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'brittle cornea'-syndroom (aandoening)
'brittle cornea'-syndroom
BCS
Brittle cornea syndrome
Ehlers-Danlos syndrome type 6B
Brittle cornea syndrome (BCS) is a form of Ehlers-Danlos syndrome with characteristics of severe ocular manifestations due to extreme corneal thinning and fragility with rupture in the absence of significant trauma. BCS generally progresses to blindness.
Id719096006
StatusPrimitive
Associated morphologymorfologische afwijking
Finding sitestructuur van cornea
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologydysplasie
Finding sitebotstructuur
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologydysplasie
Finding sitestructuur van huid
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Has interpretationdun
InterpretsCorneal thickness
DHD Diagnosis thesaurus reference set
SNOMED CT to Orphanet simple map90354
SNOMED CT to ICD-10 extended map
TargetQ79.6
RuleTRUE
AdviceALWAYS Q79.6
CorrelationSNOMED CT source code to target map code correlation not specified