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Eiken syndrome; Eiken skeletal dysplasia; bone modeling defect of hands & feet

Epidemiology: rare Pathology: skeletal dysplasia Genetics: - autosomal recessive - associated with defects in PTHR1 Clinical manifestations: - multiple epiphyseal dysplasia - extremely retarded ossification, principally of the epiphyses, pelvis, hands & feet - abnormal modeling of the bones in hands & feet - abnormal persistence of cartilage in the pelvis - mild growth retardation

General

genetic syndrome (multisystem disorder) skeletal dysplasia

Database Correlations

OMIM 600002

References

UniProt :accession Q03431