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progressieve niet-infectieuze anterieure vertebrale fusie (aandoening)
progressieve niet-infectieuze anterieure vertebrale fusie
syndroom van Kopenhagen
Progressive non-infectious anterior vertebral fusion
Copenhagen syndrome
Progressive non-infectious anterior vertebral fusion (PAVF) is an early childhood spinal disorder with characteristics of the gradual onset of thoracic and/or lumbar spine ankylosis often in conjunction with kyphosis with distinctive radiological features. Prevalence is unknown, but PAVF (mostly isolated cases) has been reported in approximately 80-100 cases. Neurological abnormalities are exceptional. PAVF can occur isolated or less frequently, as part of a syndrome. Syndrome associated manifestations include facial dysmorphism, absence of one cervical vertebrae, radio-ulnar synostosis, exostosis.
Id719268008
StatusPrimitive
Associated morphologydysplasie
Finding sitebotstructuur
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetQ76.4
TermOverige congenitale misvormingen van wervelkolom, niet verband houdend met scoliose
SNOMED CT to Orphanet simple map2062
SNOMED CT to ICD-10 extended map
TargetQ76.4
RuleTRUE
AdviceALWAYS Q76.4 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified