Abstract | OBJECTIVES: STUDY DESIGN: Women with blood Phe levels >240 micromol/L (n=526; to convert micromol/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 micromol/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.
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Authors | Bobbye Rouse, Colleen Azen |
Journal | The Journal of pediatrics
(J Pediatr)
Vol. 144
Issue 2
Pg. 235-9
(Feb 2004)
ISSN: 0022-3476 [Print] United States |
PMID | 14760268
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
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Topics |
- Abnormalities, Multiple
(etiology)
- Child
- Child, Preschool
- Cognition
- Developmental Disabilities
(etiology)
- Diet, Protein-Restricted
- Face
(abnormalities)
- Female
- Fetal Growth Retardation
(etiology)
- Gestational Age
- Heart Defects, Congenital
(etiology)
- Humans
- Infant
- Infant, Newborn
- Intelligence Tests
- Microcephaly
(etiology)
- Phenylalanine
(blood)
- Phenylketonuria, Maternal
(complications, diet therapy)
- Pregnancy
- Pregnancy Outcome
- Prenatal Care
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