Benign recurrent intrahepatic cholestasis (BRIC): a rare diagnostic challenge

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"an iranian girl with benign recurrent intrahepatic cholestasis "

this report presents an 11 year-old girl with benign recurrent cholestasis (bric) who developed episodes of severe jaundice and pruritus at the ages of 2.5 and 10 years. each episode lasted for 3-4 months. the peak level of serum bilirubin reached 33-37 mg/dl.liver function tests were all normal during the attack except for increased alkaline phosphatase and prolonged prothrombin time responsiv...

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Benign Recurrent Intrahepatic Cholestasis with a Single Heterozygote Mutation in the ATP8B1 Gene

Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive inherited disorder characterized by multiple recurrent episodes of severe cholestatic jaundice without obstruction of extrahepatic bile duct. We present the case of a 7-year-old boy with BRIC confirmed by mutation analysis in the ATP8B1 gene and typical clinical manifestation. Despite inheritance of BRIC, we detected...

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Endoscopic nasobiliary drainage improves jaundice attack symptoms in benign recurrent intrahepatic cholestasis: A case report

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Hereditary recurrent intrahepatic cholestasis from birth.

Obstructive jaundice in the first months of life is a fairly frequent phenomenon. Approximately twothirds of these infants have atresia of the hepatic ducts, and about two-thirds of the remaining can be classified as 'neonatal hepatitis', 'giant cell hepatitis', or 'thick bile syndrome' (Craig and Landing, 1952; de Toni and Romano, 1962; Gellis, 1961). 'Thick bile syndrome' is defined as 'neona...

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ژورنال

عنوان ژورنال: Jaffna Medical Journal

سال: 2020

ISSN: 2651-0200,0379-3877

DOI: 10.4038/jmj.v32i2.106