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Living with Dentin dysplasia type I

A rare form of dentin dysplasia in which teeth have very short, sharp roots or no roots.

Understanding Dentin dysplasia type I

Dentin dysplasia type I is a rare form of dentin dysplasia in which teeth have short, sharp, cone-shaped roots or no roots.

It affects how the dentin and tooth roots develop. GARD lists onset in childhood.

Signs & symptoms

GARD doesn't list a symptom list. Based on its summary, the main feature is:

  • Short, cone-shaped tooth roots or rootless teeth

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include genetic specialists and gum, teeth and mouth specialists.

  3. Consider genetic testing

    Changes in SMOC2 are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.

Care & treatment

Care team
Genetic specialists (geneticists); Gum, teeth and mouth specialists (odontologists)
Expert care
Ask about multidisciplinary care centers for rare conditions
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with rare disease patient organizations for support
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
How can we protect teeth that have short or no roots?
Can genetic testing of the SMOC2 gene help our family?
How often should dental X-rays and checkups be done?

Quick facts

Symptoms usually startChild
InheritanceAutosomal Dominant, Autosomal Recessive
GenesSMOC2

Changes in the SMOC2 gene are linked to this condition. GARD lists both autosomal dominant and autosomal recessive inheritance.

Genetic

Also known as: dd-i; dentin dysplasia, type 1; dentin dysplasia, type i; dentin dysplasia, type i, with microdontia and misshapen teeth; dentine dysplasia - shield's type i; dtdp1

Patient organizations

Rare-disease groups that can help

Source: NIH GARD · Dentin dysplasia type IGARD ID 1807 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Dentin dysplasia type I.

Explore the Dentin dysplasia type I blog

Explore research for Dentin dysplasia type I

Explore actively recruiting Dentin dysplasia type I studies with HealthSpark.

HealthSpark

Find actively recruiting studies for Dentin dysplasia type I with HealthSpark. Share your city to explore research near you.

  • Recruiting studies from ClinicalTrials.gov
  • Share your city to look for research near you
  • Eligibility is confirmed by the study team

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Powered by HealthSpark, our AI chat helper · Study information from ClinicalTrials.gov · Eligibility is confirmed by the study team.

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