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Caroli's disease II

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A congenital disease characterized by non-obstructive cystic dilatation of the small intra-hepatic bile ducts with secondary development of cholangitis, abscess of the liver, and septicaemia.

Description

A congenital disease characterized by non-obstructive cystic dilatation of the small intra-hepatic bile ducts with secondary development of cholangitis, abscess of the liver, and septicaemia. Symptoms are nausea, vomiting, gastralgia, fever. Both sexes affected. Onset in first years of life or later (frequently around 35 years of age). Associated disorders may include portal hypertension, liver cirrhosis, splenomegaly, and cysts of other organs, such as the spleen, kidneys, and pancreas. Infection with gram-negative bacteria is a frequent fatal complication. When associated with with periportal fibrosis and portal hypertension, the condition is often referred to as Caroli's syndrome. Aetiology unknown. Autosomal recessive inheritance is assumed.

Bibliography

  • J. Caroli, J. Soupault, J. Kossakowski, L. Plocker, M. Paradowska:
    La dilatation polykystique congénitale des voies biliaires intrahépaique. Essai de classification.
    Semaine des Hôpitaux de Paris, 1958, 34: 488-495.
  • J. Caroli:
    Une affection nouvelle, sans doute congénitale, des voies biliaires: La dilatation kystique unilobulaire des canaux hépatiques.
    Semaine des Hôpitaux de Paris, 1958, 34: 496-502.
  • J. Caroli, V. Corco:
    Maladies des voies biliaires intra-hépatiques segmentaires. Paris, Masson, 1964.
  • J. Caroli:
    Diseases of the intrahepatic biliary tree.
    Clinics in Gastroenterology, London, 1973, 2: 147-161.

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