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letaal congenitaal contractuursyndroom type 1 (aandoening)
letaal congenitaal contractuursyndroom type 1
ziekte van Herva
LCCS1
arthrogryposis multiplex congenita van Fins type
Lethal congenital contracture syndrome type 1
Multiple contracture syndrome Finnish type
Herva disease
An autosomal recessive disorder that is the most severe, neonatally lethal form of arthrogryposis a disorder characterized by congenital nonprogressive joint contractures. The contractures can involve the upper or lower limbs and/or the vertebral column, leading to various degrees of flexion or extension limitations evident at birth.
Id715418007
StatusPrimitive
Associated morphologycontractuur
Finding sitestructuur van gewrichtsregio
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Has interpretationverlaagd
Interprets‘range of motion’ van gewricht
referentieset met complexe 'mapping' naar ICD-10
TargetQ68.8
RuleTRUE
AdviceALWAYS Q68.8
CorrelationSNOMED CT source code to target map code correlation not specified