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Microcephalic osteodysplastic primordial dwarfism types I and III

ORPHA:2636· ICD-10 Q87.1

Definition

A rare, severe, primary bone dysplasia characterized by intrauterine and postnatal growth retardation, microcephaly, facial dysmorphism, skeletal dysplasia, low-birth weight and brain anomalies.

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