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Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (disorder)
Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations
RVCL-S - retinal vasculopathy, cerebral leukoencephalopathy, systemic manifestations
RVCL - retinal vasculopathy cerebral leukoencephalopathy
An inherited group of small vessel diseases comprised of cerebroretinal vasculopathy (CRV), hereditary vascular retinopathy (HRV) and hereditary endotheliopathy with retinopathy, nephropathy and stroke all exhibiting progressive visual impairment as well as variable cerebral dysfunction. There is evidence the disease is caused by heterozygous mutation in the TREX1 gene on chromosome 3p21.
retinale vasculopathie met cerebrale leuko-encefalopathie en systemische manifestaties
RVCL-S
retinale vasculopathie met cerebrale leukodystrofie en systemische manifestaties
Id783787000
StatusPrimitive
Pathological processAutoimmune process
Clinical courseProgressive
DHD Diagnosis thesaurus reference set
ICD-10 complex map reference set
TargetH35.0
RuleTRUE
AdviceALWAYS H35.0 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified
TargetG93.4
RuleTRUE
AdviceALWAYS G93.4
CorrelationSNOMED CT source code to target map code correlation not specified