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Maternal tyrosinaemia II: Management and successful outcome

  • Metabolic Diseases
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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.

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Abbreviations

PKU:

phenylketonuria

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Authors and Affiliations

  1. Department of Nutrition and Dietetics, Royal Children's Hospital, Flemington Road, 3052, Parkville, Victoria, Australia

    D. E. M. Francis

  2. Murdoch Institute, Royal Children's Hospital, Flemington Road, 3052, Parkville, Victoria, Australia

    D. M. Kirby & G. N. Thompson

  3. Department of Paediatrics, University of Melbourne, 3052, Melbourne, Australia

    G. N. Thompson

Authors
  1. D. E. M. Francis
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  2. D. M. Kirby
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  3. G. N. Thompson
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Francis, D.E.M., Kirby, D.M. & Thompson, G.N. Maternal tyrosinaemia II: Management and successful outcome. Eur J Pediatr 151, 196–199 (1992). https://doi.org/10.1007/BF01954383

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