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 - 24/08/11
, Colleen Azen, MS
Reprint requests: Bobbye Rouse, MD, Department of Pediatrics, Children's Hospital, University of Texas Medical Branch, 301 University Blvd, Galveston, TX 77555-0359.Abstract |
Objectives |
The Maternal Phenylketonuria Study was designed to determine the effect of a phenylalanine (Phe)-restricted diet in reducing the morbidity on the fetus. Congenital abnormalities were noted, with the focus on the effect of congenital heart defects (CHDs) and microcephaly (MICRO) on developmental outcome at 4 and 6 years of age.
Study design |
Women with blood Phe levels >240 μmol/L (n=526; to convert μmol/L to mg/dL, divide by 60) were enrolled; 382 contributed 572 pregnancies. The women had 413 offspring examined at birth and annually. At 4 years, the McCarthy General Cognitive Index was administered, and at 6 years, the Wechsler Intelligence Scale for Children Revised was administered.
Results |
Microcephaly was noted in 137 (33%) of the offspring, and 32 (7.7%) had CHD. Maternal blood Phe levels were higher for infants with CHD and MICRO than for infants with CHD only (P=.02). Mean Phe levels at 4 to 8 weeks gestation predicted CHD (P<.0001). The McCarthy General Cognitive Index score was lower with CHD (P=.005) and MICRO (P=.0017), as was the Wechsler Intelligence Scale for Children Revised full-scale IQ score (P=.0002 for CHD and P=.0001 for MICRO). None of the subjects who had offspring with CHD had Phe control between 120 and 360 μmol/L during the first 8 to 10 weeks of gestation.
Conclusions |
Women with phenylketonuria need to be educated regarding diet for life. This should help improve diet control before conception and throughout pregnancy.
Le texte complet de cet article est disponible en PDF.Abbreviations : APE, CHD, HPA, MICRO, Phe, PKU
Plan
 | Supported by the National Institute of Child Health and Human Development, contract nos. NO1-HD-2-3148, NO1-HD-2-3149, NO1-HD-2-3155, and NO1-HD-3156; the Grant National Health Research and Development Program, Ottawa, no. 6606-3265 GCRC, UTMB, MO1-RR-00073; the National Center for Research Resources; the National Institutes of Health; and United States Public Health Service. |
Vol 144 - N° 2
P. 235-239 - février 2004 Retour au numéro
Article précédent
- The effect of participation in the WIC program on preschoolers' diets
- Anna Maria Siega-Riz, Sibylle Kranz, Dan Blanchette, Pamela S. Haines, David K. Guilkey, Barry M. Popkin
- Maternal age and other predictors of newborn blood pressure
- Matthew W Gillman, Janet W Rich-Edwards, Sheryl L Rifas-Shiman, Ellice S Lieberman, Ken P Kleinman, Steven E Lipshultz
Bienvenue sur EM-consulte, la référence des professionnels de santé.
L’accès au texte intégral de cet article nécessite un abonnement.
Déjà abonné à cette revue ?