ANESTHETIC CONSIDERATIONS IN PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS (BYLERS DISEASE)

Progressive familial intrahepatic cholestasis (PFIC) or Byler's diseas e is one of the most common forms of intrahepatic cholestasis of metab olic and genetic origin Affected children progress to terminal cirrhos is 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 feature s of PFIC and the anaesthetic implications of chronic cholestasis in c hildren (malnutrition, cirrhosis, portal hypertension, chronic hypoxae mia) are reviewed.