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Ulna hypoplasia-intellectual disability syndrome

ORPHA:2249· ICD-10 Q87.2

Definition

A rare genetic multiple congenital anomalies syndrome characterized by mesomelic shortness of the forearms mainly due to bilateral symmetrical ulnar hypoplasia, bilateral clubfeet, anonychia congenita (absent toe and finger nails), limited knee and elbow movement, severe varus deformity and severe intellectual disability. There have been no further descriptions in the literature since 1995.

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