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Severe oculo-renal-cerebellar syndrome (disorder)
Severe oculo-renal-cerebellar syndrome
ORC (oculo-renal-cerebellar) syndrome
Oculorenocerebellar syndrome
Hunter Jurenka Thompson syndrome
A rare multiple congenital anomalies/dysmorphic syndrome with characteristics of profound intellectual disability, choreoathetosis, progressive spastic diplegia, progressive tapetoretinal degeneration with loss of retinal vessels and glomerulopathy resulting in death late in the first or early in the second decade of life. Absence of the cerebellar granular layer has been reported.
Id1208341008
StatusPrimitive
Clinical courseProgressive
InterpretsMovement
Associated morphologyDegenerative abnormality
Finding siteRetinal structure
Finding siteLimb structure
ICD-10 complex map reference set
TargetQ87.8
RuleTRUE
AdviceALWAYS Q87.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified