April 12, 2019


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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Etude anatomique d’un nouveau cas. The first symptoms typically include feverintermittent abdominal painand hepatomegaly. 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.

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

Imperforate anus Rectovestibular fistula Persistent cloaca Rectal atresia. National Center for Biotechnology InformationU. Modern imaging techniques allow the diagnosis to be made more easily and without invasive imaging of the biliary tree.

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Maladie de Caroli monolobaire – EM|consulte

In diffuse disease management if generally with conservative measures; liver transplantation may be an option 1. Recurrent pyogenic cholangitis Recurrent pyogenic cholangitis.

Journal page Archives Csroli list. Mortality is indirect and caused by complications. Portal hypertension may be present, resulting in other conditions including splenomegalyhematemesisand melena. Creative Commons Attribution 4. Webarchive template wayback links Infobox medical condition new.

The second form is more diffuse, and when associated with portal hypertension and congenital hepatic fibrosisis often referred to as “Caroli syndrome. Polycystic liver disease Congenital hepatic fibrosis Peliosis hepatis bile duct: Assessment of portal venous hypertension by catheterisation of hepatic vein.

GastroenterologyMedical genetics. Acta Paediatr Acad Sci Hung.

Eur J Gastroenterol Hepatol. Benhamouand R. Specialised Social Services Eurordis directory. 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.

Get a printable copy PDF file of the complete article 2. On a CT scan, Caroli disease can be observed by noting the many fluid-filled, tubular structures extending to the liver. There is a slight female gender bias. 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.

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Hydatid cyst Von Hippel—Lindau disease Tuberous sclerosis. Log in Sign up. The course is largely dependent on the associated disorders.

In diffuse cases of Caroli disease, treatment options include conservative or endoscopic therapy, internal biliary bypass procedures, and liver transplantation in carefully selected cases. The disease may be diffuse, lobar or segmental. Anatomical, clinical and nosological studies ]. Thank you for updating your details. 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.

Asphyxiating thoracic dysplasia basal body: Ciliopathy Hepatology Rare diseases Syndromes affecting the hepatobiliary system Syndromes with tumors.

Antibiotics are used for cholangitis.

Fibrose hépatique congénitale.

Please review our privacy policy. Top of the page – Article Outline. Presentation is in childhood or young adulthood. Can Med Assoc J. Journal of the Pancreas.