Hospital coures one weekHospital coures one week
Condition improved and
Diagnosis on discharge Benign
recurrent intrahepatic cholestasis
Patient seen after one and half year
after admission in Mostafa Kamel
Hospital in Alexandria for liver
Results of liver biopsy: preserved
lobular architecture with cholestatic
changes in hepatocytes
Liver biopsy: histopathology
shows preserved lobular
architecture with cholestatic
changes in hepatocytes
Final confirmed diagnosis
Bening recurrent Intermittent
Benign Recurrent Intrahepatic CholestasisBenign Recurrent Intrahepatic Cholestasis
Type 1Type 1 (BRIC1): (Summerskill–Tygstrup–Walsh
The molecular defect of BRIC 1 is
localized on the FIC1 (ATP8B1) gene
Typically the disease begins with recurrent
episodes of jaundice in the first decade of life that
continue into adult life.
Cholestatic episodes often follow a viral infection of
the upper respiratory tract.
and are heralded by pruritis, loss of appetite, anorexia
and nausea. Nearly every other patient complains of
The jaundice lasts for 3–4 months, then
spontaneously subsides, and usually recurs
in approximately yearly intervals.
Asymptomatic periods of several years,
however, are also well documented.
A marked hyperbilirubinemia, with a moderate
elevation of alkaline phosphatase and typically
normal g-glutamyl transpeptidase and
aminotransferase levels is observed (atypical
cases without pruritus and with high serum g-GT
have been reported).
On cholangiography (MRCP or ERCP) the bile
ducts are radiographically normal.
The liver architecture is normal. A bland
cholestasis, i.e. without inflammatory
changes, is present (Fig.1). There is no
fibrosis and the disease does not progress to
cirrhosis. During clinically asymptomatic
periods the histological findings are entirely
The biopsy showed preserved lobular architecture with marked
cholestasis within hepatocytes with mild inflammatory cell
ttt with corticosteroids,
acid, cholestyramine, low fat
diet, and rifampin have all been
tried and are ineffective.
In patients with intense pruritis,
plasmapheresis may lead to some
improvement of symptoms and
Although not readily understandable based on
our current understanding of the
pathophysiology of the disorder, a recent
report describes complete and long-lasting
resolution of pruritis as well as normalization
of serum bile salt concentrations in cholestatic
BRIC patients within 24 h. after endoscopic
Patients with BRIC 1 should be
reassured that their disease is
benign and does not progress to
chronic liver disease.