Anthropometric Assessment and some of Contributing Factors in Control of Phenylketonuria Patients in Khuzestan Province

Untreated phenyl ketonuria (PKU) results in high serum phenylalanine and severe irreversible mental and motor deterioration. The aim of this study was to investigate the anthropometric incices and some of contributing factors in disease control in patients suffering from PKU <18yr old in Khuzestan province. Subjects and This study was conducted in 2012 on 56 PKU patients aged 2 months-18yr (34 males, 22 females) in Abuzar children’s hospital, the only center for referral and management of PKU in Khuzestan province. Weight and height were measured using standard methods and were compared with the WHO standards. Nutritional indices, including weight for age (W/A), height for age (H/A), and body mass index for age (BMI/A) were determined using WHO Anthro-software. Based on advised serum phenylalanine levels, patients were divided to two controlled and uncontrolled groups. W/A and H/A indices were lower than -2SD in 17.85% and 15.09% of subjects respectively. According to height index two patients had severe malnutrition (<-3SD); both of them were girls. Nine percent of patients (4 males, 1 female) had BMI< -2SD. Regarding W/A, H/A, BMI/A there were no difference between the controlled and uncontrolled groups. Anthropometric data in PKU patients showed an inappropriate condition of growth without correlation to disease control. Providing specific semi-synthetic foods (limited by cost) and sufficient supplementation can improve these indices.