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neonataal Marfan-syndroom (aandoening)
neonataal Marfan-syndroom
neonatale MFS
neonataal syndroom van Marfan
Neonatal Marfan syndrome
A rare severe and life-threatening genetic disease occurring during the neonatal period. The disease has characteristics of classical Marfan syndrome manifestations in addition to facial dysmorphism (megalocornea, iridodonesis, ectopia lentis, crumpled ears, loose redundant skin giving a 'senile' facial appearance), flexion joint contractures, pulmonary emphysema and a severe, rapidly progressive cardiovascular disease (including ascending aortic dilatation and severe mitral and/or tricuspid valve insufficiency). Additionally, skeletal manifestations (arachnodactyly, dolichostenomelia, pectus deformities) are also associated.
Id763839005
StatusPrimitive
Occurrenceneonataal
referentieset met complexe 'mapping' naar ICD-10
TargetQ87.4
RuleTRUE
AdviceALWAYS Q87.4 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified