ارزیابی وضعیت تن سنجی و عوامل مرتبط با کنترل بیماری در مبتلایان به فنیل کتونوری در استان خوزستان

Background and objective: untreated phenyl ketonuria (pku)results in high serum phenylalanine and severe irreversible mentaland motor deterioration. the aim of this study was to investigatethe anthropometric incices and some of contributing factors indisease control in patients suffering from pku < 18yr old inkhuzestan province.subjects and methods: this study was conducted in 2012 on 56pku patients aged 2 months-18yr (34 males, 22 females) inabuzar children’s hospital, the only center for referral andmanagement of pku in khuzestan province. weight and heightwere measured using standard methods and were compared withthe 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 advisedserum phenylalanine levels, patients were divided to two controlledand uncontrolled groups.results: w/a and h/a indices were lower than -2sd in 17.85%and 15.09% of subjects respectively. according to height indextwo patients had severe malnutrition ( < -3sd); both of them weregirls. nine percent of patients (4 males, 1 female) had bmi < -2sd.regarding w/a, h/a, bmi/a there were no difference between thecontrolled and uncontrolled groups.conclusions: anthropometric data in pku patients showed aninappropriate condition of growth without correlation to diseasecontrol. providing specific semi-synthetic foods (limited by cost)and sufficient supplementation can improve these indices.