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Growth and nutrition: long term follow-up in early treated phenylketonuric and hyperphenylalaninemic children

Koepp, P.; Hinze, G.; Held, K.R.

Monatsschrift Kinderheilkunde Organ der Deutschen Gesellschaft für Kinderheilkunde 129(3): 154-159

1981

ISSN/ISBN: 0026-9298
PMID: 6112681
Document Number: 172969
In a retrospective longitudinal study physical growth was related to nutritional data in 35 (23 male, 12 female) early treated patients with phenylketonuria and hyperphenylalaninemic variants. In males growth was slightly retarded only in the first year of life, weight gain was normal in both sexes as was growth in females. Bone changes were noted in 10/33 patients. Protein was prescribed as phenylalanine free protein hydrolysate or phenylalanine free aminoacid mixture and as natural protein according to individual phenylalanine tolerance. In 34/35 patients total serum protein was normal whereas hemoglobin was near the lower normal limit. Serum phenylalanine increased continuously with age.

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