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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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Fibrose hépatique congénitale.

Proc Staff Dd Mayo Clin. Collaborative Hypertext of Radiology. Loading Stack – 0 images remaining. The first symptoms typically include feverintermittent abdominal painand hepatomegaly.

For a further CT differential, consider also focal hypodense hepatic lesions on a non contrast CT scan. Management and treatment Management depends on the clinical presentation, localization and stage of the disease. At the other end of the fibropolycystic disease spectrum are Von Meyenburg complexes, also known as maldaie hamartomas which result from discrete foci of ductal plate malformation affecting the smallest bile ducts 5.

Images in this article Mwladie. Multiple biliary hamartomas Multiple biliary hamartomas. Archived from the original on Presentation is in childhood or young adulthood. Patients with severe disease may be candidates for liver transplantation.


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

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Synonyms or Alternate Spellings: There are two patterns of Caroli disease: Specialised Social Services Eurordis directory. Other search option s Alphabetical list. Caroli disease Turbo spin echo T2-weighted axial MRI of Caroli disease, showing cystic dilatations of bile ducts shown as white. Diagnostic methods The diagnosis is suspected on clinical grounds and confirmed through detection of cystic dilatation in the biliary tree through imaging studies. About Blog Go ad-free. Polycystic kidney disease cargo: Prognosis Quality of life may be significantly affected by recurrent cholangitis.

Check for errors and try again. In diffuse disease management if generally with conservative measures; liver transplantation may be an option 1. Genetic counseling Most cases of CD are sporadic. When the intrahepatic bile duct wall has protrusions, it is clearly seen as central dots or a linear streak. By using this site, you agree to the Terms of Use and Privacy Policy. Associated Data Supplementary Materials. Imperforate anus Rectovestibular fistula Persistent cloaca Rectal atresia.


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Caroli disease

The diagnosis is suspected on clinical grounds and confirmed through detection of cystic dilatation in the biliary tree through imaging studies. Manifestations are those of complications, mostly bacterial cholangitis, and include abdominal pain and biliary colic, fever with chills, and jaundice. The periportal fibrosis type may present with pain or signs of portal hypertension, including haematemesis from esophageal varices The material is in no way intended to naladie professional medical care by a qualified specialist acroli should not be used as a basis for diagnosis or treatment.

Antibiotics are used to treat the inflammation of the bile duct, and ursodeoxycholic acid is used for hepatolithiasis. Caroli disease is a rare autosomal recessive disorder which has no recognised gender predilection.

Open in a separate window. Regular follow-ups, including ultrasounds and liver biopsies, are performed.