Greig cephalopolysyndactyly syndrome

Dr Daniel J Bell and Radswiki et al.

The Greig cephalopolysyndactyly syndrome (GCPS) is a rare pleiotropic, multiple congenital anomaly syndrome. 

It is primarily characterized by:

Precise estimates of incidence are difficult to determine, as ascertainment is erratic (estimated range 1 - 9 per 1,000,000) 2.

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

David Middleton Grieg (1864-1936) Scottish surgeon, first described this syndrome in 1926 5.

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Article information

rID: 15078
Section: Syndromes
Tag: cases
Synonyms or Alternate Spellings:
  • Greig syndrome
  • Greig cephalopolysyndactyly syndrome (GCPS)
  • Greig cephalo-polysyndactyly syndrome (GCPS)
  • Greig cephalo-polysyndactyly syndrome

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