In the early days of
parenteral nutrition of children
liver disease resulting in steatosis and
cholestasis was assumed to be an inevitable complication of the procedure. Since then, the management of
parenteral nutrition has improved so much that nowadays adolescents have a fair chance of surviving more than 15 to 20 years without severe
liver disease. Nevertheless, we still see cases of
parenteral nutrition-associated
cholestasis (PNAC) due to various conditions such as
recurrent infections, inflammatory response, inappropriate composition of the nutrient mixture, contaminants of the nutrient
solution, and toxic substances from infusion bags and tubes. Recent research indicates that the administration of ursodesoxycholic
acid and
cysteine can prevent or even improve the
cholestasis. A reversal of PNAC has been documented in an adolescent after small bowel
transplantation from Japan. There is ample opportunity for prevention of PNAC with respect to the various pathophysiologic aspects: prevention, early detection, and management of
infections, avoiding
glucose overloads, cyclic infusion of nutrients, light protection of the
solution, choice of paediatric
amino acid solutions, and most important, oral or
enteral feeding to support the bile flow by stimulating the cholecystokinine release.