[Ursodeoxycholic acid treatment shortens the course of cholestasis in two patients with benign recurrent intrahepatic cholestasis].

[benign recurrent intrahepatic cholestasis]

Benign recurrent intrahepatic cholestasis (BRIC ) is a rare autosomal-crecessive or sporadic disorder , characterized by repeated episodes of unexplained cholestasis followed by prolonged asymptomatic periods .We present two male patients aged 12 and 15 years old who initially presented cholestasis and who had no family history of liver or biliary disease . Typically , alkaline phosphatase and bilirubin levels were elevated , with a slight increase in transaminases and gamma-glutamyltransferase . In both patients biliary tract disease was ruled out by endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiography while no significant abnormalities of the liver parenchyma were found on liver biopsy . Early treatment with ursodeoxycholic acid (UDCA ) reduced the duration of the cholestasis episode compared with that of other episodes in which the patient had received other treatment or the treatment was late (1 or 2 months vs 4 months in patient 1 and 1 -3 months vs 5 months in patient 2 , respectively ).Consensus is lacking on the treatment of BRIC although several treatment have been used . UDCA could be effective in increasing bile acid secretion and in reducing serum bilirubin levels . In the two patients described , the initial dosage was 15 mg /kg /day , which was subsequently maintained at 6 mg /kg /day , which was subsequently maintained 6 mg /kg /day until biochemical parameters returned to normal . Nevertheless , larger studies are required to confirm that UDA shortens episodes of cholestasis in BRIC .

Diseases
Validation

Diseases presenting "cholestasis" symptom

benign recurrent intrahepatic cholestasis

carcinoma of the gallbladder

congenital toxoplasmosis

erythropoietic protoporphyria

familial mediterranean fever

megacystis-microcolon-intestinal hypoperistalsis syndrome

neonatal adrenoleukodystrophy

x-linked adrenoleukodystrophy

zellweger syndrome

This symptom has already been validated