TRANSIENT TYROSINEMIA OF THE NEWBORN: DIETARY AND CLINICAL ASPECTS
Mary Ellen Avery M.D.1,
Carol L. Clow R.T.1,
John H. Menkes M.D.1,
Angeles Ramos M.D.1,
Charles R. Scriver M.D.1,
Leo Stern M.D.1, and
Barbara P. Wasserman A.B.1
1 The deBelle Laboratory for Biochemical Genetics, and the Premature Unit, at the Montreal Children's Hospital; Department of Pediatrics, McGill University, Montreal, P.Q.; and The Harriet Lane Service, Department of Pediatrics, Johns Hopkins Medical School, Baltimore
Neonatal tyrosinemia occurs with high frequency in newborn infants. The severity and prevalence of tyrosinemia is greater with higher protein intakes (e.g., 6 gm/kg/day) and in the more immature infant. Ascorbic acid in "recommended" amounts will not always prevent tyrosinemia if the substrate intake is great and the infant immature and presumably deficient in apoenzyme (p-hydroxyphenylpyruvic acid oxidase). Tyrosinemia itself produces no consistent clinical syndrome and in general can be deemed relatively innocuous but each infant should be assessed as an individual, since the occasional patient shows clinical manifestations of lethargy. There appears to be no indication for a change in general dietary recommendations for newborn infants.
Submitted on August 12, 1966
Accepted on September 19, 1966
