𝔖 Bobbio Scriptorium
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Unilateral partial tibia defect with preaxial polydactyly, general micromelia, and trigonomacrocephaly with a note on “developmental resistance”

✍ Scribed by Wiedemann, Hans-Rudolf ;Opitz, John M.


Publisher
John Wiley and Sons
Year
1983
Tongue
English
Weight
335 KB
Volume
14
Category
Article
ISSN
0148-7299

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✦ Synopsis


We report a boy with predominantly unilateral severe tibia defect with a high grade of preaxial polydactyly . Family history suggests the possibility of autosomal dominant inheritance with reduced penetrance and quite variable expressivity . The boy's phenotype and other previously reported examples of predominantly unilateral involvement in autosomal dominant and autosomal recessive limb mutations strongly suggest a hypothesis of developmental resistance in the uninvolved parts.