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Q. What is metabolic disorders?
In these metabolic disorders, since there is an inability to metabolize a specific amino acid the basis of dietary management is to restrict the offending amino acid(s) by means of a special low protein diet. In addition, to meet the requirement for growth and development it is essential to supplement the diet with the appropriate amino acid mixture, which does not contain the offending amino acid.
Low-protein food products are recommended to persons with particular types of metabolic disorders such as homocystinuria (HCU), phenylketonuria (PKU), tyrosinemia etc. Each of these disorders requires food products, which are low, in particular type of amino acid i.e. methonine in the case of HCU, phenylalanine in the case of PKU. However, it is not easy to find high protein foods with low content of these amino acids. Hence, the patients are given low-protein foods as you would realize while studying these disorders.
Nursing Care and Treatment The only treatment is surgical repair and child may be operated before 18 months as per the choice of surgeon so that speech pattern of child may
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