C0162510 - Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease.

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Lang Dictionary CODE			LUI	preferred no	SUI	preference Yes / No	Terms, descriptions
							CUI C0162510

Russian	Medical Subject Headings Russian	D016767	L3351696	preferred	S3879180	Y	КАРОЛИ БОЛЕЗНЬ
Russian	Medical Subject Headings Russian	D016767	L0896324	no	S1100141	Y	KAROLI BOLEZN'
Russian	MDRRUS	10007672	L15718662	no	S19079386	Y	Синдром Кароли
Russian	MDRRUS	10007671	L15766704	no	S19021010	Y	Болезнь Кароли
Russian	MDRRUS	10013003	L16092703	no	S19402841	N	Врожденная дилатация внутрипеченочных желчных протоков
Russian	MDRRUS	10013003	L16092703	no	S19402841	Y	Врожденная дилатация внутрипеченочных желчных протоков
	Medical Subject Headings Czech				A13063758	AT195261893	Vzácné dědičné postižení intrahepatálních žlučových cest s jejich ektaziemi; vyskytují se dále anomálie na dalších orgánech (ledviny, pankreas). Projevuje se ikterem, recidivami cholangitidy s následným postižením jater. V léčbě může přicházet v úvahu transplantace jater. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
	NCI Thesaurus				A17689241	AT197986626	A rare congenital disorder characterized by cystic dilatation of the intrahepatic bile ducts. It is associated with cholangitis and the formation of stones.
	SNOMEDCT_US	3308767019			A27175397	AT217617000	A rare congenital disorder characterized by multifocal, segmental dilatation of the large intrahepatic bile ducts. It may present at any age and predominantly affects females. Less than 250 cases have been described worldwide. Caroli disease is characterized by bile ductal ectasia without other apparent hepatic abnormalities. It presents with recurrent bacterial cholangitis, biliary stones causing biliary pain or episodes of pancreatitis. The more common variant of this disease, named Caroli syndrome, is characterized by dilatations of the large bile duct associated with congenital hepatic fibrosis. The etiology of Caroli disease is unknown and its occurrence is sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner.
	SNOMEDCT_US	3308768012			A27175397	AT217668889	A rare congenital disorder characterised by multifocal, segmental dilatation of the large intrahepatic bile ducts. It may present at any age and predominantly affects females. Less than 250 cases have been described worldwide. Caroli disease is characterised by bile ductal ectasia without other apparent hepatic abnormalities. It presents with recurrent bacterial cholangitis, biliary stones causing biliary pain or episodes of pancreatitis. The more common variant of this disease, named Caroli syndrome, is characterised by dilatations of the large bile duct associated with congenital hepatic fibrosis. The aetiology of Caroli disease is unknown and its occurrence is sporadic, whereas Caroli syndrome is generally inherited in an autosomal recessive manner.
	SNOMED Clinical Terms, Spanish	3131074014			A27401586	AT220115521	Un trastorno congénito raro caracterizado por dilatación segmentaria multifocal de los grandes conductos biliares intrahepáticos. Puede presentarse a cualquier edad y afecta de manera predominante a las mujeres. Se describieron menos de 250 casos en todo el mundo. La enfermedad de Caroli se caracteriza por ectasia de los conductos biliares sin otras anomalías hepáticas aparentes. Se presenta con colangitis bacteriana recurrente, cálculos biliares que producen dolor biliar o episodios de pancreatitis. La variante más frecuente de esta enfermedad, denominada síndrome de Caroli, se caracteriza por dilataciones de los grandes conductos biliares asociadas con fibrosis hepática congénita. Se desconoce la etiología de la enfermedad de Caroli y su ocurrencia es esporádica, mientras que el síndrome de Caroli en general se hereda en forma autosómica recesiva.
	Medical Subject Headings				A2783239	AT38143785	Congenital cystic dilatation of the intrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC). It consists of 2 types: simple Caroli disease is characterized by bile duct dilatation (ectasia) alone; and complex Caroli disease is characterized by bile duct dilatation with extensive hepatic fibrosis and portal hypertension (HYPERTENSION, PORTAL). Benign renal tubular ectasia is associated with both types of Caroli disease.