The effect of family nutritional knowledge and diet treatment compliance on the growth, development and nutritional status of phenyketonuria patients aged 6-10 years old

The Effect of Family Nutritional Knowledge and Diet Treatment Compliance on the Growth, Development and Nutritional Status of Phenyketonuria Patients Aged 6-10 Years Old. Hasan Kalyoncu University Graduate Education Institute, Department of Nutrition and Dietetics, Graduate Thesis, Gaziantep, 2021. Phenylketonuria (PKU) is a congenital protein metabolism disorder caused by a deficiency of the enzyme phenylalanine hydroxylase (PAH). In order to prevent irreversible neurological damage in PKU patients, dietary therapy should be lifelong. Families' knowledge and attitudes towards dieting affect blood phenylalanine levels, nutritional status of patients and their growth. This study was planned to evaluate the effects of family nutritional knowledge and adherence to diet therapy on average blood phenylalanine levels, nutritional status and growth-development in patients with Classical PKU aged 6-10 years. The mean age of the patients was 8,67±1,41 years. Vitamin D, magnesium, phosphorus, iron, total protein and albumin values of the patients were within the reference range, while vitamin B12 and calcium were slightly above the reference range. The blood phenylalanine levels of the patients were high with an average of 538,0±386,9 mmol/L. The percentage of meeting the daily phenylalanine requirement of the patients was 86,9±54%. The difference between the education level of the mother and the blood phenylalanine value was found to be statistically significant (p<0,05). The blood phenylalanine values of non-working fathers were higher than those of working fathers children (p<0,05). While the correlation between the blood phenylalanine level classification and intelligence scores of the patients was not found statistically significant (p>0,05); those with high blood phenylalanine levels had an increased rate of borderline intelligence and dull intelligence. While the correlation between the blood phenylalanine level classification of the patients and the BMI Z-score was statistically significant (p<0,05), the prevalence of obesity was higher in patients with good or bad blood phenylalanine levels (p<0,05). The relationship between their families' nutritional knowledge and their intelligence scores was statistically significant (p<0,05), and the frequency of dull intelligence was higher in patients with poor nutritional knowledge (p<0,05). A statistically significant difference was found between their families' nutritional knowledge and BMI Z-score (p<0,05). The prevalence of overweight in patients with poor nutritional knowledge is higher than in patients with moderate nutritional knowledge (p<0,05). Patients should be given detailed education about treatment and diet, they should be informed about how the disease is and how to manage this disease, and education should be continuous according to changing conditions. Keywords: Phenylketonuria, Level of Knowledge, Growth and Development, Diet Therapy.