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The bone dysplasia is characterized by acromesomelia, i.e. relative shortening of the distal (acromelic) and middle (mesomelic) segments, as opposed to the proximal (rhizomelic) segments. This is especially demonstrated in the hand, where the distal and middle phalangeal segments are proportionately shorter than the proximal segments, which may interfere with the ability to make a tight fist.


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