74162007: Progressive intrahepatic cholestasis (disorder)

SNOMED CT Concept\Clinical finding (finding)\...

\Viscus structure finding (finding)\Liver finding\...

\Disease of liver\Chronic liver disease\Progressive intrahepatic cholestasis
\Disease of liver\Metabolic and genetic disorder affecting the liver\Progressive intrahepatic cholestasis

\Cholestasis\Intrahepatic cholestasis\Progressive intrahepatic cholestasis

\Finding of trunk structure\Liver finding\Disease of liver\Chronic liver disease\Progressive intrahepatic cholestasis
\Finding of trunk structure\Liver finding\Disease of liver\Metabolic and genetic disorder affecting the liver\Progressive intrahepatic cholestasis
\Finding of trunk structure\Liver finding\Cholestasis\Intrahepatic cholestasis\Progressive intrahepatic cholestasis
\Finding of trunk structure\Disorder of trunk (disorder)\Disease of liver\Chronic liver disease\Progressive intrahepatic cholestasis
\Finding of trunk structure\Disorder of trunk (disorder)\Disease of liver\Metabolic and genetic disorder affecting the liver\Progressive intrahepatic cholestasis

\Disease\Genetic disease\Hereditary disease\Autosomal hereditary disorder\Autosomal recessive hereditary disorder\Progressive intrahepatic cholestasis
\Disease\Disorder of body system\Disease of liver\Chronic liver disease\Progressive intrahepatic cholestasis
\Disease\Disorder of body system\Disease of liver\Metabolic and genetic disorder affecting the liver\Progressive intrahepatic cholestasis
\Disease\Disorder of trunk (disorder)\Disease of liver\Chronic liver disease\Progressive intrahepatic cholestasis
\Disease\Disorder of trunk (disorder)\Disease of liver\Metabolic and genetic disorder affecting the liver\Progressive intrahepatic cholestasis
\Disease\Metabolic disease\Metabolic and genetic disorder affecting the liver\Progressive intrahepatic cholestasis
\Disease\Metabolic disease\Disorder of bilirubin metabolism\Progressive intrahepatic cholestasis

Status: current. Date: 31-Jan 2002

Descriptions:

Id Description Lang Type Status Case? Module

123149016 Progressive intrahepatic cholestasis en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
123150016 Byler syndrome en Synonym (core metadata concept) Inactive Entire term case sensitive (core metadata concept) SNOMED CT core
123151017 Familial intrahepatic cholestasis en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
123152012 Fatal intrahepatic cholestasis en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
814631018 Progressive intrahepatic cholestasis (disorder) en Fully specified name Active Entire term case insensitive (core metadata concept) SNOMED CT core
4570357019 Progressive familial intrahepatic cholestasis en Synonym (core metadata concept) Active Entire term case insensitive (core metadata concept) SNOMED CT core
4570358012 PFIC - progressive familial intrahepatic cholestasis en Synonym (core metadata concept) Active Entire term case sensitive (core metadata concept) SNOMED CT core
4570359016 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. en Definition Inactive Entire term case sensitive (core metadata concept) SNOMED CT core
5449630011 Progressive familial intrahepatic cholestasis (PFIC) refers to a heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. en Definition Active Entire term case sensitive (core metadata concept) SNOMED CT core

Expanded Value Set

Outbound Relationships	Type	Target	Active	Characteristic	Refinability	Group	Values
Progressive intrahepatic cholestasis	Is a	Metabolic and genetic disorder affecting the liver	true	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly
Progressive intrahepatic cholestasis	Is a	Chronic liver disease	true	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly
Progressive intrahepatic cholestasis	Is a	Intrahepatic cholestasis	true	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly
Progressive intrahepatic cholestasis	Is a	Disorder of bilirubin metabolism	true	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly
Progressive intrahepatic cholestasis	Is a	Autosomal recessive hereditary disorder	true	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly
Progressive intrahepatic cholestasis	Clinical course	Progressive	true	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly	1
Progressive intrahepatic cholestasis	Finding site	Intrahepatic biliary tract structure	true	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly	2

Inbound Relationships	Type	Active	Source	Characteristic	Refinability	Group
Progressive familial intrahepatic cholestasis type 4 (disorder)	Is a	True	Progressive intrahepatic cholestasis	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly
Progressive familial intrahepatic cholestasis type 3	Is a	True	Progressive intrahepatic cholestasis	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly
North American Indian childhood cirrhosis	Is a	True	Progressive intrahepatic cholestasis	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly
Progressive familial intrahepatic cholestasis type 2 (disorder)	Is a	True	Progressive intrahepatic cholestasis	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly
Progressive familial intrahepatic cholestasis type 1 (disorder)	Is a	True	Progressive intrahepatic cholestasis	Congenital syphilitic hepatomegaly	Congenital syphilitic hepatomegaly

Reference Sets

US English

Congenital syphilitic hepatomegaly

Back to Start

Id	Description	Lang	Type	Status	Case?	Module
123149016	Progressive intrahepatic cholestasis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
123150016	Byler syndrome	en	Synonym (core metadata concept)	Inactive	Entire term case sensitive (core metadata concept)	SNOMED CT core
123151017	Familial intrahepatic cholestasis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
123152012	Fatal intrahepatic cholestasis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
814631018	Progressive intrahepatic cholestasis (disorder)	en	Fully specified name	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
4570357019	Progressive familial intrahepatic cholestasis	en	Synonym (core metadata concept)	Active	Entire term case insensitive (core metadata concept)	SNOMED CT core
4570358012	PFIC - progressive familial intrahepatic cholestasis	en	Synonym (core metadata concept)	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core
4570359016	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.	en	Definition	Inactive	Entire term case sensitive (core metadata concept)	SNOMED CT core
5449630011	Progressive familial intrahepatic cholestasis (PFIC) refers to a heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin.	en	Definition	Active	Entire term case sensitive (core metadata concept)	SNOMED CT core