Summary
This is a case report of a 36 years old man who has been suffering for 20 years from benign recurrent intrahepatic cholestasis (BRIC). BRIC is a rare autosomal recessive disease characterised by prolonged episodes of intrahepatic cholestasis and pruritus alternating with periods of nearly normal liver function, and does not progress to cirrhosis. Since all former approaches to medical treatment of the patients severe pruritus were ineffective, the patient was treated by 3 sessions of albumin dialysis (MARS, Molecular Adsorbents Recirculating System). MARS dialysis decreased serum bilirubin levels by more than 60 % and effectively lowered serum bile acid levels by 45 %. The course of serum parameters was accompanied by a dramatic clinical improvement of the patients symptoms (pruritus, jaundice, fatigue etc.). MARS therapy appeared to shorten the duration of the cholestatic attack.
Key words
Albumin - Bile Acids - Bilirubin - Cholestasis - Detoxification - Dialysis - Liver - Pruritus
References
-
1
Summerskill W HJ, Walshe J M.
Benign recurrent intrahepatic ”obstructive“ jaundice.
Lancet.
1959;
2
686-90
-
2
Tygstrup N.
Intermittent possibly familial intrahepatic cholestatic jaundice.
Lancet.
1960;
1
1171-2
-
3
Houwen R HJ, Beharloo S, Blankenship K. et al .
Genome screening by searching for shared segments: mapping a gene for benign recurrent intrahepatic cholestasis.
Nat Genet.
1994;
8
380-386
-
4
Bull L N, van Eijk M JT, Pawlikowska L. et al .
A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis.
Nat Genet.
1998;
18
219-24
-
5
Stange J, Mitzner S R, Risler T. et al .
Molecular Adsorbent Recycling System (MARS): Clinical Results of a New Membrane-Based Blood Purification System for Bioartificial Liver Support.
Artif Organs.
1999;
23
319-30
-
6
Brenard R, Geubel A P, Benhamou J P.
Benign recurrent intrahepatic cholestasis. A report of 26 cases.
J Clin Gastroenterol.
1989;
11
546-551
-
7
Westermann G, Lügering N, August C. et al .
Ein klassischer Fall der benignen recurrierenden intrahepatischen Cholestase (Summerskill-Walshe-Tygstrup-Syndrom).
Med Klinik.
2000;
95
349-54
Author for correspondence
Dr. med. D. Huster
Department of Medicine II
University of Leipzig
Philipp-Rosenthal-Straße 27
04103 Leipzig
Email: husterd@medizin.uni-leipzig.de
