Nasobiliary drainage induces long-lasting remission in benign recurrent intrahepatic cholestasis

被引:91
作者
Stapelbroek, JM
van Erpecum, KJ
Klomp, LWJ
Venneman, NG
Schwartz, TP
Henegouwen, GPV
Devlin, J
van Nieuwkerk, CMJ
Knisely, AS
Houwen, RHJ
机构
[1] Univ Med Ctr, Dept Pediat Gastroenterol, NL-3508 AB Utrecht, Netherlands
[2] Univ Med Ctr, Dept Gastroenterol, NL-3508 AB Utrecht, Netherlands
[3] Univ Med Ctr, Lab Metab & Endocrine Dis, NL-3508 AB Utrecht, Netherlands
[4] Kings Coll Hosp London, Inst Liver Studies, London SE5 8RX, England
[5] Free Univ Med Ctr, Dept Gastroenterol, Amsterdam, Netherlands
关键词
D O I
10.1002/hep.20998
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Benign recurrent intrahepatic cholestasis (BRIC) is characterized by episodic cholestasis and pruritus without anatomical obstruction. Effective medical treatment is not available. We report complete and long-lasting disappearance of pruritus and normalization of serum bile salt concentrations in cholestatic BRIC patients within 24 hours after endoscopic nasobiliary drainage (NBD). Relative amounts of phospholipids and bile salts in bile collected during NBD appeared to be normal, but phospholipids other than phosphatidylcholine (especially sphingomyelin) were increased. In conclusion, we propose that temporary endoscopic nasobiliary drainage should be considered in cholestatic BRIC patients.
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页码:51 / 53
页数:3
相关论文
共 12 条
[1]
A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis [J].
Bull, LN ;
van Eijk, MJT ;
Pawlikowska, L ;
DeYoung, JA ;
Juijn, JA ;
Liao, M ;
Klomp, LWJ ;
Lomri, N ;
Berger, R ;
Scharschmidt, BF ;
Knisely, AS ;
Houwen, RHJ ;
Freimer, NB .
NATURE GENETICS, 1998, 18 (03) :219-224
[2]
Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease) [J].
Emond, JC ;
Whitington, PF .
JOURNAL OF PEDIATRIC SURGERY, 1995, 30 (12) :1635-1641
[3]
Kalicinski PJ, 2003, EUR J PEDIATR SURG, V13, P307
[4]
Characterization of mutations in ATP8B1 associated with hereditary cholestasis [J].
Klomp, LWJ ;
Vargas, JC ;
van Mil, SWC ;
Pawlikowska, L ;
Strautnieks, SS ;
van Eijk, MJT ;
Juijn, JJ ;
Pabón-Peña, C ;
Smith, LB ;
DeYoung, JA ;
Byrne, JA ;
Gombert, J ;
van der Brugge, G ;
Berger, R ;
Jankowska, I ;
Pawlowska, J ;
Villa, E ;
Knisely, AS ;
Thompson, RJ ;
Freimer, NB ;
Houwen, RHJ ;
Bull, LN .
HEPATOLOGY, 2004, 40 (01) :27-38
[5]
Melter M, 2000, AM J GASTROENTEROL, V95, P3522
[6]
A mouse genetic model for familial cholestasis caused by ATP8B1 mutations reveals perturbed bile salt homeostasis but no impairment in bile secretion [J].
Pawlikowska, L ;
Groen, A ;
Eppens, EF ;
Kunne, C ;
Ottenhoff, R ;
Looije, N ;
Knisely, AS ;
Killeen, NP ;
Bull, LN ;
Elferink, RPJO ;
Freimer, NB .
HUMAN MOLECULAR GENETICS, 2004, 13 (08) :881-892
[7]
A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis [J].
Strautnieks, SS ;
Bull, LN ;
Knisely, AS ;
Kocoshis, S ;
Dahl, N ;
Arnell, H ;
Sokal, E ;
Dahan, K ;
Childs, S ;
Ling, V ;
Tanner, MS ;
Kagalwalla, AF ;
Németh, A ;
Pawlowska, J ;
Baker, A ;
Mieli-Vergani, G ;
Freimer, NB ;
Gardiner, RM ;
Thompson, RJ .
NATURE GENETICS, 1998, 20 (03) :233-238
[8]
Familial intrahepatic cholestasis 1: Studies of localization and function [J].
Ujhazy, P ;
Ortiz, D ;
Misra, S ;
Li, SH ;
Moseley, J ;
Jones, H ;
Arias, IM .
HEPATOLOGY, 2001, 34 (04) :768-775
[9]
Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11 [J].
Van Mil, SWC ;
Van Der Woerd, WL ;
Van Der Brugge, G ;
Sturm, E ;
Jansen, PLM ;
Bull, LN ;
Van Den Berg, IET ;
Berger, R ;
Houwen, RHJ ;
Klomp, LWJ .
GASTROENTEROLOGY, 2004, 127 (02) :379-384
[10]
Benign recurrent intrahepatic cholestasis progressing to progressive familial intrahepatic cholestasis: low GGT cholestasis is a clinical continuum [J].
van Ooteghem, NAM ;
Klomp, LWJ ;
van Berge-Henegouwen, GP ;
Houwen, RHJ .
JOURNAL OF HEPATOLOGY, 2002, 36 (03) :439-443