Caroli Disease

Common Name(s)

Caroli Disease

Caroli disease is a rare disorder characterized by abnormal widening of the large intrahepatic bile ducts.  It may present with recurrent bacterial cholangitis, biliary stones causing biliary pain or episodes of pancreatitis. Age of onset is variable and mainly females are affected. The more common variant of this disease, Caroli syndrome, is characterized by dilations of the large bile duct in association with congenital hepatic fibrosis. Patients with Caroli syndrome may present with signs and symptoms of portal hypertension, bacterial cholangitis, hepatomegaly, splenomegaly, esophageal varices, and gastrointestinal hemorrhage. Caroli syndrome may be found in association with autosomal recessive polycystic kidney disease, autosomal dominant polycystic kidney disease, medullary sponge kidney, and medullary cystic disease. Caroli disease may be sporadic or autosomal dominant, whereas Caroli syndrome is generally transmitted in an autosomal recessive manner.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Caroli Disease" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Caroli Disease" returned 3 free, full-text research articles on human participants. First 3 results:

[Caroli syndrome with autosomal recessive polycystic kidney disease].
 

Author(s): Jae Sung Ko

Journal: Korean J Gastroenterol. 2011 Jan;57(1):51-3.

 

Last Updated: 24 Jan 2011

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[A case of caroli disease with biliary stones].
 

Author(s): Byung Hyo Cha, Sang Hyub Lee, Jin-Hyeok Hwang, So Yeon Kim, Haer Young Kim

Journal: Korean J Gastroenterol. 2009 Oct;54(4):201-4.

 

Last Updated: 21 Oct 2009

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Hepatic cirrhosis caused by Caroli disease.
 

Author(s): C Rinaldi Lesmana, Abdul Harris, Agoes Kooshartoro, K Poerniati, Ronald Hukom, Heriawan S Czeresna, Laurentius A Lesmana

Journal: Acta Med Indones. ;37(1):33-5.

 

Last Updated: 9 May 2005

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Caroli Disease" returned 0 free, full-text review articles on human participants.

 
 
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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Clinical and Molecular Investigations Into Ciliopathies
 

Status: Recruiting

Condition Summary: Autosomal Recessive Polycystic Kidney Disease; Congenital Hepatic Fibrosis; Caroli's Disease; Polycystic Kidney Disease; Joubert Syndrome; Cerebro-Oculo-Renal Syndromes; COACH Syndrome; Senior-Loken Syndrome; Dekaban-Arima Syndrome; Cogan Oculomotor Apraxia; Nephronophthisis; Bardet-Biedl Syndrome; Alstrom Syndrome; Oral-Facial-Digital Syndrome

 

Last Updated: 11 Nov 2014

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UAB HRFD Core Center: Core A: The Hepato/Renal Fibrocystic Diseases Translational Resource
 

Status: Recruiting

Condition Summary: Hepato/Renal Fibrocystic Disease; Autosomal Recessive Polycystic Kidney Disease; Joubert Syndrome; Bardet Biedl Syndrome; Meckel-Gruber Syndrome; Congenital Hepatic Fibrosis; Caroli Syndrome; Oro-Facial-Digital Syndrome Type I; Nephronophthisis; Glomerulocystic Kidney Disease

 

Last Updated: 12 Jan 2014

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