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Evaluation of food and diet in disease phenylketonuria
Čermáková, Venuše ; Faměra, Oldřich (advisor) ; Renata, Renata (referee)
This diploma thesis pursues the hypothesis, that strict abiding or failures in low protein diet are deciding about the health of patients with phenylketonuria. The goal of the thesis was to evaluate the composition of the diet for selected set of patients from the dietological and nutritious point of view in dependence on individual health condition, to compare the values with the diet of healthy people and to evaluate the availability of special healthcare and nutrition for phenylketonuria patients. The summary of literature gives informations about phenylketonuria, causes of its origin, metabolism of phenylalanin, diagnostics, possibilities of treatment, risks of repercussion of not treating, impact of nutrition composition of patients' health and dietological recommendations. For assessment of the bilance of nutrients and energy in the diet of phenylketonuria patients a group of 10 people was selected with different age, sex and dietary level, 5 people with phenylketonuria a 5 healthy persons. For each person a detailed weekly diet was setup and nutritious intake was evaluated, observed was daily intake of Phe, protein, fat, carbohydrate and energy. For persons with phenylketonuria the nutritious was evaluated in two steps, with and without usage of medical agent. The values of the patients were compared with healthy persons. From the results a necessity of using medical amino acid agents was derived, in other case there is a risk of serious deficit of protein intake, for some also of fat and energy. A complicated situation arises for women with phenylketonuria, who plan pregnancy. The research implies necessity of strict adherence to low protein diet before conception and frequent monitoring of Phe blood values during pregnancy. The diet must be compiled cautiosly with considering the individual state of patiens, a thorough education from childhood to adulthood is recommended. The situation would be simplified with a better approach of the state in the area of compensation the costs for foods, but also additional support for phenylketonuria patiens, to improve especially the availability of special foods for low protein diet and treatment possibility.

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