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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Caroli disease

Radiological, endoscopic, and surgical intervention may be required for patients with biliary obstruction, abscess formation and liver or bile duct stones. Health care resources for this disease Expert centres Diagnostic tests 2 Patient organisations 23 Orphan drug s 0.

Log in Sign up. Proc Staff Meet Mayo Clin. In diffuse disease management if generally with conservative measures; liver transplantation may be an option 1. Etude anatomique d’un nouveau cas.

Fibrose hépatique congénitale.

For all other comments, please send your remarks via contact us. Caroli disease is a rare autosomal recessive disorder which has no recognised gender predilection. You can move this window by clicking on the headline. Besides bacterial cholangitis, complications include liver abscess, biliary infection, and in late stages, cholangiocarcinoma.

Ann Chir Thorac Cardiovasc. 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.

Access to the PDF text. Caroli disease is a congenital disorder comprising of multifocal cystic dilatation of segmental intrahepatic bile ducts.

J Chir Paris Nov; This article has been cited by other articles in PMC. Contact Help Who are we? Cysts of the jaws Odontogenic cyst Periapical cyst Dentigerous cyst Odontogenic keratocyst Nasopalatine duct cyst liver: Hydatid cyst Von Hippel—Lindau disease Tuberous sclerosis. Access to the text HTML.

Fièvre récurrente : penser à la maladie de Caroli – EM|consulte

The manifestation of ductal plate malformation depends on the level of the biliary tree that is affected Prognosis is generally poor. Thank you for updating your details. Congenital fibrosis of the liver as a familial defect.

Biliary hamartomas Caroli cxroli Choledochal cysts Bile duct hamartoma.

When the intrahepatic bile duct wall has protrusions, it is clearly seen as central dots or a linear streak. Acta Paediatr Acad Sci Hung. Medical College of Wisconsin. At the other end of the fibropolycystic disease spectrum are Von Meyenburg complexes, also known as biliary hamartomas which result from discrete foci of ductal plate malformation affecting the smallest bile ducts 5.

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Cwroli propos d’une observation. PKHD1 is expressed primarily in the kidneys with lower levels in the liverpancreasand lungsa pattern consistent with phenotype of the disease, which primarily affects the liver and kidneys.

Mortality is indirect and caused by complications.

Maladie de Caroli monolobaire. Eur J Gastroenterol Hepatol. Ciliopathy Hepatology Rare diseases Syndromes affecting the hepatobiliary system Syndromes with tumors. Cases and figures Imaging differential diagnosis. You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted. Clinical description Caroli disease can present at any age.

Edit article Share article View revision history. Caroli disease can present at any age. Polycystic kidney disease cargo: The key diagnostic procedure is magnetic resonance cholangiography showing a characteristic aspect of abnormal bile ducts.

The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis kaladie diagnosis or treatment.

The course is largely dependent on the associated disorders.