Caroli disease

MeSH: D016767ORPHA: 530351 Treatment Available

Overview

bile duct disease that is characterized by abnormal dilatation of the intrahepatic bile ducts

Available Treatments (1)

DrugFormStatusCountriesLead Time
Ursodeoxycholic acid
Capsule, TabletFDA Approved63d

Clinical Presentation

Signs and symptoms associated with Caroli disease, sourced from HPO and Orphanet clinical annotations.

CholestasisJaundiceIntrahepatic cholestasisAbdominal painConjugated hyperbilirubinemiaElevated circulating alkaline phosphatase concentrationCholangitisElevated gamma-glutamyltransferase levelPolycystic kidney dysplasiaPruritusCholelithiasisCirrhosisAscitesSplenomegalyWeight lossFeverIncreased total leukocyte countVomitingNauseaAnorexiaEsophageal varixHepatomegalyChillsElevated circulating alanine aminotransferase concentrationConjunctival icterusAbdominal rigidityHepatic fibrosisPortal hypertensionBiliary cirrhosisCholangiocarcinomaLiver abscess

Classification & Codes

MeSH Code

D016767

Orphanet Code

ORPHA:53035
Caroli disease
MeSHD016767
OrphanetORPHA:53035
Treatments1 drug(s)
Symptoms on record31 signs
Statuspublished

Treatment Summary

Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO