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Lenz-Majewski hyperostotic dysplasia

ORPHA:2658· ICD-10 Q87.1

Definition

A rare primary bone dysplasia characterized by growth delay, generalized and progressive craniotubular hyperostosis, intellectual disability, distinct craniofacial appearance, dental anomalies, loose skin, and distal-limb anomalies.

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