| Progressive intrahepatic cholestasis (disorder) | | 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. | | progressieve intrahepatische cholestase |
| | Id | 74162007 | | Status | Primitive |
| PALGA thesaurus simple reference set for pathology |
| DHD Diagnosis thesaurus reference set |
| ICD-10 complex map reference set | | Target | K83.1 | | Rule | TRUE | | Advice | ALWAYS K83.1 | | Correlation | SNOMED CT source code to target map code correlation not specified |
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