Conditions / Genetic

short-rib thoracic dysplasia 7 with or without polydactyly

info · Genetic · ICD-10: Q77.2

An asphyxiating thoracic dystrophy that has_material_basis_in homozygous or compound heterozygous mutation in the WDR35 gene on chromosome 2p21.1.

Signs and symptoms

  • Epicanthus
  • Micromelia
  • Hypoplastic scapulae
  • Pulmonary hypoplasia
  • Short lingual frenulum
  • Renal hypoplasia
  • Brachydactyly
  • Short stature
  • Nail dysplasia
  • Hepatic fibrosis

Also known as: SRPS5; SRTD7; short rib-polydactyly syndrome type V