Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome

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Overview

Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome is an extremely rare genetic condition that affects growth and metabolism. The name describes its three main features: babies are smaller than expected during pregnancy (intrauterine growth restriction or IUGR), children remain short in stature as they grow, and diabetes develops unusually early in adulthood. This syndrome is caused by changes in the NAPRT gene, which plays a role in how the body processes a vitamin called niacin (vitamin B3) and produces a key molecule called NAD+ that cells need for energy and many other functions. Because NAD+ is important throughout the body, this condition can affect multiple systems. In addition to growth problems and early diabetes, some individuals may experience other metabolic or developmental challenges. The condition is present from before birth, as the growth restriction begins in the womb. Treatment is currently focused on managing symptoms. Growth hormone therapy may be considered for short stature, and diabetes is managed with standard approaches including blood sugar monitoring, dietary changes, and medications such as insulin or oral diabetes drugs. Because this syndrome is so rare, there is limited long-term data on outcomes, and care is typically coordinated by a team of specialists including endocrinologists and clinical geneticists. Research into NAD+ metabolism may eventually lead to more targeted therapies.

Key symptoms:

Baby smaller than expected during pregnancyLow birth weightShort stature throughout childhood and into adulthoodDiabetes developing in early adulthoodSmall head size at birthDelayed growth milestonesHigh blood sugar levelsPossible feeding difficulties in infancyPossible reduced muscle mass

Clinical phenotype terms (3)— hover any for plain English
Inheritance

Autosomal dominant

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome.

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No actively recruiting trials found for Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome at this time.

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No specialists are currently listed for Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome.

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 Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome.

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How will my child's growth be monitored, and is growth hormone therapy an option?,At what age should we start screening for diabetes, and how often?,Are there any dietary supplements like niacin that might help with this condition?,What type of diabetes is expected, and what will the treatment plan look like?,Should other family members be tested for the NAPRT gene variant?,Are there any clinical trials or research studies we can participate in?,What specialists should be part of our care team going forward?

Common questions about Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome

What is Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome?

Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome is an extremely rare genetic condition that affects growth and metabolism. The name describes its three main features: babies are smaller than expected during pregnancy (intrauterine growth restriction or IUGR), children remain short in stature as they grow, and diabetes develops unusually early in adulthood. This syndrome is caused by changes in the NAPRT gene, which plays a role in how the body processes a vitamin called niacin (vitamin B3) and produces a key molecule called NAD+ that cells need for energy and

How is Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome inherited?

Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome typically begin?

Typical onset of Intrauterine growth restriction-short stature-early adult-onset diabetes syndrome is neonatal. Age of onset can vary across affected individuals.