Document Detail

The international collaborative study of maternal phenylketonuria: status report 1994.
MedLine Citation:
PMID:  7766945     Owner:  NLM     Status:  MEDLINE    
Neonatal screening for phenylketonuria (PKU) has created a problem as females with PKU are reaching child-bearing age. Surveys have revealed that maternal phenylalanine blood concentrations greater than 1200 mumol/l are associated with fetal microcephaly, congenital heart defects and intrauterine growth retardation. It is estimated that as many as 3000 hyperphenylalaninemic females may be at risk of producing these fetal abnormalities. To examine this problem, the international maternal PKU collaborative study was developed to evaluate the efficacy of a phenylalanine-restricted diet in reducing fetal morbidity. Preliminary findings have indicated that phenylalanine restriction should begin before conception for females with PKU planning a pregnancy. Dietary control should maintain maternal blood phenylalanine levels between 120 and 360 mumol/l and should provide adequate energy, protein, vitamin and mineral intake. Pregnant hyperphenylalaninemic females who achieved metabolic control after conception or by the 10th week of pregnancy had a better offspring outcome than anticipated. The results of 402 pregnancies are reviewed.
R Koch; H L Levy; R Matalon; B Rouse; W B Hanley; F Trefz; C Azen; E G Friedman; F de la Cruz; F Güttler
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Publication Detail:
Type:  Clinical Trial; Controlled Clinical Trial; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Acta paediatrica (Oslo, Norway : 1992). Supplement     Volume:  407     ISSN:  0803-5326     ISO Abbreviation:  Acta Paediatr Suppl     Publication Date:  1994 Dec 
Date Detail:
Created Date:  1995-07-06     Completed Date:  1995-07-06     Revised Date:  2008-05-12    
Medline Journal Info:
Nlm Unique ID:  9315043     Medline TA:  Acta Paediatr Suppl     Country:  NORWAY    
Other Details:
Languages:  eng     Pagination:  111-9     Citation Subset:  IM    
Children's Hospital Los Angeles, CA 90027, USA.
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MeSH Terms
Case-Control Studies
International Cooperation
Phenylalanine / blood
Phenylketonuria, Maternal / blood,  complications,  diet therapy*
Pregnancy Outcome*
Prospective Studies
Grant Support
Reg. No./Substance:

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