Rare Diseases Symptoms Automatic Extraction

Benign recurrent intrahepatic cholestasis. Some reflections on a case followed for 20 years.

[benign recurrent intrahepatic cholestasis]

Benign recurrent intrahepatic cholestasis (BRIC) is a form of cholestasis of obscure aetiology characterized by recurrent episodes of jaundice and itching associated with a morphological picture of pure intrahepatic cholestasis. No effective treatment has yet been found among the many that have been proposed and the invariably benign nature of the condition has been questioned. A case of BRIC followed for a period of 20 years is described. This case is of great interest from these two points of view: 1) the histologic and electron microscopic findings 23 and 41 years after the first episode of cholestasis, respectively, failed to reveal evidence of the possible future development of cirrhosis; 2) treatment with ursodeoxycholic acid proved ineffective both therapeutically and in the prevention of episodes of bile stasis: on the contrary, calculosis of the common bile duct appeared after 8 months from the onset of the treatment.

Diseases presenting "recurrent episodes" symptom

  • acute rheumatic fever
  • adrenomyeloneuropathy
  • benign recurrent intrahepatic cholestasis
  • child syndrome
  • cholangiocarcinoma
  • cystinuria
  • familial mediterranean fever
  • hydrocephalus with stenosis of the aqueduct of sylvius
  • kabuki syndrome
  • neuralgic amyotrophy
  • pyruvate dehydrogenase deficiency
  • systemic capillary leak syndrome
  • triple a syndrome

You can validate or delete this automatically detected symptom