Benign recurrent intrahepatic cholestasis: late initial diagnosis in adulthood.
Benign recurrent intrahepatic cholestasis (BRIC) is a rare autosomal recessive or sporadic disorder, characterized by recurrent episodes of intense pruritus and jaundice that resolve spontaneously without leaving considerable liver damage. The attacks can start at any age, but the first attack is usually seen before the second decade of life. We report the case of a young adult male patient with BRIC who presented with recurrent cholestatic jaundice and pruritus with negative work up for all possible etiologies and a liver biopsy consistent with intrahepatic cholestasis. He improved on treatment with rifampicin and has not suffered another attack on follow up. Although in adulthood, BRIC diagnosis should be kept in mind in patients with recurrent cholestatic attacks with symptom free intervals after main bile duct obstruction and other congenital or acquired causes of intrahepatic cholestasis excluded.
Department of Gastroenterology, Gulhane Military Medical Academy, Haydarpasa Training Hospital, Istanbul, Turkey. fatihermis2@hotmail.com
Fatih
F
Ermis
Kemal
K
Oncu
Melih
M
Ozel
Yusuf
Y
Yazgan
Ahmet Kemal
AK
Gurbuz
Levent
L
Demirturk
Hakan
H
Demirci
Taner
T
Akyol
Aptullah
A
Hahoglu
D000367:Age Factors; D001706:Biopsy; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D006801:Humans; D041781:Jaundice, Obstructive / Q000188:drug therapy / Q000209:etiology*; D008099:Liver / Q000473:pathology; D008297:Male; D011537:Pruritus / Q000188:drug therapy / Q000209:etiology*; D012293:Rifampin / Q000627:therapeutic use; D055502:Secondary Prevention; D016896:Treatment Outcome; D055815:Young Adult
D005765:Gastrointestinal Agents; D012293:Rifampin