Juvenile polyposis syndrome

Overview

What is juvenile polyposis?

Juvenile polyposis syndrome (JPS) is a medical condition characterized by an increased likelihood to have noncancerous growths called juvenile polyps in the large intestine (colon) and other parts of the gastrointestinal (GI) tract such as the stomach, small intestine, and rectum (the lower part and end of the colon that connects to the anus). Most people living with JPS will have some polyps by age 20. The number of polyps found in someone living with JPS can vary greatly from a few polyps to more than 100 over the course of a lifetime.

Juvenile polyps in GI tract may be silent and not cause any signs they are present; however, more often they will result in medical issues such as abdominal pain, loose stools (diarrhea), GI bleeding, and a decreased amount of healthy red blood cells (anemia). Although the polyps themselves are noncancerous (or benign) there is a higher risk for people with JPS to develop a GI cancer. Studies have found a 10 to 50 percent risk of developing a cancer of the gastrointestinal tract, most often colorectal cancer in people living with JPS.

The "juvenile" in the name of JPS does not necessarily predict the timing that a person living with the condition will develop polyps, instead it is describing the type of polyps. Juvenile polyps are formed from normal colonic or GI tissue that has become rearranged in a haphazard manner to form a sphere shaped head attached to the tissue lining the GI tract by a long narrow stalk. There can be breaks in the surface of the polyp that lead to the symptom of GI bleeding. Juvenile polyps as part of JPS or as single growths do often appear in childhood, but they can also arise in adults.

Single juvenile polyps are relatively common and having one polyp does not mean an individual has JPS. JPS is formally diagnosed when an individual has any one of the following:

  • Five or more juvenile polyps in the colon (large intestine) or rectum (the lower part and end of the colon that connects to the anus)
  • Juvenile polyps in the GI tract that are not the colon or rectum
  • Any number of juvenile polyps combined with one or more family members with JPS
  • Identification of at least one disease causing change (pathogenic variant) in the BMPR1A or SMAD4 gene

JPS is most often caused by genetic changes (pathogenic variants) in the BMPR1A gene or SMAD4 gene. These gene changes can be inherited from a parent or be found in a person for the first time (de novo. JPS can also be found as part of a larger pattern of health problems that happen more frequently together (a syndrome) such as Juvenile polyposis/hereditary hemorrhagic telangiectasia syndrome (JPS/HHT syndrome).

References
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Are there other names for juvenile polyposis?

How common is Juvenile polyposis syndrome?

What's the usual abbreviation for juvenile polyposis syndrome?

What do doctors call juvenile polyposis syndrome?

What are the most common health problems in juvenile polyposis syndrome?

What type of syndrome is juvenile polyposis syndrome?

Is juvenile polyposis syndrome more common in people from certain countries?

Are there other names for juvenile polyposis?

Juvenile polyposis syndrome (JPS) is known by several other names including:

  1. JPS
  2. JIP
  3. Juvenile polyposis syndrome
  4. Juvenile intestinal polyposis
  5. Juvenile gastrointestinal polyposis
References
How common is Juvenile polyposis syndrome?

It is believed that about between 1 in 16,000 people and 1 in 100,000 people are living with Juvenile polyposis syndrome.

References
  • Larsen Haidle J, Howe JR. Juvenile Polyposis Syndrome. 2003 May 13 [Updated 2017 Mar 9]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2019. Available from: https://www.ncbi.nlm.nih.gov/books/NBK1469/[/link
What's the usual abbreviation for juvenile polyposis syndrome?

Juvenile polyposis syndrome is commonly abbreviated as JPS. Less commonly, it may be abbreviated as JIP, which stands for juvenile intestinal polyps.

References
  • Accessed on November 14, 2019: Genetics Home Reference, Juvenile Polyposis Syndrome (https://ghr.nlm.nih.gov/condition/juvenile-polyposis-syndrome)
What do doctors call juvenile polyposis syndrome?

Juvenile polyposis syndrome is not called anything differently by doctors. If you mention juvenile polyposis syndrome to your doctor, they should know what you are referring to.

What are the most common health problems in juvenile polyposis syndrome?

The most common health problem associated with juvenile polyposis syndrome is the development of juvenile polyps in the GI tract, particularly the colon or rectum. These polyps can cause bleeding and an increased risk of developing GI cancer.

References
  • Accessed on November 14, 2019: Joy Larsen Haidle, MS, CGC and James R Howe, MD; GeneReviews, Juvenile Polyposis Syndrome (https://www.ncbi.nlm.nih.gov/books/NBK1469/)
  • Cohen, Shlomi, et al. (2019) Management of Juvenile Polyposis Syndrome in Children and Adolescents A Position Paper From the ESPGHAN Polyposis Working Group. Journal of Pediatric Gastroenterology and Nutrition (2019) 68:3, 453-462.
What type of syndrome is juvenile polyposis syndrome?

Juvenile polyposis syndrome is considered a cancer syndrome because it carries risks of cancer if left untreated. Additionally, screening for these specific types of cancers are available for those who have a clinical or genetic diagnosis of juvenile polyposis syndrome.

References
  • Accessed on November 14, 2019: Genetics Home Reference, Juvenile Polyposis Syndrome (https://ghr.nlm.nih.gov/condition/juvenile-polyposis-syndrome)
Is juvenile polyposis syndrome more common in people from certain countries?

As of 2020, there is not data showing that juvenile polyposis syndrome is more common in certain parts of the world over others.

References
  • Accessed on November 14, 2019: Joy Larsen Haidle, MS, CGC and James R Howe, MD; GeneReviews, Juvenile Polyposis Syndrome (https://www.ncbi.nlm.nih.gov/books/NBK1469/)

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