Maternal tyrosinaemia II: Management and successful outcome
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- Francis, D.E.M., Kirby, D.M. & Thompson, G.N. Eur J Pediatr (1992) 151: 196. doi:10.1007/BF01954383
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A 25-year-old woman with tyrosinaemia type II was treated from the 5th week of pregnancy with a protein-restricted diet supplemented with a tyrosine/phenylalanine-free amino acid mixture. Tyrosine concentrations were maintained in the range 100–200 μmol/l by restricting natural protein intake to 0.16 g/kg per 24h in early pregnancy, with increases up to 0.38 g/kg per 24h in the last trimester. This treatment maintained plasma phenylalanine concentrations in the range 20–40 μmol/l. Maternal weight gain and fetal growth were normal, and the mother remained asymptomatic throughout the pregnancy. A normal infant was born at term with length, weight and head circumference between the 25–50 th per centiles.
Key wordsDiet Pregnancy Amino acids Protein