Abstract

The level of maternal phenylalanine which will maintain a normal pregnancy. At what stage of pregnancy a low phenylalanine diet is most effective in preventing the effects of maternal PKU on the developing fetus. If the beneficial effects of a low phenylalanine diet can be improved when initiated prior to conception compared to post-conception. Whether the diet reduces the frequency of mental retardation, spontaneous abortions, low birth weight,congenital malformations, neurological and behavioral impairment found in infants of mothers with hyperphenylalaninemia.The levels of tyrosine and trace metals during pregnancy and ascertain the effects of supplementation if levels are found to be low on pregnancy outcome.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Type
Research and Development Contracts (N01)
Project #
N01HD043807-010
Application #
2315479
Study Section
Project Start
1984-05-01
Project End
1991-04-14
Budget Start
1990-06-15
Budget End
1991-04-14
Support Year
Fiscal Year
1990
Total Cost
Indirect Cost

  Institution

Name
Children's Hospital of Los Angeles
Department
Type
DUNS #
094878337
City
Los Angeles
State
CA
Country
United States
Zip Code
90027

  Publications

Azen, C; Koch, R; Friedman, E et al. (1996) Summary of findings from the United States Collaborative Study of children treated for phenylketonuria. Eur J Pediatr 155 Suppl 1:S29-32
Koch, R; Wenz, E; Azen, C et al. (1993) The effect of nutrient intake on pregnancy outcome in maternal phenylketonuria. Ann N Y Acad Sci 678:348-9
Platt, L D; Koch, R; Azen, C et al. (1992) Maternal phenylketonuria collaborative study, obstetric aspects and outcome: the first 6 years. Am J Obstet Gynecol 166:1150-60;discussion 1160-2
Azen, C G; Koch, R; Friedman, E G et al. (1991) Intellectual development in 12-year-old children treated for phenylketonuria. Am J Dis Child 145:35-9
Di Silvestre, D; Pandya, A; Koch, R et al. (1990) DNA haplotype analyses of patients with hyperphenylalaninemia. Am J Hum Genet 47:706-11
Koch, R; Hanley, W; Levy, H et al. (1990) A preliminary report of the collaborative study of maternal phenylketonuria in the United States and Canada. J Inherit Metab Dis 13:641-50
Lang, M J; Koch, R; Fishler, K et al. (1989) Nonphenylketonuric hyperphenylalaninemia. Am J Dis Child 143:1464-6
Ledley, F D; Koch, R; Jew, K et al. (1988) Phenylalanine hydroxylase expression in liver of a fetus with phenylketonuria. J Pediatr 113:463-8
Koch, R; Wenz, E; Bauman, C et al. (1988) Treatment outcome of maternal phenylketonuria. Acta Paediatr Jpn 30:410-6
Koch, R; Friedman, E G; Wenz, E et al. (1986) Maternal phenylketonuria. J Inherit Metab Dis 9 Suppl 2:159-68