progressieve intrahepatische cholestase
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progressieve familiaire intrahepatische cholestase type 4 (aandoening)
progressieve familiaire intrahepatische cholestase type 4
PFIC4
Progressive familial intrahepatic cholestasis type 4
PFIC4 - progressive familial intrahepatic cholestasis type 4
Progressive familial intrahepatic cholestasis type IV
TJP2 deficit
Disease with characteristics of early childhood onset of severe progressive liver disease. Caused by homozygous or compound heterozygous mutation in the TJP2 gene on chromosome 9q21.
Id1295517006
StatusPrimitive
Clinical courseprogressief
Finding sitestructuur van intrahepatische galwegen
Occurrencevroege kinderleeftijd
SNOMED CT to ICD-10 extended map
TargetK76.8
RuleTRUE
AdviceALWAYS K76.8
CorrelationSNOMED CT source code to target map code correlation not specified