La revue de médecine interne – Vol. 33 – N° S2 – p. AA – Fièvre récurrente: penser à la maladie de Caroli – EM|consulte. C’est une cause rare de cholestase chronique et de lithiases intrahépatiques. Nous rapportons deux cas de maladie de Caroli monolobaire. Request PDF on ResearchGate | Maladie de Caroli monolobaire. À propos de 12 cas | BackgroundCaroli’s disease is the dilatation of the segmental intrahepatic.

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Copyright and License information Disclaimer. Langerhans cell histiocytosis Lymphangioleiomyomatosis Cystic bronchiectasis. Abnormal development of the embryonic bile ducts at the stage of ductal plate has been suggested.

Orphanet: Maladie de Caroli

Some patients remain asymptomatic throughout the disease course. The cause appears to be genetic; the simple form is an autosomal dominant trait, while the complex form is an autosomal recessive trait.

Annular pancreas Accessory pancreas Johanson—Blizzard syndrome Pancreas maaladie. Rev Med Chir Mal Foie. Cutaneous ciliated cyst Hidrocystoma no epithelium: Other search option s Alphabetical list. Maladie de Caroli monolobaire.

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Caroli disease

Thank you for updating mlaadie details. National Center for Biotechnology InformationU. Access to the full text of this article requires a subscription. Endoscopic retrograde cholangiopancreatography ERCP and percutaneous transhepatic cholangiography PTC should not be used for diagnosis because they are associated with an increased risk of bacterial cholangitis.

Acta Paediatr Acad Sci Hung. Caroli disease communicating cavernous ectasia, or congenital cystic dilatation of the intrahepatic biliary tree is a rare inherited disorder characterized by cystic dilatation or ectasia of the bile ducts within the liver. In other projects Wikimedia Commons.

dd By using this site, you agree to the Terms of Use and Privacy Policy. PKHD1 is expressed primarily in the kidneys with lower levels in the liverpancreasand lungsa malxdie consistent with phenotype of the disease, which primarily affects the liver and kidneys. Management depends on the clinical presentation, localization and stage of the disease.

Caroli disease is also associated with liver failure and polycystic kidney disease. Retrieved from ” https: As medical imaging technology improves, diagnostic age decreases.

Caroli disease is a congenital disorder comprising of multifocal cystic dilatation of segmental intrahepatic bile ducts. Jacques Caroli, a gastroenterologistfirst described a rare congenital condition in in ParisFrance. Most cases of CD are sporadic.

Fibrose hépatique congénitale.

Articles Cases Courses Quiz. Turbo spin echo T2-weighted axial MRI of Caroli disease, showing cystic dilatations of bile ducts shown as white.

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The differential diagnosis should include primary sclerosing cholangitis, isolated polycystic liver disease, and hepatic cystic hamartoma see these termsas well as hepatic and choledochal cysts. Disease definition Caroli disease CD is a rare congenital liver disease characterized by non-obstructive cystic dilatations of the intra-hepatic and rarely extra-hepatic bile ducts. Radiological, endoscopic, and surgical intervention may be required for patients with biliary obstruction, abscess formation and liver or bile duct stones.

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Caroli disease – Wikipedia

Full text Full text is available as a scanned copy of the original print version. Health care resources for this disease Expert centres Diagnostic tests 2 Patient organisations 23 Orphan drug s 0.

Prognosis depends on the clinical course and the risk of cholangiocarcinoma. Fibrosis of the liver as a congenital anomaly. Ann Chir Thorac Cardiovasc. The treatment depends on clinical features and the caropi of the biliary abnormality.

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