BENIGN RECURRENT INTRAHEPATIC CHOLESTASIS - 08/09/11
Résumé |
Benign recurrent intrahepatic cholestasis (BRIC) was first described by Summerskill and Walshe in 1959.80 They referred to the disorder as “benign recurrent intrahepatic obstructive jaundice” to differentiate it from other disorders that cause jaundice and that had already been described at that time. The disorder is characterized by repeated self-limited episodes of severe pruritus and jaundice lasting from several weeks to months. Although the syndrome does not lead to progressive liver dysfunction and cirrhosis, symptoms associated with each attack may be associated with significant morbidity. In 1969, Tygstrup85 developed diagnostic guidelines for BRIC that are still in use today. These criteria include a history of several episodes of jaundice separated by a symptom-free interval of at least 6 months in the absence of an inciting drug or toxin.
• | At least two episodes of jaundice separated by a symptom-free interval lasting several months to years |
• | Laboratory values consistent with intrahepatic cholestasis |
• | Severe pruritis secondary to cholestasis |
• | Liver histology demonstrating centrilobular cholestasis |
• | Normal intrahepatic and extrahepatic bile ducts confirmed by cholangiography |
• | Absence of factors known to be associated with cholestasis, (i.e., drugs, pregnancy) |
The original report described BRIC in two English patients.80 The disorder has subsequently been reported in patients from Northern and Mediterranean Europe, Africa, North and South America, and Japan.8, 20, 22, 49, 73, 78, 84 More than 100 cases have now been described worldwide. Although many reported cases were sporadic, it is now recognized that approximately 50% of patients with BRIC have a family history of cholestasis.49, 84 Genetic studies in affected families have confirmed that BRIC is inherited as an autosomal recessive disorder, and mapping studies have placed the defective gene on the long arm of chromosome 18.23, 42
This article reviews the clinical presentation, diagnostic criteria, laboratory abnormalities, and proposed etiologic factors responsible for BRIC. Recent studies describing the genetic defect in BRIC and the relationship of this defect to progressive familial intrahepatic cholestasis (PFIC), Byler disease, are also discussed.
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| Address reprint requests to Mitchell L. Shiffman, MD, Hepatology Section, Medical College of Virginia Commonwealth University, Box 980341, Richmond, VA 23298, e-mail: mshiffma@hsc.vcu.edu |
Vol 3 - N° 3
P. 509-528 - août 1999 Retour au numéro
Article précédent
- TOTAL PARENTERAL NUTRITION AND CHOLESTASIS
- Iqbal S. Sandhu, Catherine Jarvis, Gregory T. Everson
- PRIMARY BILIARY CIRRHOSIS AND PRIMARY SCLEROSING CHOLANGITIS
- Paul Angulo, Keith D. Lindor
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