Greig-Syndrom

The Greig cephalopolysyndactyly syndrome (GCPS) is a rare pleiotropic, multiple congenital anomaly syndrome. It is primarily characterized by:

Epidemiology

Precise estimates of incidence are difficult to determine, as ascertainment is erratic (estimated range 1 - 9 per 1,000,000) .

Associations

Pathology

GCPS is caused by loss of function mutations in the GLI3 transcription factor gene and is inherited in an autosomal dominant pattern.

History and etymology

David Middleton Grieg (1864-1936) Scottish surgeon, first described this syndrome in 1926 .

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