Odontomicronychial dysplasia

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ORPHA:1811OMIM:601319Q84.8
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What is Odontomicronychial dysplasia?

Odontomicronychial dysplasia is an extremely rare genetic condition that affects the development of teeth and nails. The name comes from 'odonto' (teeth), 'micro' (small), 'onychia' (nails), and 'dysplasia' (abnormal development). People with this condition typically have abnormally small or underdeveloped nails (micronychial) along with dental abnormalities, which may include missing teeth, small teeth, or teeth with abnormal shapes. Some affected individuals may also have mild abnormalities of the hair or skin, placing this condition within the broader group of ectodermal dysplasias — disorders that affect structures derived from the outer layer of the developing embryo. Because this condition is so rare, with only a handful of cases described in the medical literature, our understanding of the full range of symptoms and the best approaches to management is limited. There is currently no cure for odontomicronychial dysplasia. Treatment focuses on managing the specific symptoms each person experiences. Dental care is a major part of management, including dental prosthetics, restorations, or implants to replace missing or malformed teeth. Nail abnormalities are generally managed with protective care and cosmetic approaches. Affected individuals typically have a normal life expectancy, and the condition primarily impacts appearance and dental function rather than overall health.

Key symptoms:

Very small or underdeveloped fingernails and toenailsMissing teethAbnormally small teethTeeth with unusual shapesDelayed eruption of teethThin or brittle nailsDry skinFine or sparse hair

Clinical phenotype terms (9)— hover any for plain English
  • Ectodermal dysplasiaHP:0000968
  • Short nailHP:0001799
  • Thin nailHP:0001816
  • Premature loss of primary teethHP:0006323
  • Premature eruption of permanent teethHP:0006337
  • Slow-growing nailsHP:0008383
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 ↗OMIM ↗NORD ↗

Treatments

Source: openFDA + DailyMed · NDA / BLA labels with structured indications · refreshed weekly

No FDA-approved treatments are currently listed for Odontomicronychial dysplasia.

View clinical trials →

Source: ClinicalTrials.gov · synced daily · phases, status, and PI names normalized at ingest

No actively recruiting trials found for Odontomicronychial dysplasia at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Odontomicronychial dysplasia community →

Source: NPI Registry + PubMed · trial PI roles cross-referenced with ClinicalTrials.gov · ranked by match score (publications + PI activity + community signal)

No specialists are currently listed for Odontomicronychial dysplasia.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

Treatment Centers

8 centers

Source: NORD Rare Disease Centers + NIH Undiagnosed Diseases Network (UDN) · centers verified active within last 12 months

🏨 Children's

Children's Hospital Colorado Rare Disease Program

Children's Hospital Colorado

📍 Aurora, CO

👤 Boston Children's Hospital Rare Disease Program

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Boston Children's Hospital Rare Disease Program

Boston Children's Hospital

📍 Boston, MA

👤 Boston Children's Hospital Rare Disease Program

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

🏨 Children's

Ann & Robert H. Lurie Children's Hospital Genetics

Lurie Children's Hospital

📍 Chicago, IL

👤 Boston Children's Hospital Rare Disease Program

🏥 NORD

Cincinnati Children's Hospital Medical Center

Cincinnati Children's

📍 Cincinnati, OH

👤 Boston Children's Hospital Rare Disease Program

🏨 Children's

Nationwide Children's Hospital Rare Disease Center

Nationwide Children's Hospital

📍 Columbus, OH

👤 Boston Children's Hospital Rare Disease Program

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

Travel Grants

No travel grants are currently matched to Odontomicronychial dysplasia.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Odontomicronychial dysplasia

Source: PubMed + NIH RePORTER + openFDA + clinical-journal RSS · last 30 days · disease-tagged at ingest by AI extraction with human QC

No recent news articles for Odontomicronychial dysplasia.

Follow this condition to be notified when news becomes available.

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.What dental treatments will my child need, and when should they start?,Should we pursue genetic testing, and what might it tell us?,Are there other body systems we should monitor for problems?,What is the chance of passing this condition to future children?,Are dental implants an option, and at what age can they be placed?,Are there any specialists we should see regularly?,How can we support our child's self-esteem regarding their appearance?

Common questions about Odontomicronychial dysplasia

What is Odontomicronychial dysplasia?

Odontomicronychial dysplasia is an extremely rare genetic condition that affects the development of teeth and nails. The name comes from 'odonto' (teeth), 'micro' (small), 'onychia' (nails), and 'dysplasia' (abnormal development). People with this condition typically have abnormally small or underdeveloped nails (micronychial) along with dental abnormalities, which may include missing teeth, small teeth, or teeth with abnormal shapes. Some affected individuals may also have mild abnormalities of the hair or skin, placing this condition within the broader group of ectodermal dysplasias — disord

How is Odontomicronychial dysplasia inherited?

Odontomicronychial dysplasia follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Odontomicronychial dysplasia typically begin?

Typical onset of Odontomicronychial dysplasia is neonatal. Age of onset can vary across affected individuals.

Frequently asked questions about Odontomicronychial dysplasia

Auto-generated from canonical disease facts (Orphanet, OMIM, ClinicalTrials.gov, openFDA, NPPES). Not a substitute for clinical guidance.

  1. What is Odontomicronychial dysplasia?

    Odontomicronychial dysplasia is a rare disease catalogued in international rare-disease ontologies (Orphanet ORPHA:1811, OMIM 601319). It is typically inherited as autosomal dominant. Age of onset is generally neonatal. For verified primary sources, see the UniteRare Odontomicronychial dysplasia page.

  2. How is Odontomicronychial dysplasia inherited?

    Odontomicronychial dysplasia follows autosomal dominant inheritance. Genetic counseling is recommended for affected families to understand recurrence risk in offspring and the likelihood of unaffected siblings being carriers. Variants in the underlying gene(s) may be identified via clinical genetic testing.

  3. Are there FDA-approved treatments for Odontomicronychial dysplasia?

    Approved treatments for Odontomicronychial dysplasia are tracked from openFDA and DailyMed primary sources. Many rare diseases have no specific FDA-approved therapy; for those, supportive care and management of complications form the basis of clinical care. Orphan-drug-designation status is noted where applicable.

  4. Are there clinical trials for Odontomicronychial dysplasia?

    Active clinical trials for Odontomicronychial dysplasia are tracked daily from ClinicalTrials.gov. Trial availability changes frequently; check the UniteRare trial listings for the current count and recruitment status. Sponsors of rare-disease research often welcome inquiries even when a trial is not actively recruiting at a given moment.

  5. How do I find a specialist for Odontomicronychial dysplasia?

    Verified Odontomicronychial dysplasia specialists are identified through ClinicalTrials.gov principal-investigator records, peer-reviewed publication authorship (via PubMed), and the NPPES NPI registry. NORD-designated Centers of Excellence and NIH-affiliated rare-disease clinics are also tracked. UniteRare's specialist directory is updated continuously as new evidence becomes available.

See full Odontomicronychial dysplasia page for complete clinical details, sources, and verified-specialist listings.

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