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Thiel-Behnke corneal dystrophy

Disease definition

Thiel-Behnke corneal dystrophy (TBCD) is a rare form of superficial corneal dystrophy characterized by sub-epithelial honeycomb-shaped corneal opacities in the superficial cornea, and progressive visual impairment.

ORPHA:98960

  • Synonym(s):
    • Anterior limiting membrane dystrophy type II
    • Corneal dystrophy of Bowman layer type II
    • Curly fiber corneal dystrophy
    • Honeycomb corneal dystrophy
    • TBCD
    • Waardenburg-Jonker corneal dystrophy
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant 
  • Age of onset: Adolescent, Childhood, Adult
  • ICD-10: H18.5
  • OMIM: 602082
  • UMLS: C1562894
  • MeSH: C535942
  • GARD: 9275
  • MedDRA: -

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