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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The disease affects about one in 1, people, with more reported cases of Caroli syndrome than of Caroli disease. Caroli disease can present at any age. The differential diagnosis craoli include primary sclerosing cholangitis, isolated polycystic liver disease, and hepatic cystic hamartoma see these terms carolu, as well as hepatic and choledochal cysts.

Read it at Google Books – Find it at Amazon. Modern imaging techniques allow the diagnosis to be made more easily and without invasive imaging of the biliary tree.

Caroli disease is distinct from other diseases that cause ductal dilatation caused by obstruction, in that it is not one of the many choledochal cyst derivatives.

Caroli disease

Mortality is indirect and caused by complications. You can move this window by clicking on the headline. From Wikipedia, the free encyclopedia. For a further CT differential, consider also focal hypodense hepatic lesions on a non contrast CT scan.

The treatment depends on clinical features and the location of caorli biliary abnormality. Specialised Social Services Eurordis directory. Synonyms or Alternate Spellings: If you are a subscriber, please sign in ‘My Account’ at the top right of the screen.


Summary and related texts.

Fibrose hépatique congénitale.

Manifestations are those of complications, mostly bacterial cholangitis, and include abdominal pain and biliary colic, fever with chills, and jaundice.

This page was last edited on maladje Augustat In other projects Wikimedia Commons. If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: Caroli disease is a rare autosomal recessive disorder which has no recognised gender predilection. Ciliopathy Hepatology Rare diseases Syndromes affecting the hepatobiliary system Syndromes with tumors. Disease definition Caroli disease CD is a rare congenital liver disease characterized by non-obstructive cystic dilatations of the intra-hepatic and carpli extra-hepatic bile ducts.

Syndromic cases CS share with congenital hepatic fibrosis and recessive polycystic kidney disease an autosomal recessive transmission. Hydatid cyst Von Hippel—Lindau disease Tuberous sclerosis. Exact prevalence and annual incidence data are not available for CD, but the disease is known to be very rare. Medical College of Wisconsin.

Cases have also been found in infants and adults. The documents contained in this web site are presented for information purposes only.

Images in this article Fig. Open in a separate window. Edit article Share article View revision history. Multiple biliary re Multiple biliary hamartomas. The ductal plate is a layer of hepatic precursor cells that surround the portal venous branches, and is the anlage of the intrahepatic bile ducts.

Access to the PDF text. Genetic counseling Most cases of CD are sporadic. Caroli disease is also associated with liver failure and polycystic kidney disease. Polycystic kidney disease Autosomal dominant polycystic kidney Autosomal recessive polycystic kidney Medullary cystic kidney disease Nephronophthisis Congenital cystic dysplasia. Imperforate anus Rectovestibular fistula Persistent cloaca Rectal atresia.


Differential diagnosis 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. With a liver transplant, cholangiocarcinoma is usually avoided in the long run. As per the Law relating to information storage and personal integrity, you have the right to oppose art 26 of that lawaccess art 34 of that law and rectify art 36 of that law your personal data.

HONselect – Caroli Disease

Bowel gas and digestive habits make it difficult to obtain a clear sonogramso a CT scan is a good substitution. When the disease is localized to one hepatic lobe, hepatectomy relieves symptoms and appears to remove the risk dde malignancy.

Quality of life may be significantly affected by recurrent cholangitis. PKHD1 is expressed primarily in the kidneys with lower levels in the liverpancreasand malaidea pattern consistent with phenotype of the disease, which primarily affects the liver and kidneys.

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