Aase-Smith syndrome
Related people
A familial deformity syndrome of variable expressivity, characterised by congenital hypoplastic anaemia and connatal triphalangy of the thumbs. Other features may be hydrocephalus with Dandy-Walker anomaly, cleft palate, and multiple contractures of the joints, narrow shoulders. Occur in males; present from birth. The syndrome was originally described in two male siblings. Some writers consider this and the Diamond-Blackfan syndrome to be the same entity. Inheritance is probably autosomal recessive.
Bibliography
- J. M Aase, D. W. Smith:
Congenital anemia and triphalangeal thumbs: a new syndrome.
Journal of Pediatrics, St. Louis, 1969, 74: 471-474. - J. M Aase, D. W. Smith:
Dysmorphogenesis of joints, brain, and palate. A new inherited syndrome.
Journal of Pediatrics, St. Louis, 1968, 73: 606-609.