Generalized juvenile polyposis/juvenile polyposis coli

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ORPHA:329971OMIM:174900D12.6
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Overview

Generalized juvenile polyposis, also called juvenile polyposis syndrome (JPS), is a rare inherited condition in which multiple non-cancerous growths called juvenile polyps develop throughout the digestive tract, particularly in the stomach, small intestine, and colon. The term "juvenile" refers to the type of polyp, not the age of the patient, though symptoms often begin in childhood or adolescence. Juvenile polyposis coli is a subtype where the polyps are mainly limited to the colon and rectum. These polyps can cause rectal bleeding, anemia (low red blood cell count from chronic blood loss), abdominal pain, diarrhea, and in some cases, the passage of polyps through the rectum. Over time, if left untreated, some of these polyps can develop into colorectal cancer or other gastrointestinal cancers. The lifetime risk of cancer in people with JPS is estimated to be between 10% and 50%, which is why regular monitoring is essential. Treatment focuses on surveillance with regular colonoscopies and upper endoscopies to find and remove polyps before they become cancerous. In severe cases where polyps are too numerous to manage with endoscopy alone, surgery to remove part or all of the colon may be necessary. Genetic counseling is recommended for affected families to help identify at-risk relatives early.

Key symptoms:

Rectal bleeding or blood in the stoolAnemia (low red blood cells) causing tiredness and palenessAbdominal pain or crampingDiarrheaPassing polyps through the rectumFailure to thrive or poor growth in childrenLow protein levels in the blood (protein-losing enteropathy)Swelling of the hands, feet, or face due to low proteinRectal prolapse (tissue pushing out of the rectum)Nausea or vomitingConstipationIron deficiencyIntussusception (part of the intestine sliding into another part)

Clinical phenotype terms (11)— hover any for plain English
HematocheziaHP:0002573Anemic pallorHP:0001017Adenomatous colonic polyposisHP:0005227Multiple gastric polypsHP:0004394Small intestinal polyposisHP:0030256Rectal polyposisHP:0100896Duodenal polyposisHP:0004783
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Generalized juvenile polyposis/juvenile polyposis coli.

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No actively recruiting trials found for Generalized juvenile polyposis/juvenile polyposis coli at this time.

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No specialists are currently listed for Generalized juvenile polyposis/juvenile polyposis coli.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Generalized juvenile polyposis/juvenile polyposis coli.

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Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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Family & Caregiver Grants

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene mutation do I or my child have, and does it affect the type of monitoring needed?,How often should colonoscopies and upper endoscopies be performed?,At what age should surveillance begin for my other children or family members?,What are the signs that surgery might become necessary instead of continued polyp removal?,Should I or my child be screened for hereditary hemorrhagic telangiectasia?,What dietary changes or supplements can help manage anemia?,Are there any clinical trials or new treatments being studied for this condition?

Common questions about Generalized juvenile polyposis/juvenile polyposis coli

What is Generalized juvenile polyposis/juvenile polyposis coli?

Generalized juvenile polyposis, also called juvenile polyposis syndrome (JPS), is a rare inherited condition in which multiple non-cancerous growths called juvenile polyps develop throughout the digestive tract, particularly in the stomach, small intestine, and colon. The term "juvenile" refers to the type of polyp, not the age of the patient, though symptoms often begin in childhood or adolescence. Juvenile polyposis coli is a subtype where the polyps are mainly limited to the colon and rectum. These polyps can cause rectal bleeding, anemia (low red blood cell count from chronic blood loss),

How is Generalized juvenile polyposis/juvenile polyposis coli inherited?

Generalized juvenile polyposis/juvenile polyposis coli follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.