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Severe early-childhood-onset retinal dystrophy

ORPHA:364055· ICD-10 H35.5

Definition

Severe early childhood onset retinal dystrophy (SECORD) is an inherited retinal dystrophy 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.

Prevalence
Unknown
Inheritance
Autosomal recessive
Age of onset
Childhood, Infancy