Dataset Information

Benign Recurrent Intrahepatic Cholestasis (BRIC): An African Case Report.

ABSTRACT: Benign recurrent intrahepatic cholestasis (BRIC) is a rare disorder characterised by recurrent episodes of cholestatic jaundice. First described in 1959, BRIC has been reported in patients all over the world including of African descent. Here, we describe a case of a 21-year-old male with recurring episodes of cholestatic jaundice where we diagnosed BRIC and terminated an episode with rifampicin. To our knowledge, this is the first case report of BRIC diagnosed in Africa.

SUBMITTER: Salyani A

PROVIDER: S-EPMC7085836 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Benign Recurrent Intrahepatic Cholestasis (BRIC): An African Case Report.

Salyani Adil A Barasa Linda L Rajula Allan A Ali Sayed K SK

Case reports in gastrointestinal medicine 20200310

Benign recurrent intrahepatic cholestasis (BRIC) is a rare disorder characterised by recurrent episodes of cholestatic jaundice. First described in 1959, BRIC has been reported in patients all over the world including of African descent. Here, we describe a case of a 21-year-old male with recurring episodes of cholestatic jaundice where we diagnosed BRIC and terminated an episode with rifampicin. To our knowledge, this is the first case report of BRIC diagnosed in Africa. ...[more]

PMID: 32231815

Similar Datasets

Project description:Hereditary spherocytosis (HS) is not a rare disease in the department of hematology; however, in the late stage of the disease, patients often have very severe cholestasis and are referred to the department of hepatology. Hepatologists may have trouble determining the source of cholestasis, causing treatment difficulties. We report two 20-year-old patients complaining of "skin and eyes turned to yellow". Patient 1 had no previous hematologic disorders, and patient 2 had a history of anemia without treatment. Laboratory tests suggested anemia and elevated bilirubin in both patients. The direct bilirubin levels were more significantly elevated than the indirect bilirubin levels in both patients, and the patients both suffered from abdominal pain and pancreatitis. However, the degree of anemia could not fully explain the jaundice. Magnetic resonance imaging findings suggested the presence of hepatosplenomegaly and gallstones. Genetic testing identified new mutations in the relevant genes, ultimately confirming the diagnosis of HS. The liver biopsy results for both patients showed obvious intrahepatic cholestasis. Patient 1 underwent splenectomy at a bilirubin level of 125.4 µmol/L, and the bilirubin level returned to normal after surgery, with a good prognosis. However, Patient 2 suffered from pancreatitis during hospitalization and was unable to undergo splenectomy. Endoscopic retrograde cholangiopancreatography was implemented, but the bilirubin level continued to rise, and Patient 2 ultimately gave up treatment and passed away. For hepatologists, identifying the source of jaundice (hemolysis, hepatocyte destruction, or biliary obstruction) is important for treatment, supplemented by liver biopsy and genetic testing if necessary. In the 2 cases covered in this article, early-stage HS caused hemolytic jaundice with predominantly elevated indirect bilirubin, and as the disease progressed, patients developed severe cholestasis probably related to transient biliary obstruction caused by gallstones and hepatocellular injury due to abnormal bilirubin metabolism. In addition, in patients with HS combined by intrahepatic cholestasis, early consideration of splenectomy may delay disease progression and achieve a better prognosis. Of course, this conclusion needs to be confirmed by more clinical studies.

Dataset Information

Benign Recurrent Intrahepatic Cholestasis (BRIC): An African Case Report.

Publications

Benign Recurrent Intrahepatic Cholestasis (BRIC): An African Case Report.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets