Medical versus surgical management of progressive familial intrahepatic cholestasis - PFIC
| Journal | Volume 68 - 2005 |
| Issue | Fasc.4 - Symposium |
| Author(s) | Jankowska I., Pawlowska J., Kaliciński P., Ismail H., Kamiński A., Lacaille F., Revillon Y., Socal E., Strautnieks SS, Thompson R, Bull L.N., Socha J |
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| (1) Department of Gastroenterology and Immunology, (2) Department of Surgery and Transplantology, The Children's Memorial Health Institute, Warsaw. (3) Department of Gastroenterology and Nutrition , Necker-Enfants Malades Hospital, Paris, (4) Department of Pediatric Surgery, Necker-Enfants Malades Hospital, Paris, (5) Department of Hepatology, Catholic University, Brussels, (6) Department of Liver Studies and Transplantation, King's College London, 6UCSF Liver Center Laboratory, San Francisco General Hospital, San FranciscO. CA, USA. |
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Pharmacological methods used so far for treating PFIC have not proved clear efficacy. Clinical and bio- chemical improvement was observed only in a few chil- dren treated with ursodeoxycholic acid (UDCA). In the last decade partial external biliary diversion (PEBD) became standard procedure performed on PFIC children with no response to medical treatment. There is still a group of patients who cannot benefit from this procedure because of technical conditions (earlier per- formed cholecystectomy) or postoperative complica- tions like dyselectrolytaemia due to the excessive amount of bile. In 1998 Holland et al. described ileal bypass (IB) as promising in PFIC children after chole- cystectomy. The only treatment for children with PFIC and hepatic cirrhosis remains liver transplantation. The aim of our study was a retrospective evaluation of different methods of treatment in children with PFIC: pharmacological therapy with phenobarbital, cholestyra- mine and UDCA, as well as surgical methods, PEBD, IB, as alternative methods to liver transplantation |
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© Acta Gastro-Enterologica Belgica. |
