THE ASSOCIATION BETWEEN DIETARY COMPLIANCE STATUS IN PHENYLKETONURIC CHILDRENS AND THE NUMBER OF CLINICAL CARE SESSIONS

Background and Aim: Phenylketonuria (PKU) is caused by deficient activity of the enzyme phenylalanine hydroxylase (PAH), which is needed to convert the essential AA phenylalanine (phe) to tyrosine. In order to prevent neurological damage, lifelong adherence to a low-phe diet is required. In peridic care programs, the patients parents referred to elected hospital for evaluating by clinical team and advised in terms of dietary compliance by nutrition expert. The objective of present study was to investigate the association between dietary compliance status in phenylketonuric childrens with the number of clinical care sessions. Methods: In this analytical cross-sectional study, 11phenylketonuric patients (6 female, 5male) with mean age of 12.72±7.42 years, were selected by census method and investigated in terms of dietary compliance in a 9-month period. 3-day recall questionnaire were also completed in First week of each month. The data were entered in SPSS software version 16.0 and were analyzed by correlation tests. Significant was set at P value < 0.05.Results: 68% of patients(7) were atleast deficient in one of macronutrient groups and energy intake. 85.71% of patients with intake deficiency had irregular referrals to elected hospital (1-2 referrals in a 9-month period). A negative significant correlation was observed between the number of clinical care sessions and intake deficiency (p <0.02, r= 0.82).Conclusion: It seems that nutritional counseling sessions have major role in informing parents to patient s dietary compliance and essential cares.

کلیدواژه ها:

phenylketonuria ، nutritional intake ، deficiency