Conditions / Syndrome

plasminogen deficiency type I

info · Syndrome · ICD-10: E88.02

A syndrome characterized by decreased serum plasminogen activity, decreased plasminogen antigen levels, and chronic mucosal pseudomembranous lesions typically manifesting as ligneous conjunctivitis that has_material_basis_in homozygous or compound heterozygous

A syndrome characterized by decreased serum plasminogen activity, decreased plasminogen antigen levels, and chronic mucosal pseudomembranous lesions typically manifesting as ligneous conjunctivitis that has_material_basis_in homozygous or compound heterozygous mutation in PLG on chromosome 6q26.

Signs and symptoms

  • Conjunctivitis
  • Hydrocephalus
  • Decreased circulating plasminogen concentration
  • Macrocephaly
  • Gingivitis
  • Cerebellar hypoplasia
  • Generalized hypotonia
  • Periodontitis
  • Blindness
  • Dandy-Walker malformation

Also known as: hypoplasminogenemia