Process for preparing a phenylalanine-free dietary product in dr

Details Process for preparing a phenylalanine-free dietary product in dr Process for preparing a phenylalanine-free dietary product in dr

1993-06-25

1995-02-28

Page, Thurman K.

Drug, bio-affecting and body treating compositions

Preparations characterized by special physical form

Tablets, lozenges, or pills

424474, 424475, 424489, 514960, 426 72, 426 74, 426454, 426656, 426657, 427 214, 427 222, A61K 928, A61K 930, A61K 942, A61K 914

Patent

active

053935322

DESCRIPTION:

BRIEF SUMMARY
DESCRIPTION

The invention concerns a phenylalanine-free dietetic product based on amino acids, which is intended for persons, in particular children, adults and pregnant women who suffer from phenylketonuria, as well as to a process for preparing this phenylalanine-free dietetic product and a carrier to be used therewith.
Phenylketonuria belongs to genetically determined diseases representing disorders in which altered coding sequences of deoxyribonucleic acid determine the state of the disease. Because of genetic mutation, anomalous proteins are expressed. For example, the pathogenesis of enzymopathies (those diseases caused by reduced or absent enzyme activity) is based on the accumulation of the substrates left unmetabolized and their metabolites.
Presently eleven different mutations are known in the phenylalanine hydroxylase gene. When such mutation results in a complete lack of activity by such enzyme, then there will be a so-called phenylketonuria (hereafter PKU). If the enzyme effects only a reduced activity, then this condition leads to the so-called hyperphenylalanine anemias which must also be treated in part.
Because of the metabolic disorder, the phenylalanine accumulates in the body of the ailing person or patient and its concentration in the blood and tissue rises much above the normal range. As the concentration increases, the phenylalanine is then catabolized through metabolic bypasses ordinarily left unused by the body.
The increased level of phenylalanine in the plasma leads to a degradation of numerous metabolic mechanisms in the brain. If PKU is left untreated, children will incur brain immaturity with intellectual backwardness of varying degrees.
It was assumed until recently that by the onset of puberty, an increased level of phenylalanine no longer causes metabolic disorders in the brain. More recent investigations relating to young adults for whom treatment had been stopped during their school years, however, showed that there are be neurological disorders, such as measurable degradation of reaction times.
Phenylketonuria can be treated with food low in phenylalanine. As a result the patients develop normally physically and psychologically and can be educated. Moreover, they reach the age of reproduction in a problem-free manner and accordingly young female PKU patients are increasingly becoming pregnant.
In order to achieve normal intellectual and physical development of a PKU-afflicted child, the phenylalanine level in the plasma must be lowered by means of a low phenylalanine diet to normal values and must be stabilized there.
For that purpose such children receive diets with restricted amounts of protein and just as much phenylalanine as required by the child's body for protein build-up (growth). Accordingly, only such diets can be used which are inherently low in protein and hence also low in phenylalanine.
However, a diet low in phenylalanine by itself would deprive the child of sufficient amounts of other amino acids which are equally important for sustenance. Accordingly, children suffering from PKU require a protein source in addition to a phenylalanine-low diet, said source lacking phenylalanine but containing sufficient amounts of all other amino acids.
Special products already are known which are composed of protein constituents, particularly amino acids, but which do not contain phenylalanine. Vitamins, minerals and trace elements are additionally integrated into such mixtures because a PKU-afflicted child would not receive these nutrients in adequate amounts from the phenylalanine-low diet.
Presently, PKU patients are urged to sustain their phenylalanine-low diet throughout life, particularly in the case of pregnant PKU-women. Such groups of patients must observe the PKU diet as well as take the known special products.
It has been thought so far that protein metabolism requires the simultaneous presence of all, and hence also of the non-essential, amino acids. Therefore the known, conventionally used special products or phenylalanine-free mixtures of amino acids for i

REFERENCES:
patent: 3764703 (1973-10-01), Bergstrom et al.
Chemical Abstracts, 98:142181g, Lieske et al., 1983.

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