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syndroom van primordiale dwerggroei met microcefalie en insulineresistentie (aandoening)
syndroom van primordiale dwerggroei met microcefalie en insulineresistentie
Microcephalic primordial dwarfism, insulin resistance syndrome
A rare genetic disease with characteristics of severe pre and postnatal growth failure with short stature and microcephaly, facial dysmorphism (including a small jaw and prominent midface), severe insulin resistance, fatty liver, hypertriglyceridemia developing in childhood and primary gonadal failure. Mild global learning difficulties and acanthosis nigricans have also been reported.
Id1220596009
StatusPrimitive
Has interpretationonder referentiebereik
InterpretsBody height
Associated morphologycongenitale kleinheid
Finding sitestructuur van hoofd
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
SNOMED CT to Orphanet simple map436182
SNOMED CT to ICD-10 extended map
TargetQ87.1
RuleTRUE
AdviceALWAYS Q87.1 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified