomatous polyposis (FAP), Gardner Syndrome, Lynch Syndrome, Muir-Torre syndrome, Celiac sprue, Puetz-Jeghers, Crohn's Disease and Juvenile Polyposis Syndrome are risk factors for developing this cancer.

Juvenile polyposis syndrome (JPS) is a genetically heterogeneous, rare, childhood-onset, autosomal dominant disease that presents characteristically as hamartomatous polyposis throughout the GI tract and can present with diarrhea, GI tract hemorrhage, and protein-losing enteropathy.

Zhou XP, Woodford-Richens K, Lehtonen R, et al.: Germline mutations in BMPR1A/ALK3 cause a subset of cases of juvenile polyposis syndrome and of Cowden and Bannayan-Riley-Ruvalcaba syndromes.