Pku Children: A Literature Review

Phenylketonuria is defined as an inborn error where the body is unable to convert phenlyanine into tyrosine in the body. The myth is that people who phenylketonuria should follow their diet until the age five. After age five, children should avoid eating high-protein foods, which increases high Phe in the bloodstream. Although, doctors advised children to stay on a low-Phe diet that requires adequate consumption of energy, protein, and nutrients. If untreated in children, this can cause seizure, disability, and behavioral problems. Therefore, studies say PKU children require dietary intervention to maintain physical and mental health. Evidenced based articles suggest that doctors and dietitian to monitor micronutrients and micronutrients among
Dietary compliance is easier to provide treatment in infancy and childhood than adolescent and adults (Giovannini et al, 2012). However, the evaluation of blood Phe levels plays an influence that affects neurological issues in children. Pku children have lower IQ, which is associated with low academic performance. The nutritional supplement called long chain polyunsaturated fatty acids (LCUFA). It contains more protein to maintain Phe blood levels. LCUFA supplement with Phe infant formula can help infants improve DHA and EPA for brain development. (Giovannini et al, 2012). Several studies have found the amount of protein substitute is available for PKU patients to try it out. Glycomacropeptide, a protein source can help improve PKU patients to ensure it’s Phe-free from other sources. A recent study finds that 11 PKU patient preferred the GMP diet. Results show that plasma levels decrease in the GMP diet than amino acid Interestingly, the authors believed that taking GMP supplement is safe to take and maintain protein retention (Giovannini et al, 2012).Also, another supplement sapropterin can control blood Phe levels only, but preferably combined with dietary treatment. Care is needed to provide a Phe restricted diet as they may experience nutrition deficiency, which must be taken seriously. In addition, the new protocol for managing PKU diet and saproptretin is required in Phe blood levels (Giovannini et al,