نوع المستند : المقالة الأصلية
المؤلفون
1
Food science and technology, National Research Center, Cairo, Egypt
2
Nutrition and Food Science Dept., Faculty of Home Economics, Helwan University, Cairo, Egypt
المستخلص
Phenylketonuria (PKU) is an inherited disorder that caused an amino acid phenylalanine to build up in the body. It is caused by the deficiency in the phenylalanine hydroxylase (PAH) causing a severe neurological damage when left untreated. The present study was conducted to create some of the newer high nutritional value food products and assess their effect on children with PKU disorder. In one week randomized, crossover study, two product of functional black raisin juice, functional black raisin cupcake were compared in 60 children from 4 to 6 years old, 40 children who were diagnosed as PKU children, and 20 healthy were divided into 6 groups. Children were taken Cupcakes and juice separately, and cupcakes and juice together from 3 to 7 days. All groups had Blood samples collection 3 times (fasting pre eating products, twice weekly at the 3 day and 7 days of eating) for laboratory analysis. The test that identified The total phenolic, flavonoid, carotenoid, mineral content {Iron (Fe), Zinc (Zn), Magnesium (Mg)} , the total amino acid and sensory evaluation of low phenylalanine (PHE) functional cupcakes with whey low protein ,inulin, canola oil ,black raisins ,pumpkin fruits and low phenylalanine functional juice with (whey low protein, inulin, black raisins , pumpkin fruits, and Lactobacillus acidophilus). Anthropometric measurements including (Body weight and Body mass index) and laboratory data (Serum PHE and tyrosine) in PKU children who get juice and cupcake only separately, juice and cupcake together groups, Normal children who get juice and cupcake only separately, PKU Children how not received any products cupcake or juice. Functional cupcake and juice with black raisins had highest values of Total Phenolic, Flavonoid and the functional cupcake and juice with pumpkin fruits had highest value of carotenoid. The mineral content (Fe), (Zn), (Mg) of functional cupcake and juice had highest values than control. The sensory evaluation of functional juice and cupcake show that the functional product that made with black raisin had highest score than control and pumpkin so the experimental design in PKU children made with black raisin only. In these study the (PHE) range show in the amino acid analysis in the treated cupcake and beverages are acceptable according to recommendation. The anthropometric result the body mass index ,weight of children how eat functional cupcakes and juice together had highest values .The Children who eat functional cupcakes and juice together showed the lowest level of serum phenylalanine and tyrosine level had highest in children how eat cupcake and juice together. This study conclude that the ingredient of cupcake and juice appeared to contribute to lower blood phenylalanine level in PKU children.
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