Abstract

The primary purpose of this project is to determine the best method of managing women during pregnancy who have hyperphenylalaninemia (HPA) including: classical phenylketonuria (PKU) - blood phenylalanine concentrations of more than 20 mg/dl while on a normal diet; variant PKU - 10-20 mg/dl; or benign HPA - 4-10 mg/dl. Fetal morbidity, with subsequent problems in growth and development, associated with maternal PKU will be assessed in relation to maternal phenylalanine levels.

  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 #
N01HD023155-010
Application #
6079346
Study Section
Project Start
1992-05-01
Project End
2000-04-30
Budget Start
1999-05-01
Budget End
2000-04-30
Support Year
Fiscal Year
1999
Total Cost
Indirect Cost

  Institution

Name
University of Illinois at Chicago
Department
Nutrition
Type
Schools of Allied Health Profes
DUNS #
121911077
City
Chicago
State
IL
Country
United States
Zip Code
60612

  Publications

Hanley, William B (2008) Finding the fertile woman with phenylketonuria. Eur J Obstet Gynecol Reprod Biol 137:131-5
Rouse, Bobbye; Azen, Colleen (2004) Effect of high maternal blood phenylalanine on offspring congenital anomalies and developmental outcome at ages 4 and 6 years: the importance of strict dietary control preconception and throughout pregnancy. J Pediatr 144:235-9
Hanley, W B; Azen, C; Koch, R et al. (2004) Maternal Phenylketonuria Collaborative Study (MPKUCS)--the 'outliers'. J Inherit Metab Dis 27:711-23
Michals-Matalon, Kimberlee; Platt, Lawrence D; Acosta P, Phyllis et al. (2002) Nutrient intake and congenital heart defects in maternal phenylketonuria. Am J Obstet Gynecol 187:441-4
Koch, R; Friedman, E; Azen, C et al. (2000) The International Collaborative Study of Maternal Phenylketonuria: status report 1998. Eur J Pediatr 159 Suppl 2:S156-60
Rouse, B; Matalon, R; Koch, R et al. (2000) Maternal phenylketonuria syndrome: congenital heart defects, microcephaly, and developmental outcomes. J Pediatr 136:57-61
Hanley, W B; Platt, L D; Bachman, R P et al. (1999) Undiagnosed maternal phenylketonuria: the need for prenatal selective screening or case finding. Am J Obstet Gynecol 180:986-94
Koch, R; Fishler, K; Azen, C et al. (1997) The relationship of genotype to phenotype in phenylalanine hydroxylase deficiency. Biochem Mol Med 60:92-101
Levy, H L; Goss, B S; Sullivan, D K et al. (1994) Maternal mild hyperphenylalaninemia: results of treated and untreated pregnancies in two sisters. J Pediatr 125:467-9