Phenylketonuria (PKU) is an inherited inborn error of amino acid metabolism. Deficiency in phenylalanine hydroxylase (PAH), inherited autosomal recessively, prevents conversion of phenylalanine to tyrosine causing accumulation of phenylalanine. Subsequent increase of phenylalanine levels in the brain to toxic levels causes the clinical manifestations of the disease. Strategies for medical management that is safe for both mother and fetus will be presented. The anesthetic management of labor and delivery as well as operative deliveries will be discussed.
KeywordsInborn errors of metabolism Anesthesia Obstetrical Phenylketonuria Pregnancy
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