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progressieve intrahepatische cholestase (aandoening)
progressieve intrahepatische cholestase
Progressive intrahepatic cholestasis
PFIC - progressive familial intrahepatic cholestasis
Fatal intrahepatic cholestasis
Progressive familial intrahepatic cholestasis
Familial intrahepatic cholestasis
A heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. Three types of PFIC have been identified and are related to mutations in hepatocellular transport system genes involved in bile formation. Main clinical manifestations include cholestasis, pruritus and jaundice.
Id74162007
StatusPrimitive
Clinical courseprogressief
referentieset voor pathologie
DHD Diagnosethesaurus-referentieset
referentieset met complexe door RIVM geautoriseerde nationale 'mapping' naar ICD-10 voor diagnosethesaurus
TargetK83.1
TermObstructie van galweg
referentieset met complexe 'mapping' naar ICD-10
TargetK83.1
RuleTRUE
AdviceALWAYS K83.1
CorrelationSNOMED CT source code to target map code correlation not specified
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