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Canadian Journal of Anaesthesia

, Volume 42, Issue 12, pp 1126–1133 | Cite as

Anaesthetic considerations in progressive familial intrahepatic cholestasis (Byler’s disease)

  • Gerhard Müller
  • Francis Veyckemans
  • Marianne Carlier
  • Luc J. Van Obbergh
  • Marc De Kock
  • Etienne M. Sokal
  • Jean-Bernard Otte
Occasional Reviews
  • 362 Downloads

Abstract

Progressive familial intrahepatic cholestasis (PFIC) or Byler’s disease is one of the most common forms of intrahepatic cholestasis of metabolic and genetic origin. Affected children progress to terminal cirrhosis before adulthood and at present the only curative treatment of PFIC is orthotopic liver transplantation (OLT). We present a retrospective review of 40 general anaesthetics administered in our hospital to 22 patients with PFIC undergoing various procedures. The clinical features of PFIC and the anaesthetic implications of chronic cholestasis in children (malnutrition, cirrhosis, portal hypertension, chronic hypoxaemia) are reviewed.

Key words

anaesthesia: paediatric, inhalation, intravenous liver: cholestasis, cirrhosis, hypoxaemia syndromes: Byler’s disease, progressive familial intrahepatic cholestasis 

Résumé

La cholestase intrahépatique progressive familiale (PFIC) ou maladie de Byler est une des formes les plus courantes de cholestase intrahépatique de cause métabolique et génétique. Les enfants qui en sont atteints évoluent vers la cirrhose terminale avant l’âge adulte. Jusqu’à présent, le seul traitement curatif de la PFIC est la transplantation hépatique. Nous avons revu de manière rétrospective 40 anesthésies générales administrées dans notre hôpital à 22 enfants souffrant de PFIC. Les différentes présentations cliniques de la PFIC et les implications anesthésiques de la cholestase chronique de l’enfant (malnutrition, cirrhose, hypertension portale, hypoxémie chronique) sont présentées.

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Copyright information

© Canadian Anesthesiologists 1995

Authors and Affiliations

  • Gerhard Müller
    • 3
  • Francis Veyckemans
    • 3
  • Marianne Carlier
    • 3
  • Luc J. Van Obbergh
    • 3
  • Marc De Kock
    • 3
  • Etienne M. Sokal
    • 3
    • 1
  • Jean-Bernard Otte
    • 3
    • 2
  1. 1.Department of Paediatric GastroenterologyCatholic University of Louvain Medical School, Cliniques Universitaires St LucBrusselsBelgium
  2. 2.Department of Paediatric SurgeryCatholic University of Louvain Medical School, Cliniques Universitaires St LucBrusselsBelgium
  3. 3.Departments of AnaesthesiologyCatholic University of Louvain Medical School, Cliniques Universitaires St LucBrusselsBelgium

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