Multinodular goiter-cystic kidney-polydactyly syndrome

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ORPHA:2091OMIM:138790Q87.8
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Overview

Multinodular goiter-cystic kidney-polydactyly syndrome is an extremely rare genetic condition that affects multiple body systems at the same time. As the name suggests, the three main features of this syndrome are multinodular goiter (multiple lumps or nodules that form in the thyroid gland in the neck), cystic kidneys (fluid-filled sacs that develop in the kidneys), and polydactyly (being born with extra fingers or toes). The thyroid nodules can lead to an enlarged thyroid gland, which may affect how the thyroid works and can sometimes cause problems with hormone levels. The kidney cysts can vary in severity and may affect how well the kidneys filter waste from the blood. The extra digits are usually noticed at birth and can occur on the hands, feet, or both. Because this syndrome is so rare, there are very few reported cases in the medical literature. Treatment is based on managing each symptom individually. Thyroid nodules may be monitored with ultrasound and treated with medication or surgery if needed. Kidney cysts require regular monitoring of kidney function, and polydactyly can often be corrected with surgery. A team of different specialists is usually needed to provide the best care for people with this condition. The syndrome is also sometimes referred to by its Orphanet designation ORPHA:2091.

Also known as:

Key symptoms:

Multiple lumps or nodules in the thyroid glandEnlarged thyroid gland (goiter)Fluid-filled cysts in the kidneysExtra fingers or toes at birthPossible thyroid hormone imbalancePossible reduced kidney functionSwelling in the neck areaDifficulty swallowing if goiter is largeHigh blood pressure from kidney problemsFatigue related to thyroid or kidney dysfunction

Clinical phenotype terms (5)— hover any for plain English
Preaxial foot polydactylyHP:0001841Multinodular goiterHP:0005987
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

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 Multinodular goiter-cystic kidney-polydactyly syndrome.

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No actively recruiting trials found for Multinodular goiter-cystic kidney-polydactyly syndrome at this time.

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No specialists are currently listed for Multinodular goiter-cystic kidney-polydactyly 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 Multinodular goiter-cystic kidney-polydactyly syndrome.

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Community

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Latest news about Multinodular goiter-cystic kidney-polydactyly syndrome

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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.How severe are the thyroid nodules and kidney cysts in my case or my child's case?,How often should we have thyroid and kidney imaging done?,Is genetic testing recommended for our family, and what might it tell us?,What signs should I watch for that would mean the condition is getting worse?,When is the best time to have surgery for the extra fingers or toes?,Are there any dietary or lifestyle changes that could help protect kidney function?,Should other family members be screened for this condition?

Common questions about Multinodular goiter-cystic kidney-polydactyly syndrome

What is Multinodular goiter-cystic kidney-polydactyly syndrome?

Multinodular goiter-cystic kidney-polydactyly syndrome is an extremely rare genetic condition that affects multiple body systems at the same time. As the name suggests, the three main features of this syndrome are multinodular goiter (multiple lumps or nodules that form in the thyroid gland in the neck), cystic kidneys (fluid-filled sacs that develop in the kidneys), and polydactyly (being born with extra fingers or toes). The thyroid nodules can lead to an enlarged thyroid gland, which may affect how the thyroid works and can sometimes cause problems with hormone levels. The kidney cysts can

How is Multinodular goiter-cystic kidney-polydactyly syndrome inherited?

Multinodular goiter-cystic kidney-polydactyly syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.