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Severe early-childhood-onset retinal dystrophy

Disease definition

Severe early childhood onset retinal dystrophy (SECORD) is an inherited retinal dystrophy (see this term), characterized by a severe congenital night blindness, progressive retinal dystrophy and nystagmus. Best corrected visual acuity can reach 0.3 in the first decade of life and can pertain well into the second decade of life. Blindness is often complete by the age of 30 years. An overlap with Leber congenital amaurosis (LCA; see this term) occurs when patients are characterized by their visual acuity and panretinal dystrophy.

ORPHA:364055

  • Synonym(s):
    • EOSRD
    • Early-onset severe retinal dystrophy
    • SECORD
  • Prevalence: -
  • Inheritance: Autosomal recessive 
  • Age of onset: Childhood
  • ICD-10: H35.5
  • OMIM: -
  • UMLS: C1858080
  • MeSH: -
  • GARD: -
  • MedDRA: -

Additional information

Further information on this disease

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