Hyperphenylalaninaemia and outcome in intravenously fed preterm neonates

Abstract

Hyperphenylalaninaemia is likely to have occurred in many infants fed the intravenous amino acid solution Vamin 9. In this study of 336 preterm infants plasma phenylalanine was measured weekly during their hospital stay. Reference data on plasma phenylalanine were prepared for 243 infants who did not receive Vamin. Only 1% of these infants had a peak plasma phenylalanine concentration greater than 150 μmol/l (maximum 202, μmol/l) compared with 23% in 93 infants fed Vamin 9, seven of whom had concentrations >300 μmol/l (maximum 704 μmol/l). High concentrations only occurred when the total energy to protein energy ratio in the intravenous solutions decreased to less than 8 5:1 and always occurred with a ratio less than 6 5:1, implying that hyperphenylalaninaemia may be minimised with an intravenous energy intake of greater than 34 kcal (142 kJ)/g protein. Nevertheless, foliow up at 18 months post-term showed that increased plasma phenylalanine in this instance was not associated with any impairment of the Bayley mental development index (or subscales including fine motor, cognitive, or language development), the psychomotor development index, or the social maturity quotient. Thus, despite theoretical concern, an adverse outcome after hyperphenylalaninaemia induced by intravenous feeding has not been observed.