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.
| Kecskemethy, H H; Lobbregt, D; Levy, H L (1993) The use of gelatin capsules for ingestion of formula in dietary treatment of maternal phenylketonuria. J Inherit Metab Dis 16:111-8 |
| 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 |
| Levy, H L; Lobbregt, D; Sansaricq, C et al. (1992) Comparison of phenylketonuric and nonphenylketonuric sibs from untreated pregnancies in a mother with phenylketonuria. Am J Med Genet 44:439-42 |
| Levy, H L; Lobbregt, D; Koch, R et al. (1991) Paternal phenylketonuria. J Pediatr 118:741-3 |
| Waisbren, S E; Levy, H L (1990) Effects of untreated maternal hyperphenylalaninemia on the fetus: further study of families identified by routine cord blood screening. J Pediatr 116:926-9 |
