FB2025_05 , released December 11, 2025

CV Term Report

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General Information
Term	progressive familial intrahepatic cholestasis	ID (Ontology)	DOID:0070221 (Human Disease)
Definition	An intrahepatic cholestasis characterized by early onset of chronic unremitting cholestasis of hepatocellular origin that progresses to hepatic fibrosis, cirrhosis, and end-stage liver disease before adulthood.
Also Known As	"Byler disease" ; "PFIC"
Comment
Links to External Ontologies
	DO.org
Annotations
Records annotated with this term OR any of its CHILD TERMS
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Records annotated with this exact term (annotations to child terms are NOT included)
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Full annotation statements including this term (annotations to child terms are NOT included), and relevant FlyBase records
No relevant statements available

Spanning Tree (Parents/Children)

  cholestasis
   |__intrahepatic cholestasis
       |__progressive familial intrahepatic cholestasis  7 rec.
           |__progressive familial intrahepatic cholestasis 1 1 rec.
           |__progressive familial intrahepatic cholestasis 2 1 rec.
           |__progressive familial intrahepatic cholestasis 3 4 rec.
           |__progressive familial intrahepatic cholestasis 4 1 rec.
           |__progressive familial intrahepatic cholestasis 5 1 rec.

Spanning Tree View Settings
Parents/Children View Depth	Show hierarchy levels: for parents, for children
Relationships
Is a	intrahepatic cholestasis
Part of
Synonyms & Secondary IDs
Synonyms
	"Byler disease" EXACT "PFIC" EXACT OMO:0003012
Secondary IDs

External Crossreferences & Linkouts
	MIM:PS211600 NCI:C84453 ORDO:172 SNOMEDCT_US_2023_03_01:74162007 UMLS_CUI:C0268312