Juvenile polyposis of infancy

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ORPHA:79076OMIM:175050D12.6
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2Specialists8Treatment centers

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Overview

Juvenile polyposis of infancy (also called JPI or infantile juvenile polyposis) is a very rare and serious condition where many small growths called polyps form throughout the digestive tract — including the stomach, small intestine, and large intestine — in babies and very young children. Unlike the more common form of juvenile polyposis that appears in older children or adults, this form starts in the first months of life and tends to be much more severe. The polyps can bleed, block the gut, and prevent the body from absorbing the nutrients it needs to grow. Babies with this condition often have severe diarrhea, blood in their stools, and fail to gain weight normally. They may also lose large amounts of protein through the gut, leading to swelling and serious nutritional problems. In some cases, the condition is linked to changes in a gene called PTEN or SMAD4, and it can occur alongside other abnormalities in the body. Treatment focuses on managing symptoms, providing nutrition support, and removing polyps through endoscopy or surgery. Because the disease is so widespread and starts so early, it is very difficult to treat and can be life-threatening. Families need close support from a team of specialists to manage this complex condition.

Also known as:

Key symptoms:

Severe, ongoing diarrheaBlood in the stool (red or dark tarry stools)Failure to gain weight or grow normallySwelling of the bellySwelling of the legs or body due to low protein levelsVomitingAnemia (low red blood cell count causing paleness and tiredness)Loss of protein through the gut (protein-losing enteropathy)Rectal prolapse (part of the bowel pushing out through the bottom)Malnutrition and vitamin deficienciesIntestinal obstruction or blockage

Clinical phenotype terms (43)— hover any for plain English
Protein-losing enteropathyHP:0002243Subcutaneous lipomaHP:0001031HemangioblastomaHP:0010797Large foreheadHP:0002003Hamartomatous polyposisHP:0004390HematocheziaHP:0002573
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Juvenile polyposis of infancy.

View clinical trials →

No actively recruiting trials found for Juvenile polyposis of infancy at this time.

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Specialists

2 foundView all specialists →
KM
Kim E. Nichols, MD
Memphis, Tennessee
Specialist

Rare Disease Specialist

PI on 2 active trials
RM
Randall E Brand, MD
PITTSBURGH, PA
Specialist
PI on 1 active trial

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 Juvenile polyposis of infancy.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Juvenile polyposis of infancy

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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

Financial assistance programs specifically for caregivers of rare disease patients.

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.What genetic tests should my child have, and what do the results mean for our family?,How often will my child need endoscopy or colonoscopy to check on the polyps?,What nutritional support does my child need, and how will we manage feeding at home?,What warning signs should make me bring my child to the emergency room right away?,Are there any clinical trials or research studies my child might be eligible for?,What is the long-term plan for managing this condition as my child grows?,Should other family members be tested for related genetic conditions?

Common questions about Juvenile polyposis of infancy

What is Juvenile polyposis of infancy?

Juvenile polyposis of infancy (also called JPI or infantile juvenile polyposis) is a very rare and serious condition where many small growths called polyps form throughout the digestive tract — including the stomach, small intestine, and large intestine — in babies and very young children. Unlike the more common form of juvenile polyposis that appears in older children or adults, this form starts in the first months of life and tends to be much more severe. The polyps can bleed, block the gut, and prevent the body from absorbing the nutrients it needs to grow. Babies with this condition often

At what age does Juvenile polyposis of infancy typically begin?

Typical onset of Juvenile polyposis of infancy is infantile. Age of onset can vary across affected individuals.

Which specialists treat Juvenile polyposis of infancy?

2 specialists and care centers treating Juvenile polyposis of infancy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.