Assessment of the nutritional status of infants and children with biliary atresia.

Since liver transplantation for children with end-stage liver disease has become accepted therapy clinicians have shown interest in the nutritional depletion that occurs with biliary atresia, since children with this condition form the largest group presenting for possible transplantation. Eleven infants and children with biliary atresia (age range 1,5 months-7 years) seen at the Red Cross War Memorial Children's Hospital over a 4-month period (March-June 1988) were studied. All but one had severe cholestasis at the time of the study. Nutrient intake during the hospitalised period was noted. Clinical nutritional parameters were documented and serum levels of vitamins A, D, E and the trace elements zinc and copper were measured. All patients with cholestasis (10/11) over 3 months of age (8/10) showed evidence of severe growth stunting, with weights below the 3rd percentile. Head circumference measurements were less than 5th percentile in 7/8 of those over 4 months of age including a 7-year-old child who had lost his jaundice after porto-enterostomy at age 2 months. All those with cholestasis showed evidence of fat-soluble vitamin deficiency and to a lesser extent zinc deficiency, but had raised serum copper levels. Three of 4 patients receiving cholestyramine had very low levels of vitamin E despite supplementation. These findings confirm the presence of severe nutritional depletion and growth stunting in patients with biliary atresia and the failure of 'normal' nutrient and vitamin supplementation to correct these deficiencies. The importance of close attention to nutrition in adequately preparing those patients assessed as suitable for liver transplantation therapy is stressed.