Majallah-i Dānishgāh-i ̒Ulūm-i Pizishkī-i Bābul (Sep 2011)
Evaluation of Nutritional Status and Growth in Phenylketonuria Patients in Mazandaran Province Iran
Abstract
BACKGROUND AND OBJECTIVE: Phenylketonuria (PKU) is one of the most frequent metabolic diseases which is transmitted as autosomal recessive pattern due to phenylalanine hydroxylase deficiency. Appropriate diet with restricted phenylalanine is the base of treatment. According to some difficulties in our country for these patients and limited native studies exist about the results of such treatment means protein limitation and replacing an expensive and unavailable foods, this study was designed to evaluate nutritional status with calculating the calorie, protein, carbohydrate and fat of patients’ diet and iron storage and iron deficiency anemia in PKU patients.METHODS: This is a cross sectional study which evaluated all of the PKU patients in Mazandaran province during 2009-2010 in metabolic clinics in Babol and Sari, Iran. Nutritional status was evaluated according to 72 hours diet recall sheet which the patient or his/her parent recorded all of the nutrients that was eaten within 3 days then nutritional contents: carbohydrate, protein, fat and calories were evaluated by nutritionist. Demographic information was studied according to questionnaire and iron deficiency and iron deficiency anemia was studied according to blood sample and measurement of hemoglobin, hematocrit MCV, MCH, MCHC, Ferritin and response to treatment with ferrous sulfate.FINDINGS: Twenty one PKU patients were studied which 7 ones (33.3%) were female and 14 ones (66.7%) were male with mean age of 7.26±6.64 years. Iron deficiency and iron deficiency anemia was present in respectively in 10 (47.1%) and 6 patients (28.6%). Deficiency of energy, protein, carbohydrate and fat was seen in 9 patients (42.86%), 14 patients (66.67%), 9 patients (42.86%) and 6 patients (28.57%), respectively. 23.8% of patients were underweight, 23.8% of patients were microcephal and 4 patients (19%) were short stature.CONCLUSION: The results of this study showed that the nutritional status and growth of patients was not good, so attention to nutritional demands in PKU patients to reduce malnutrition and iron deficiency is essential.
