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The management of breast feeding among infants with phenylketonuria
Author(s) -
McCabe L.,
Ernest A. E.,
Neifert M. R.,
Yannicelli S.,
Nord A. M.,
Garry P. J.,
McCabe E. R. B.
Publication year - 1989
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01802044
Subject(s) - phenylalanine , medicine , breast feeding , breast milk , endocrinology , pediatrics , tyrosine , physiology , gastroenterology , chemistry , biochemistry , amino acid
Summary Treatment for phenylketonuria (PKU) involves using low phenylalanine or phenylalanine‐free formulas and supplementation with sufficient phenylalanine for normal growth and development. Eighteen infants with phenylketonuria who received breast milk as their primary phenylalanine source were compared with ten other infants with PKU who received their phenylalanine primarily from infant formulas. There were no significant differences between breast‐fed and formula‐fed infants for serum phenylalanine, serum tyrosine, length, weight, head circumference, haematocrit, haemoglobin, serum iron, total iron binding capacity, percentage iron saturation, ferritin, plasma zinc and total calorie intake. Breast‐fed infants did show lower mean corpuscular volume at 3 months and 6 months of age. Breast‐fed infants had lower phenylalanine intake at 2, 4, 5 and 6 months of age. Breast‐fed infants at 1, 2, 3, 4, 5 and 6 months of age had lower protein intake. Breast feeding may be continued in the newly diagnosed phenylketonuric infant without any apparent adverse nutritional consequences.