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retinale vasculopathie met cerebrale leuko-encefalopathie en systemische manifestaties (aandoening)
retinale vasculopathie met cerebrale leuko-encefalopathie en systemische manifestaties
RVCL-S
retinale vasculopathie met cerebrale leukodystrofie en systemische manifestaties
Retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations
RVCL-S - retinal vasculopathy, cerebral leukoencephalopathy, systemic manifestations
RVCL - retinal vasculopathy cerebral leukoencephalopathy
A rare genetic cerebral small vessel disease characterized by progressive loss of visual acuity due to retinal vasculopathy, in combination with more variable neurological signs and symptoms including stroke, cognitive decline, migraine-like headaches, and seizures, among others, typically beginning in middle age. Psychiatric features such as depression and anxiety may also occur. Systemic vascular involvement with Raynaud phenomenon, micronodular liver cirrhosis, and glomerular kidney dysfunction is present in a subset of patients.
Id783787000
StatusPrimitive
Clinical courseprogressief
Associated morphologyinflammatoire morfologie
Finding sitestructuur van immuunsysteem
Pathological processafwijkend immuunproces
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetH35.0
Term'Background' retinopathie en vasculaire veranderingen van retina
TargetG93.4
TermEncefalopathie, niet gespecificeerd
SNOMED CT to Orphanet simple map247691
SNOMED CT to ICD-10 extended map
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
TargetI77.8
RuleTRUE
AdviceALWAYS I77.8
CorrelationSNOMED CT source code to target map code correlation not specified