Abstract | AIMS: METHODS: During the last 20 years, we have treated 52 children with PFIC. PEBD was done in 21 patients (since 1995), and IB in 5 patients (since 1998), transplantation was performed in 9 patients (since 1990). The efficacy of non- transplantation surgical treatment was assessed by patients' clinical outcome, liver biochemistry, and survival without transplantation during a follow-up period of 12 to 48 months. RESULTS: In 15 out of 21 patients clinical symptoms improved after PEBD and liver function tests normalised (blood bile acids), 1 patient had to be converted to IB due to too high output biliary fistula, 2 patients were transplanted and 3 are considered for transplantation. Out of the 5 children after IB, 4 improved clinically and biochemically, but, after 12 months, symptoms recurred in 3 patients, one patient was converted successfully to PEBD. No significant influence on growth was observed, irrespective of the type of treatment in this group of patients. CONCLUSIONS: PEBD is more effective than IB for the permanent improvement of symptoms of PFIC. Ileal bypass procedure, although initially effective, does not ensure good long-term results in more than 50 % of patients, probably due to intestinal re-absorption of bile acids increasing over time.
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Authors | P J Kaliciński, H Ismail, I Jankowska, A Kamiński, J Pawłowska, T Drewniak, M Markiewicz, M Szymczak |
Journal | European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur Kinderchirurgie
(Eur J Pediatr Surg)
Vol. 13
Issue 5
Pg. 307-11
(Oct 2003)
ISSN: 0939-7248 [Print] United States |
PMID | 14618520
(Publication Type: Comparative Study, Evaluation Study, Journal Article)
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Topics |
- Adolescent
- Biliary Tract Surgical Procedures
(methods)
- Child
- Child, Preschool
- Cholestasis, Intrahepatic
(surgery)
- Female
- Follow-Up Studies
- Humans
- Infant
- Male
- Treatment Outcome
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