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Spondyloepimetaphyseal dysplasia, Missouri type

ORPHA:93356· ICD-10 Q77.7

Definition

Spondyloepimetaphyseal dysplasia, Missouri type is characterized by moderate-to-severe metaphyseal changes, mild epiphyseal involvement, rhizomelic shortening of the lower limbs with bowing of the femora and/or tibiae, coxa vara, genu varum and pear-shaped vertebrae in childhood.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant
Age of onset
Childhood, Infancy