Family Social Status and Dietary Adherence of Patients with Phenylketonuria|
Alaei, Mohammadreza; Asadzadeh-Totonchi, Gelareh; Gachkar, Latif & Farivar, Shirin
Objective: 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.
Methods: 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
Findings: 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.
Conclusion: Social status affects dietary adherence to some extent. We suggest exploring caregivers
dietary knowledge as the next step to improve dietary compliance in these patients.
Phenylketonuria; Mental Retardation; Marital status; Hyperphenylalaninaemia; PKU