CHST3-related skeletal dysplasia

CHST3-related skeletal dysplasia is a very rare bone disorder characterized clinically by short stature of prenatal onset; dislocation of the knees, hips or elbows; club feet; limitation of range of motion of large joints; progressive kyphosis; and occasional scoliosis. In a few patients, minor heart valve dysplasia has also been described. Intellect, vision and hearing are normal.

Rhizomelia

Disproportionate shortening of the proximal segment of limbs (i.e. the femur and humerus).


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