Propofol Pharmacokinetics in Children With Biliary Atresia

We studied the pharmacokinetics of an i.v. bolus dose of propofol 2.5-3.0 mg kg(-1) in eight children (age 4-24 months) with biliary atresia and in six control (ASA I) children (age 11-43 months). Blood samples were obtained for 4 h after administration of propofol. Blood concentrations of propofol were measured by high pressure liquid chromatography. Systemic clearance of propofol (Cl) and volume of distribution at steady state (V-ss) showed a highly significant correlation with body weight. Propofol Cl and V-ss, normalized for body weight, were similar in children with biliary atresia (mean 37.5 (SD 8.3) ml min(-1) kg(-1) and 3.5 (1.6) litre kg(-1), respectively) compared with control children (38.7 (6.8) ml min(-1) kg(-1) and 2.4 (0.8) litre(-1) kg(-1), respectively). We conclude that in children with biliary atresia the pharmacokinetics of propofol are similar to those of healthy children

Anesthetic Considerations in Progressive Familial Intrahepatic Cholestasis (bylers Disease)

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