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Asymmetric and symmetric long bone bowing in two sibs: An apparently new bone dysplasia

✍ Scribed by Moore, Laura A. ;Moore, Cynthia A. ;Smith, John A. ;Weaver, David D.


Publisher
John Wiley and Sons
Year
1993
Tongue
English
Weight
556 KB
Volume
47
Category
Article
ISSN
0148-7299

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✦ Synopsis


We describe 2 sibs, one with congenital asymmetric long bone bowing and one with congenital symmetric long bone bowing. Other bony abnormalities in these sibs include beaten metal appearance of the skull, dolichomacrocephaly, ocular hypertelorism, and anterior beaking and bone-within-bone appearance of vertebrae. A differential diagnosis including campomelic dysplasia, kyphomelic dysplasia, hypophosphatasia, Grant syndrome, and os- teogenesis imperfecta, a n d a discussion of potential mechanisms of long bone bowing are presented. The condition that these sibs have shares some characteristics of the above bone disorders, but appears to be a separate entity which to our knowledge has not been described previously.