Maternal phenylketonuria

A rare disorder of phenylalanine metabolism, an inborn error of amino acid metabolism, characterized by the development of microcephaly, growth retardation, congenital heart disease, facial dysmorphism and intellectual disability in nonphenylketonuric offspring of mothers with excess phenylalanine (Phe) concentrations.

Hyperphenylalaninemia

An increased concentration of L-phenylalanine in the blood.


Total: 1

                      


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PMID (PMCID)
19485248
FEMALE Infant, Newborn
[The maternal phenylketonuria syndrom--still current problem].
Didycz B, Domagala L, Pietrzyk JJ.
Przegl Lek. 2009;66(1-2):4-10.
All pregnancies of women with hyperphenylalaninemia should be planned to avoid the complications in the form of maternal PKU syndrom.