Conditions / Genetic

short-rib thoracic dysplasia 14 with polydactyly

info · Genetic · ICD-10: Q04.3

An asphyxiating thoracic dystrophy that has_material_basis_in homozygous mutation in the KIAA0586 gene on chromosome 14q23.

Signs and symptoms

  • Molar tooth sign on MRI
  • Pulmonary hypoplasia
  • Generalized hypotonia
  • Narrow chest
  • Short ribs
  • Cerebellar vermis hypoplasia
  • Hyporeflexia
  • Cleft palate
  • Polyhydramnios
  • Postaxial polydactyly

Also known as: SRTD14