Disease Information for Caroli disease

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Clinical Manifestations
Signs & Symptoms
Choluria/Biluria/Bilirubin urine
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Family History
Family history/Liver disease
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Laboratory Tests
Abnormal Lab Findings (Non Measured)
URINE Color Brown
Abnormal Lab Findings - Increased
URINE Bilirubin
Diagnostic Test Results
Pathology
BX/Liver biopsy/Cystic pathology/liver and hepatic ducts
CT Scan
CT Scan/CTA Abdomen Abnormal
CT Scan/Abdomen Cyst in Liver
CT Scan/Hepatic abnormalities
Ultrasound
Ultrasound/Abdomen/Cyst in liver
Ultrasound/Cysts in liver
Ultrasound/Liver Multiple cysts/masses
Angiography
Cholangiogram abnormal/PTC
Associated Diseases & Rule outs
Associated Disease & Complications
Biliary Abscesses
Cholangiolytic lesion/inlammatory bowel disease
Cholangitis, ascending/acute
Cholelithiasis
Cholestasis/intrahepatic biliary obstruction
Cholestatic Jaundice Syndrome
Cirrhosis
Cirrhosis in childhood
Hepatic cyst
Liver cyst, congenital
Microabscesses liver
Obstructive jaundice syndrome
Peritoneal Effusion
Polycystic kidney disease
Portal hypertension
Disease Mechanism & Classification
Class
CLASS/Biliary tree involvement/disorder (ex)
CLASS/Hepatic biliary duct involvement/disorder (ex)
CLASS/Liver involvement/disorder (ex)
CLASS/Liver/gallbladder/ducts (category)
Pathophysiology
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Pathophysiology/Cystic Dilatations of Bile Ducts
Process
PROCESS/Autosomal dominant hereditary disease (ex).
PROCESS/Autosomal recessive disorder (ex)
PROCESS/Congenital/developmental (category)
PROCESS/Eponymic (category)
PROCESS/Hereditofamilial (category)
PROCESS/Anomalies/Deformities/Malformations (EX)
Definition

Congenital cystic dilatation of the intrahepatic bile ducts; It consists of 2 types: simple, with bile duct dilatation or ectasia alone, and complex, with associated extensive hepatic fibrosis and portal hypertension; Benign renal tubular ectasia is associated with both types-----------------------------------

Caroli Disease; Congenital Dilatation of Intrahepatic Bile Duct; Caroli Disease is a rare inherited disorder characterized by abnormal widening (dilatation) of the ducts that carry bile from the liver (intrahepatic bile ducts); According to the medical literature, there are two forms of Caroli Disease; In most cases, the isolated or simple form Caroli Disease, affected individuals experience recurrent episodes of inflammation of the bile ducts (cholangitis) and unusual accumulation of pus (abscesses) on the liver; A second form of Caroli Disease is associated with abnormal formation of bands of fibrous tissue in the portal area of the liver (congenital hepatic fibrosis); The portal area of the liver is a groove (fissure) where the portal vein and the hepatic artery enter the liver; The portal vein is the blood vessel that carries blood from the stomach, intestine, and spleen to the liver and the hepatic artery is the blood vessel that carries blood from the aorta; This form of Caroli Disease is also often associated with high blood pressure of the portal vein (portal hypertension), polycystic kidney disease, and, in severe cases, liver failure; Caroli Disease is thought to be inherited as either an autosomal dominant or recessive ------------------[NORD 2005]---------------

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External Links Related to Caroli disease
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Wikipedia
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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