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Raine dysplasia (disorder)
Raine dysplasia
Lethal osteosclerotic bone dysplasia
Raine syndrome
A rare disorder defined by generalized osteosclerosis with periosteal bone formation, characteristic facial dysmorphism, brain abnormalities including intracerebral calcifications, and neonatal lethal course. Mutations in the FAM20C gene have a causative role in lethal osteosclerotic bone dysplasia. The condition is transmitted in an autosomal recessive manner.
Raine-dysplasie
Raine-syndroom
letale osteosclerotische botdysplasie
syndroom van Raine
Id389239007
StatusPrimitive
Associated morphologyDysplasia
Finding siteBone structure
OccurrenceCongenital
Pathological processPathological developmental process
Has interpretationAbove reference range
InterpretsBone density scan
ICD-10 complex map reference set
TargetQ78.2
RuleTRUE
AdviceALWAYS Q78.2
CorrelationSNOMED CT source code to target map code correlation not specified