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 #
N01HD023148-016
Application #
6077329
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
Children's Hospital of Los Angeles
Department
Type
DUNS #
094878337
City
Los Angeles
State
CA
Country
United States
Zip Code
90027

  Publications

Hanley, William B (2008) Finding the fertile woman with phenylketonuria. Eur J Obstet Gynecol Reprod Biol 137:131-5
Brumm, V L; Azen, C; Moats, R A et al. (2004) Neuropsychological outcome of subjects participating in the PKU adult collaborative study: a preliminary review. J Inherit Metab Dis 27:549-66
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
Matalon, Reuben; Surendran, Sankar; Matalon, Kimberlee Michals et al. (2003) Future role of large neutral amino acids in transport of phenylalanine into the brain. Pediatrics 112:1570-4
Koch, Richard; Azen, Colleen; Friedman, Eva et al. (2003) Research design, organization, and sample characteristics of the Maternal PKU Collaborative Study. Pediatrics 112:1519-22
Guttler, Flemming; Azen, Colleen; Guldberg, Per et al. (2003) Impact of the phenylalanine hydroxylase gene on maternal phenylketonuria outcome. Pediatrics 112:1530-3
Widaman, Keith F; Azen, Colleen (2003) Relation of prenatal phenylalanine exposure to infant and childhood cognitive outcomes: results from the International Maternal PKU Collaborative Study. Pediatrics 112:1537-43
Koch, Richard; Hanley, William; Levy, Harvey et al. (2003) The Maternal Phenylketonuria International Study: 1984-2002. Pediatrics 112:1523-9
Waisbren, Susan E; Azen, Colleen (2003) Cognitive and behavioral development in maternal phenylketonuria offspring. Pediatrics 112:1544-7

Showing the most recent 10 out of 32 publications