The Management Of Urea Cycle Disorders Essay example

The Management Of Urea Cycle Disorders Essay example

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This article studies the management of urea cycle disorders (UCD) with modifications to protein intake through the diet. Urea cycle disorders result in ineffective metabolism of nitrogen, which can lead to serious consequences. Treating UCD with changes in diet is a newer, less explored way to treating these disorders, however they are being shown successful in reducing nitrogenous waste by moderating protein intake. Previously a lower protein diet has been used as a treatment for UCDs, however new prescription dietary treatments are being studied. Possible prescription dietary treatments include: natural protein intake, branched chain amino acid supplements, essential amino acid supplements, nutritional support, and vitamin/mineral/fatty acid supplements.
This study showed that protein prescription decreased as patient age increased because it is not as well tolerated in older patients, however infants were prescribed more protein than older children because they are growing so much. Although natural protein intake was preferred, it was often supplemented with the other supplementary protein options. In fact, it was reported that one advantage of replacing natural protein with the essential amino acids was that it provided high protein, but decreased the nitrogen burden on the urea cycle. The perfect dietary option for treatment of UCDs has not been found, but advances have been made in determining combinations of protein treatment that work the best for patients.

Part C—Reflection
Reading this article was extremely applicable to my future career as a pharmacist. Urea cycle disorders are not uncommon and can cause severe problems is left untreated. So, as a pharmacist, I learned about the possible options to treat thi...


... middle of paper ...


...ffect this enzyme has and how crucial it is for regulation in the body.
As a pharmacist, it will be essential for me to be aware of the effects of pyruvate dehydrogenase complex and what can result if deficiencies occur. We also learned how thiamine plays an important role in the enzyme’s function, so I will be in a great position to counsel patients on the importance of getting adequate thiamine in their diets as well as explaining the harmful effects of alcoholism on this enzyme’s function. Making patients aware of these things is extremely important because the defects in the pyruvate dehydrogenase complex can cause neurological damage and even death. By working with other healthcare professionals and educating patients about the effects of this condition, we will make patients aware and more likely to take precautionary measure to prevent this where possible.

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