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ulnar hypoplasia-split foot syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

ulnar hypoplasia-split foot syndrome

MONDO:0010750

Also known as:Van den Berghe-Dequecker syndromeulnar hypoplasia-lobster-claw deformity of feet syndromeVan De Berghe Dequeker syndrome

Overview

Ulnar hypoplasia-split foot syndrome is characterized by the association of severe ulnar hypoplasia, absence of fingers two to five, and split-foot. It has been described in four males belonging to two generations of the same family. X-linked recessive inheritance is suggested, but autosomal dominant transmission cannot be excluded.

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 ulnar hypoplasia-split foot syndrome.

16 publications have been identified in PubMed for ulnar hypoplasia-split foot syndrome. Research spans Case Report / Case Series (60%), Review / Meta-Analysis (13%), and Clinical Trial Publication (13%).

Research Type	Count	% of Total
Patient case studies	9	60%

Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 10:36 AM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:314360

Orphanet

European rare disease database

Orphanet:1122

GARD

Genetic and Rare Diseases Info Center

GARD:5400

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ulnar hypoplasia-split foot syndrome

Overview

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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