Financial justification of investments into special diet for patients with phenylketonuria
Author(s) -
Georgios Konstantinidis,
Dobrila Radovanov,
Nada Konstantinidis
Publication year - 2010
Publication title -
medicinski pregled
Language(s) - English
Resource type - Journals
eISSN - 1820-7383
pISSN - 0025-8105
DOI - 10.2298/mpns1012771k
Subject(s) - phenylalanine , phenylalanine hydroxylase , medicine , tyrosine , phenylketonurias , amino acid , pediatrics , biochemistry , biology
Phenylketonuria is a genetic disorder of metabolism of amino acid phenylalanine, which results in the absence of phenylalanine hydroxylase, an enzyme that catalyzes the conversion of phenylalanine into tyrosine. It is an autosomal recessive disorder. Screening for phenylketonuria in Voivodina started in 2003. Screening data are shown in this paper. Treatment of phenylketonuria is based on a strict, life long, low protein diet with the controlled phenylalanine intake. Diet must start early, in the first weeks of life. The aim of the diet is to reduce natural protein intake and to cover protein needs by special phenylalanine free protein products. There is a big variety of formulas found available on the market for treatment of phenylketonuria. All of them are free of phenylalanine and very expensive.
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