European Journal of Pediatrics

, Volume 151, Issue 3, pp 196–199

Maternal tyrosinaemia II: Management and successful outcome

  • D. E. M. Francis
  • D. M. Kirby
  • G. N. Thompson
Metabolic Diseases

DOI: 10.1007/BF01954383

Cite this article as:
Francis, D.E.M., Kirby, D.M. & Thompson, G.N. Eur J Pediatr (1992) 151: 196. doi:10.1007/BF01954383

Abstract

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 words

Diet Pregnancy Amino acids Protein 

Abbreviation

PKU

phenylketonuria

Copyright information

© Springer-Verlag 1992

Authors and Affiliations

  • D. E. M. Francis
    • 1
  • D. M. Kirby
    • 2
  • G. N. Thompson
    • 2
    • 3
  1. 1.Department of Nutrition and DieteticsRoyal Children's HospitalParkvilleAustralia
  2. 2.Murdoch InstituteRoyal Children's HospitalParkvilleAustralia
  3. 3.Department of PaediatricsUniversity of MelbourneMelbourneAustralia

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