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dentin dysplasia-sclerotic bones syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

dentin dysplasia-sclerotic bones syndrome

MONDO:0007438

Also known as:dentin dysplasia sclerotic bonesdentin dysplasia with sclerotic bonessclerotic bones with dentin dysplasia

Overview

A rare, genetic odontologic disease characterized by the clinical, radiographic, and histologic features of dentine dysplasia and osteosclerosis of all long bones, with heavy cortical bone and narrowed or occluded marrow spaces. There have been no further descriptions in the literature since 1977.

GeneticsAutosomal dominant

ResearchLimited published research (2 articles)

Clinical Presentation

Features include: Abnormality of the dentition, Cortical sclerosis, and Dentinogenesis imperfecta limited to primary teeth.

Explore clinical features

Prognosis

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for dentin dysplasia-sclerotic bones syndrome.

2 publications have been identified in PubMed for dentin dysplasia-sclerotic bones syndrome. Research spans Review / Meta-Analysis (50%) and Basic Science / Preclinical (50%).

Recent Publications

Abdel-Hamid MS (2025). [PMID: 40119123](https://pubmed.ncbi.nlm.nih.gov/40119123/). *Journal of human genetics*. [Basic Science / Preclinical]
Choi WJ (2024). [PMID: 39239241](https://pubmed.ncbi.nlm.nih.gov/39239241/). *World journal of radiology*. [Review / Meta-Analysis]

Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 6:58 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:125440

Orphanet

European rare disease database

Orphanet:99792

GARD

Genetic and Rare Diseases Info Center

GARD:1808

Questions for Your Care Team

→Are there specialists or centers of excellence for this condition in my area?
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dentin dysplasia-sclerotic bones syndrome

Overview

Clinical Presentation

Explore clinical features

Prognosis

Research Landscape

Recent Publications

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team