Home Endosteal hyperostosis: Worth type

Endosteal hyperostosis: Worth type


Defining Characteristics

BMD T-score >0, no fractures, nl bone remodeling, flat forehead, elongated mandible, toras palatinus (bony protrusion of palate), benign presentation
Heavy bones!

Disease Development

Disruption of Wnt signaling system causes decreased inhibition of Wnt, resulting in the inhibition of APC/GSK3 complex. Now, B-catenin is active and able to promote transcription of genes involved in osteoblast differentiation,
causing osteosclerosis

Potential Causes

Mutation in LRP5, prevents binding of DKK1 (Wnt inhibitor)

Risk factors

Autosomal dominant inheritance