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Orofaciodigital syndrome type 6

ORPHA:2754· ICD-10 Q04.3

Definition

Joubert syndrome with orofaciodigital defect (or oral-facial-digital syndrome type 6, OFD6) is a very rare subtype of Joubert syndrome and related disorders (JSRD) characterized by the neurological features of JS associated with orofacial anomalies and often polydactyly.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive, X-linked recessive
Age of onset
Infancy, Neonatal