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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Syndromic cases CS share with congenital hepatic fibrosis and recessive polycystic kidney disease an autosomal recessive transmission. Portal hypertension may be present, resulting in other conditions including splenomegalyhematemesisand melena.

The periportal fibrosis type may present with pain or signs of portal hypertension, including haematemesis from esophageal varices Some develop intra- or extra-hepatic calculi, leading to carolj cholangitis with bacteremia and sepsisand acute pancreatitis. The manifestation of ductal plate malformation depends on the level of the biliary tree that is affected Family studies are necessary to determine if Caroli disease is due to inheritable causes.

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In diffuse disease management if generally with conservative measures; liver transplantation may be an option 1. This article has been cited by other articles in PMC.

Detailed information Article for general public Svenska National Center for Biotechnology InformationU. Other search option s Alphabetical list. Etude anatomique d’un nouveau cas. Full text Full text is available as a scanned copy of the original print version. Clinical description Caroli disease can present at any age.

Only comments written in English can be processed. Cases and figures Imaging differential diagnosis. Recurrent pyogenic cholangitis Recurrent pyogenic cholangitis. On a CT scan, Caroli disease can be observed by noting the many fluid-filled, tubular structures extending to the liver.

However, some series show that extrahepatic duct involvement may exist 2. Additional information Further information on this disease Classification s 4 Gene s 1 Other website s 2.

Jacques Caroli, a gastroenterologistfirst described a rare congenital condition maladle in ParisFrance. Multiple biliary hamartomas Multiple biliary hamartomas. From Wikipedia, the free encyclopedia. Retrieved from ” https: Caroli disease communicating cavernous ectasia, or congenital carolli dilatation of the intrahepatic biliary tree is a rare inherited disorder characterized by cystic dilatation or ectasia of the bile ducts within the liver.

Fibrose hépatique congénitale.

Biliary hamartomas Caroli disease Choledochal cysts Bile duct hamartoma. Creative Commons Attribution 4. Endoscopic retrograde cholangiopancreatography ERCP and maladiie transhepatic cholangiography PTC should not be used for diagnosis because they are associated with an increased risk of bacterial cholangitis. Modern imaging techniques allow the diagnosis to be made more easily and without invasive imaging of the biliary tree.


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Fibrose hépatique congénitale.

Assessment of portal venous hypertension by catheterisation of hepatic vein. In contrast, in the periportal type of Caroli disease or Caroli syndromeboth the central intrahepatic bile ducts and the ductal plates of the smaller peripheral bile ducts are affected, with the latter leading to the development of fibrosis.

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. For all other comments, please send your remarks via contact us.

You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Summary Epidemiology Exact prevalence and annual incidence data are not available for Maladid, but the disease is known to be very rare. Intestines Intestinal atresia Duodenal atresia Meckel’s diverticulum Hirschsprung’s disease Intestinal malrotation Dolichocolon Enteric duplication cyst.