spondyloepiphyseal dysplasia, Cantu type

disease
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Also known as SED-BDSspondyloepiphyseal dysplasia-brachydactyly-speech disorder syndrometattoo dysplasia

Summary

spondyloepiphyseal dysplasia, Cantu type (MONDO:0012716) is a disease. A subtype of spondyloepiphyseal dysplasia — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Prevalence: <1 / 1 000 000 (Worldwide) [Orphanet-validated]
  • Phenotypes (HPO): 39

Clinical features

Epidemiology

Prevalence records

2 prevalence record(s), Orphanet:

TypeClassValueGeographyValidation
Cases/families4WorldwideValidated
Point prevalence<1 / 1 000 000WorldwideValidated

Signs & symptoms

Clinical features (HPO)

39 HPO clinical features (Orphanet curated; top 39 by frequency):

HPO IDTermFrequency
HP:0000028CryptorchidismFrequent (30-79%)
HP:0000154Wide mouthFrequent (30-79%)
HP:0000174Abnormal palate morphologyFrequent (30-79%)
HP:0000179Thick lower lip vermilionFrequent (30-79%)
HP:0000215Thick upper lip vermilionFrequent (30-79%)
HP:0000306Abnormality of the chinFrequent (30-79%)
HP:0000343Long philtrumFrequent (30-79%)
HP:0000431Wide nasal bridgeFrequent (30-79%)
HP:0000463Anteverted naresFrequent (30-79%)
HP:0000470Short neckFrequent (30-79%)
HP:0000475Broad neckFrequent (30-79%)
HP:0000574Thick eyebrowFrequent (30-79%)
HP:0000581BlepharophimosisFrequent (30-79%)
HP:0000582Upslanted palpebral fissureFrequent (30-79%)
HP:0000767Pectus excavatumFrequent (30-79%)
HP:0001156BrachydactylyFrequent (30-79%)
HP:0001608Abnormality of the voiceFrequent (30-79%)
HP:0001832Abnormal metatarsal morphologyFrequent (30-79%)
HP:0002162Low posterior hairlineFrequent (30-79%)
HP:0002164Nail dysplasiaFrequent (30-79%)
HP:0002212Curly hairFrequent (30-79%)
HP:0002750Delayed skeletal maturationFrequent (30-79%)
HP:0002967Cubitus valgusFrequent (30-79%)
HP:0003026Short long boneFrequent (30-79%)
HP:0004322Short statureFrequent (30-79%)
HP:0004634Cuboid-shaped vertebral bodiesFrequent (30-79%)
HP:0005069Rhizo-meso-acromelic limb shorteningFrequent (30-79%)
HP:0005280Depressed nasal bridgeFrequent (30-79%)
HP:0005622Broad long bonesFrequent (30-79%)
HP:0006394Limited pronation/supination of forearmFrequent (30-79%)
HP:0007665Curly eyelashesFrequent (30-79%)
HP:0008496Multiple rows of eyelashesFrequent (30-79%)
HP:0008551MicrotiaFrequent (30-79%)
HP:0008839Hypoplastic pelvisFrequent (30-79%)
HP:0009103Aplasia/Hypoplasia involving the pelvisFrequent (30-79%)
HP:0009937Facial hirsutismFrequent (30-79%)
HP:0010306Short thoraxFrequent (30-79%)
HP:0011829Narrow philtrumFrequent (30-79%)
HP:0100625Enlarged thoraxFrequent (30-79%)

Identifiers

Disease identifiers

FieldValue
Canonical namespondyloepiphyseal dysplasia, Cantu type
Mondo IDMONDO:0012716
MeSHC567128
OMIM611717
Orphanet163654
DOIDDOID:0112287
ICD-11897226700
SNOMED CT718765003
UMLSC2673649
MedGen435975
GARD0010629
Is cancer (heuristic)no

Also known as: SED-BDS · spondyloepiphyseal dysplasia-brachydactyly-speech disorder syndrome · tattoo dysplasia

Disease family

This is a subtype of spondyloepiphyseal dysplasia. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › musculoskeletal system disorderskeletal system disorderbone disorderbone development diseaseosteochondrodysplasiaspondyloepiphyseal dysplasiaspondyloepiphyseal dysplasia, Cantu type

Related subtypes (43): hip dysplasia, Beukes type, spondyloepiphyseal dysplasia with congenital joint dislocations, Marshall syndrome, metatropic dysplasia, spondyloepiphyseal dysplasia with punctate corneal dystrophy, spondyloepiphyseal dysplasia, MacDermot type, progressive pseudorheumatoid arthropathy of childhood, otospondylomegaepiphyseal dysplasia, Dyggve-Melchior-Clausen disease, dyssegmental dysplasia, Rolland-Desbuquois type, Silverman-Handmaker type dyssegmental dysplasia, Wolcott-Rallison syndrome, Schimke immuno-osseous dysplasia, Richieri Costa-da Silva syndrome, Schwartz-Jampel syndrome, X-linked spondyloepimetaphyseal dysplasia, CODAS syndrome, spondyloepiphyseal dysplasia, Reardon type, brachyolmia-amelogenesis imperfecta syndrome, spondyloepiphyseal dysplasia with coronal craniosynostosis, cataracts, cleft palate, and intellectual disability, anauxetic dysplasia, spondyloepiphyseal dysplasia, Kimberley type, Ehlers-Danlos syndrome, spondylocheirodysplastic type, spondylo-megaepiphyseal-metaphyseal dysplasia, brachydactylous dwarfism, Mseleni type, TMEM165-congenital disorder of glycosylation, Steel syndrome, cataract-growth hormone deficiency-sensory neuropathy-sensorineural hearing loss-skeletal dysplasia syndrome, Roifman syndrome, progressive spondyloepimetaphyseal dysplasia-short stature-short fourth metatarsals-intellectual disability syndrome, even-plus syndrome, Smith-McCort dysplasia, cono-spondylar dysplasia, X-linked intellectual disability-cerebellar hypoplasia-spondylo-epiphyseal dysplasia syndrome, Stickler syndrome, spondyloepiphyseal dysplasia tarda, spondyloepiphyseal dysplasia, kondo-fu type, spondyloepiphyseal dysplasia, nishimura type, immunoskeletal dysplasia with neurodevelopmental abnormalities, COL2A1-related spondyloepiphyseal dysplasia, MIR140-related spondyloepiphyseal dysplasia, MGP-related spondyloepiphyseal dysplasia, spondyloepiphyseal dysplasia, Holling type

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 0.

No linked Atlas pages yet — the cross-entity mesh grows as the corpus expands.