Conditions / Syndrome

Meckel syndrome 3

info · Syndrome · ICD-10: Q61.9

A Meckel syndrome that has_material_basis_in an autosomal recessive mutation of the TMEM67 gene on chromosome 8q22.1.

Signs and symptoms

  • Multicystic kidney dysplasia
  • Malformation of the hepatic ductal plate
  • Occipital encephalocele
  • Postaxial hand polydactyly
  • Hepatic fibrosis
  • Polydactyly
  • Bile duct proliferation
  • Hepatomegaly
  • Cleft palate
  • Hydrocephalus

Also known as: MKS3; Meckel-Gruber syndrome, type 3