Family social status and dietary adherence of patients with phenylketonuria

There are several problems associated to the management of patients with phenylketonuria [PKU]. Social status could be one of the affecting factors on dietary adherence in these patients. The aim of this study was to evaluate family social status and dietary adherence of PKU patients in Iranian population. In a cross-sectional study, we studied 105 Iranian PKU patients [born 1984 to 2010], treated and followed at Mofid Children's Hospital, Tehran. Social status was defined by number of children in family, number of affected children in family, maternal and paternal education, marital and employment status of the parents. Age at diagnosis and duration of treatment were also recorded. Mean plasma phenylalanine level was considered as a sign of dietary adherence in PKU patients and was calculated considering the phenylalanine measurements throughout at least one year. Mean plasma phenylalanine concentration was 5.9 +/- 3.6 mg/dl in patients <12 years old and 13.1 +/- 3.9 mg/dl in patients >12 years old. Blood phenylalanine concentrations in 47.6% of patients were in normal age-related reference range. There was a significant association between divorced and unemployed parents, and higher levels of blood phenylalanine concentration [P=0.02 and P=0.03 respectively]. There was a significant positive correlation between number of affected children in the family [r=0.43, P<0.001], age at diagnosis [r=0.2, P=0.03], treatment duration [r=0.7, P=<0.001] and blood phenylalanine concentrations. There was no significant relation between parental education, family size and dietary adherence. Social status affects dietary adherence to some extent. We suggest exploring care-givers dietary knowledge as the next step to improve dietary compliance in these patients

effect of the ketogenic diet on the growth and biochemical parameters of the children with resistant epilepsy

The aim of this study was to evaluate the effect of the ketogenic diet on the growth parameters of the children with resistant epilepsy. A total of 36 children with resistant epilepsy who were 2 to 7 year old were put on the ketogenic diet. Their growth and biochemical parameters were studied at the beginning of the study and after 3 months. Weight decreased in all patients. Serum levels of hemoglobin, calcium, and blood sugar decreased significantly but remained in the normal range. Creatinine did not change, but BUN showed a significant increase. We can lower the complications of ketogenic diets by using more unsaturated fat, more water, and more minerals