MULTIPLE SYNOSTOSES SYNDROME 3

General Information (adopted from Orphanet):

Synonyms, Signs: SYNS3
Number of Symptoms 5
OrphanetNr:
OMIM Id: 612961
ICD-10:
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Autosomal dominant inheritance
[Omim]
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: No data available.

Symptom Information: Sort by abundance 

1
(HPO:0002967) Cubitus valgus 19589401 IBIS 49 / 7739
2
(HPO:0001440) Metatarsal synostosis 19589401 IBIS 4 / 7739
3
(HPO:0009701) Metacarpal synostosis 19589401 IBIS 4 / 7739
4
(HPO:0003041) Humeroradial synostosis 19589401 IBIS 19 / 7739
5
(HPO:0000006) Autosomal dominant inheritance 2518 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Clinical Description OMIM Wu et al. (2009) described 12 affected individuals from a 5-generation Chinese family segregating autosomal dominant multiple synostoses syndrome, with fusions of proximal interphalangeal, carpal-tarsal, and humeroradial joints. Hearing, stature, and intelligence were normal in all affected individuals. ...
Molecular genetics OMIM In a 5-generation Chinese family with autosomal dominant multiple synostoses syndrome mapping to chromosome 13q11-q12, Wu et al. (2009) identified a heterozygous missense mutation in the candidate FGF9 gene (600921.0001) that segregated with disease and was not found ...