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

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Müller, G Veyckemans, Francis [UCL] Calier, M. Van Obbergh, L J De Kock, Marc [UCL] Sokal, Etienne [UCL] Otte, Jean-Bernard [UCL]

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.

Document type Article de périodique (Journal article) – Journal Article
Publication date 1995
Journal information "Canadian journal of anaesthesia = Journal canadien d'anesthésie" - Vol. 42, no. 12, p. 1126-33 (1995)
Peer reviewed yes
issn 0832-610X
Publication status Publié
Affiliations UCL - MD/CHIR - Département de chirurgie
UCL - MD/GYPE - Département de gynécologie, d'obstétrique et de pédiatrie
MESH Subject Adolescent ; Anesthesia, General ; Anesthetics, Inhalation ; Anesthetics, Intravenous ; Child ; Child, Preschool ; Cholestasis, Intrahepatic - complications - genetics - surgery ; Chronic Disease ; Family Health ; Female ; Humans ; Infant ; Liver Cirrhosis - etiology ; Male ; Preoperative Care ; Retrospective Studies
Links
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Bibliographic reference Müller, G ; Veyckemans, Francis ; Calier, M. ; Van Obbergh, L J ; De Kock, Marc ; et. al. Anaesthetic considerations in progressive familial intrahepatic cholestasis (Byler's disease).. In: Canadian journal of anaesthesia = Journal canadien d'anesthésie, Vol. 42, no. 12, p. 1126-33 (1995)
Permanent URL http://hdl.handle.net/2078.1/22505