title: The Maternal Phenylketonuria International Study: 1984-2002.
abstract: OBJECTIVE
  The purpose of this report is to review the obstetric medical, psychological, and nutritional aspects and outcome of the women and offspring enrolled in the Maternal Phenylketonuria Study, which was established to assess the efficacy of a phenylalanine (Phe)-restricted diet in preventing the morbidity associated with this disorder.
  
  
  METHODS
  A total of 382 women with hyperphenylalaninemia (HPA) were enrolled in the study and completed 572 pregnancies. Outcome measures were analyzed with chi2, Fisher exact text, analysis of variance, t test, Wilcoxon nonparametric test, and multiple logistic regression. Outcome measures were stratified according to maternal HPA classification and the time when dietary control was achieved.
  
  
  RESULTS
  Optimal birth outcomes occurred when maternal blood Phe levels between 120 and 360 micromol/L were achieved by 8 to 10 weeks of gestation and maintained throughout pregnancy (trimester averages of 600 micromol/L). Mothers with mild HPA achieved similar birth outcomes as mothers who were in control preconceptually and those in control by 8 to 10 weeks of pregnancy.
  
  
  CONCLUSIONS
  Before conception, counseling and early entrance into a prenatal care program is essential in achieving optimal fetal outcome in women with HPA. The achievement of pre- and periconceptional dietary control with a Phe-restricted diet significantly decreased morbidity in the offspring of women with HPA.
authors.affiliation: Childrens Hospital of Los Angeles and the University of Southern California School of Medicine, Los Angeles, California 90027, USA. rkoch8@earthlink.net
authors.forename: Richard
authors.initials: R
authors.lastname: Koch
authors.forename: William
authors.initials: W
authors.lastname: Hanley
authors.forename: Harvey
authors.initials: H
authors.lastname: Levy
authors.forename: Kim
authors.initials: K
authors.lastname: Matalon
authors.forename: Reuben
authors.initials: R
authors.lastname: Matalon
authors.forename: Bobbye
authors.initials: B
authors.lastname: Rouse
authors.forename: Frederick
authors.initials: F
authors.lastname: Trefz
authors.forename: Flemming
authors.initials: F
authors.lastname: Güttler
authors.forename: Colleen
authors.initials: C
authors.lastname: Azen
authors.forename: Larry
authors.initials: L
authors.lastname: Platt
authors.forename: Susan
authors.initials: S
authors.lastname: Waisbren
authors.forename: Keith
authors.initials: K
authors.lastname: Widaman
authors.forename: Jiaping
authors.initials: J
authors.lastname: Ning
authors.forename: Eva G
authors.initials: EG
authors.lastname: Friedman
authors.forename: Felix
authors.initials: F
authors.lastname: de la Cruz
mesh_terms: D000704:Analysis of Variance; D001724:Birth Weight; D002648:Child; D002675:Child, Preschool; D005314:Embryonic and Fetal Development; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007360:Intelligence; D016015:Logistic Models; D010649:Phenylalanine / Q000097:blood; D017042:Phenylketonuria, Maternal / Q000097:blood / Q000178:diet therapy*; D010661:Phenylketonurias / Q000178:diet therapy; D011247:Pregnancy; D011256:Pregnancy Outcome; D011295:Prenatal Care; D018709:Statistics, Nonparametric
chemical_list: D010649:Phenylalanine