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Spondylometaphyseal dysplasia, Sedaghatian type

ORPHA:93317· ICD-10 Q77.8

Definition

A rare neonatal lethal form of spondylometaphyseal dysplasia characterized by severe metaphyseal chondrodysplasia, mild rhizomelic shortness of the upper limbs, and mild platyspondyly.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Antenatal, Neonatal