PMID- 36369734
OWN - NLM
STAT- MEDLINE
DCOM- 20230426
LR  - 20230503
IS  - 1502-7708 (Electronic)
IS  - 0036-5521 (Linking)
VI  - 58
IP  - 5
DP  - 2023 May
TI  - Improvement of cholestatic episodes in patients with benign recurrent 
      intrahepatic cholestasis (BRIC) treated with rifampicin. A long-term follow-up.
PG  - 512-520
LID - 10.1080/00365521.2022.2143725 [doi]
AB  - OBJECTIVE: Patients with benign recurrent intrahepatic cholestasis (BRIC) suffer 
      from recurrent episodes of cholestatic jaundice. Treatment options remain limited 
      and are mainly symptomatic. In case reports rifampicin, plasmapheresis, and 
      nasobiliary drainage have been reported to be effective. In this case series, we 
      present long-term experience indicating disease-modifying effects of non-invasive 
      treatment with rifampicin for recurrent cholestasis in BRIC type 1 (BRIC1). 
      MATERIALS AND METHODS: We included all adult BRIC1 patients diagnosed and 
      followed up at a single centre in Bergen, Norway. Data regarding clinical and 
      biochemical features during BRIC attacks with and without rifampicin treatment 
      were retrieved from medical journals and a data registry. RESULTS: Five males 
      with BRIC1 were included. Median age at diagnosis was 22 years (range 15-41). 
      Together they had suffered from 65 cholestatic attacks (including four documented 
      abortive attacks). Twenty-eight attacks were treated with rifampicin alone over 
      the last 12 years; all cases showed symptomatic relief and reduction in the 
      levels of bilirubin and alkaline phosphatase in blood. The attacks treated with 
      rifampicin seemed to have shorter duration and were less likely to result in 
      complications or hospitalization compared to attacks prior to the introduction of 
      rifampicin. No side effects attributable to rifampicin were noted. CONCLUSIONS: 
      Episodic treatment of recurrent BRIC1 attacks with rifampicin seems to ameliorate 
      severity and shorten the duration of attacks. Timely diagnosis and effective 
      treatment are of major importance in BRIC, not only to decrease complications but 
      also improving patients' quality of life.
FAU - Helgadottir, Holmfridur
AU  - Helgadottir H
AUID- ORCID: 0000-0001-8688-449X
AD  - Department of Transplantation Medicine, Division of Surgery, Inflammatory 
      Medicine and Transplantation, Norwegian PSC Research Centre, Oslo University 
      Hospital Rikshospitalet, Oslo, Norway.
AD  - Department of Medicine, Haraldsplass Deaconess Hospital, Bergen, Norway.
FAU - Folvik, Geir
AU  - Folvik G
AD  - National Centre for Ultrasound in Gastroenterology, Haukeland University 
      Hospital, Bergen, Norway.
AD  - Department of Medicine, Haukeland University Hospital, Bergen, Norway.
FAU - Vesterhus, Mette
AU  - Vesterhus M
AUID- ORCID: 0000-0003-1493-2303
AD  - Department of Transplantation Medicine, Division of Surgery, Inflammatory 
      Medicine and Transplantation, Norwegian PSC Research Centre, Oslo University 
      Hospital Rikshospitalet, Oslo, Norway.
AD  - Department of Medicine, Haraldsplass Deaconess Hospital, Bergen, Norway.
AD  - Department of Clinical Science, University of Bergen, Bergen, Norway.
LA  - eng
PT  - Journal Article
DEP - 20221111
PL  - England
TA  - Scand J Gastroenterol
JT  - Scandinavian journal of gastroenterology
JID - 0060105
RN  - VJT6J7R4TR (Rifampin)
RN  - 0 (Gastrointestinal Agents)
RN  - Cholestasis, progressive familial intrahepatic 1
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Humans
MH  - Male
MH  - Young Adult
MH  - *Cholestasis, Intrahepatic/complications/drug therapy/diagnosis
MH  - Follow-Up Studies
MH  - Quality of Life
MH  - Recurrence
MH  - *Rifampin/administration & dosage/therapeutic use
MH  - *Gastrointestinal Agents/administration & dosage/therapeutic use
OTO - NOTNLM
OT  - Intrahepatic cholestasis
OT  - benign recurrent intrahepatic cholestasis
OT  - genetic liver disease
OT  - rifampicin
OT  - therapy
EDAT- 2022/11/13 06:00
MHDA- 2023/04/24 06:42
CRDT- 2022/11/12 01:32
PHST- 2023/04/24 06:42 [medline]
PHST- 2022/11/13 06:00 [pubmed]
PHST- 2022/11/12 01:32 [entrez]
AID - 10.1080/00365521.2022.2143725 [doi]
PST - ppublish
SO  - Scand J Gastroenterol. 2023 May;58(5):512-520. doi: 
      10.1080/00365521.2022.2143725. Epub 2022 Nov 11.