Benign recurrent intrahepatic cholestasis: review and long-term follow-up of five cases.

[benign recurrent intrahepatic cholestasis]

Benign recurrent intrahepatic cholestasis (BRIC ) is a rare autosomal recessive inherited disorder characterized by intermittent episodes of cholestatic jaundice . For the patients , the disease is a physical and psychological challenge . There is no curable treatment , but symptomatic relief is described following treatment with rifampicin or plasmapheresis .Five patients suffering from BRIC followed up for 17 years by one consultant are described . Two patients were treated with rifampicin and plasmapheresis , two with rifampicin alone , and one with plasmapheresis .The treatments showed symptomatic relief , effect on biochemical parameters , and earlier clinical remission compared with no treatment or treatment with other substances like cholestyramine , antihistamines , and ursodeoxycholic acid .Both rifampicin and plasmapheresis represent important therapeutic options of acute cholestatic attacks in patients with BRIC . As a noninvasive treatment , rifampicin may be the first choice .

Diseases
Validation

Diseases presenting "first choice" symptom

acute rheumatic fever

adrenal incidentaloma

benign recurrent intrahepatic cholestasis

dentinogenesis imperfecta

lymphangioleiomyomatosis

primary hyperoxaluria type 1

pyruvate dehydrogenase deficiency

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