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The thesis deals with the diet of children with phenylketonuria. Phenylketonuria, its diagnostics and incidence are described in the theoretical part, it also further discusses the treatment of the disease, dietary measures as part of low-protein diet and recommended intakes of macronutrients. In last part, it focuses on the support of the individuals with phenylketonuria in the Czech Republic. The objective of the thesis was to map the diet of children with phenylketonuria and compare financial burden of low-protein diet with diet without restrictions. 10 children with phenylketonuria took part in the research. The collection of data was realized by the qualitative method using the semi-structured interview which was then processed into case studies. The meal plans sent by parents were evaluated in the Nutriservis Professional and PKU nutrition table, subsequently evaluated and similar meal plans without restrictions, containing common foods were created. In conclusion, both meal plans were compared. The analysis of the meal plans shows, that almost half of the respondents do not meet their daily energy needs. Carbohydrates are sufficiently present in the diet of children with phenylketonuria, on the contrary half of the respondents had a low fat intake. Proteins are consumed in sufficient amount, in some cases excessively. The meal plan was often monotonous. The reason could be high prices of special foods and rejection of the new ones. Low-protein products make up almost 60 % of all expenses for this diet. In summary, the diet for children with phenylketonuria is about 80 % more expensive than ordinary diet without restrictions. For half of the respondents, the health insurance contribution and the allowance for caregivers will cover all costs. For the second half, the expenses are covered only partially this way. The thesis can help increase public knowledge and awareness about the disease and its economic burden.

Low-protein diet is one of the methods of conservative therapies or medical treatments of chronic kidney disease in predialysis. The goal of the diet is to compensate renal dysfunction together with coverage of nutritional requirement of the patient. The diet is based on reduction or food substitution of nutrients which giving rise to metabolic complications, not on reduction of food amount in general. Dietary measures focus mainly on protein reduction to 0,6-0,8 g/kg/day. Correctly prescribed nutrition should not lead to malnutririon. More further, the diet therapy is focused on reduction of sodium, potassium and phosphorus intake. Calcium intake depends on actual calcaemia of the patient. Amount of fluid intake is prescripted by a doctor, based on diuresis. The aim of nutritional therapy is to keep optimal nutritional status of the patient and minimize complications associated to chronic kidney disease. The task of the dietitan is to educate the patient in a field of prescripted diet principles and potential risks related to protein restriction. Regular physical activity should be a part of conservative therapy in case of predialysis. Regular physical activity helps to keep muscle strength, muscle mass and physical condition of the patient. The main aim of the fieldwork was to discover aspects of...

Background: Low protein diet is one of the treatments for patients with chronic kidney disease during the pre-dialysis period. Studies about this subject have been published for more than 20 years, but the conclusions about its effect and the most appropriate composition are not clear. A low-protein diet is recommended for some patients at the Nephrology Clinic of General University Hospital. These patients are educated and re- educated by a nutrition therapist who also controls their food intake through a food diary. Objectives: To evaluate the effect of low-protein diet on the progression of chronic kidney disease in our conditions and to compare the results with the control group. Furthermore, to evaluate the nutritional status of these patients and the influence of the diet on it. Last but not least, describe a set of patients coming to education about low-protein diet. Methods: The input data was retrospectively retrieved from the medical records of patients who attended medical checks at the Nephrology Clinic of General University Hospital between 10/2016 and 3/2018. A group of 15 patients with a low-protein diet was compared with a control group of 15 patients who did not receive low-protein diet education. The main data collected was year of birth, estimated GFR, urea, creatinine, albumin,...