title: Anaesthetic considerations in progressive familial intrahepatic cholestasis (Byler's disease).
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.
authors.affiliation: Department of Anaesthesiology, Catholic University of Louvain Medical School, Cliniques Universitaires St Luc, Brussels, Belgium.
authors.forename: G
authors.initials: G
authors.lastname: Müller
authors.forename: F
authors.initials: F
authors.lastname: Veyckemans
authors.forename: M
authors.initials: M
authors.lastname: Calier
authors.forename: L J
authors.initials: LJ
authors.lastname: Van Obbergh
authors.forename: M
authors.initials: M
authors.lastname: De Kock
authors.forename: E M
authors.initials: EM
authors.lastname: Sokal
authors.forename: J B
authors.initials: JB
authors.lastname: Otte
mesh_terms: D000293:Adolescent; D000768:Anesthesia, General; D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000235:genetics / Q000601:surgery*; D002908:Chronic Disease; D005192:Family Health; D005260:Female; D006801:Humans; D007223:Infant; D008103:Liver Cirrhosis / Q000209:etiology; D008297:Male; D011300:Preoperative Care; D012189:Retrospective Studies
chemical_list: D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous