||||
Raine-dysplasie (aandoening)
Raine-dysplasie
Raine-syndroom
letale osteosclerotische botdysplasie
syndroom van Raine
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.
Id389239007
StatusPrimitive
Associated morphologydysplasie
Finding sitebotstructuur
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Has interpretationboven referentiebereik
Interpretsbotdensitometrie
SNOMED CT to Orphanet simple map1832
SNOMED CT to ICD-10 extended map
TargetQ78.2
RuleTRUE
AdviceALWAYS Q78.2
CorrelationSNOMED CT source code to target map code correlation not specified