Case Study
GF is a 10-month-old infant with a urea cycle disorder.GF is admitted into the hospital due to a hyperammonemic crisis.
LT: 25.5 inches WT: 16.5 #
-After the first 24-48 hours, the registered dietitian is going to prescribe PN He/she should work with the pharmacy to ensure that the PN solution is high in:

Question

Case Study
GF is a 10-month-old infant with a urea cycle disorder.GF is admitted into the hospital due to a hyperammonemic crisis.
LT: 25.5 inches     WT: 16.5 #
-After the first 24-48 hours, the registered dietitian is going to prescribe PN He/she should work with the pharmacy to ensure that the PN solution is high in:

Quizplus · Accepted Answer

Infants with urea cycle disorders have a deficiency in one or more of the enzymes required for the synthesis of urea from ammonia, resulting in buildup of ammonia in the blood (hyperammonemic crisis). One of the main goals of therapy is to limit protein intake to reduce ammonia production. However, protein is still needed for growth and development, so the use of PN is often necessary to meet protein and energy needs while limiting ammonia production. The PN solution should be high in branched-chain amino acids (BCAAs), as these amino acids can be catabolized in skeletal muscle, reducing the amount of ammonia produced from protein breakdown. Aromatic amino acids (AAs) and tyrosine are not beneficial in this situation, as they can actually increase ammonia production. Omega-3 fatty acids are not necessary for the management of a hyperammonemic crisis in a patient with a urea cycle disorder. Branched-chain amino acids (leucine, isoleucine, and valine) are important for protein synthesis and can help decrease ammonia levels in patients with urea cycle disorders. They also have a sparing effect on aromatic amino acids and can improve protein metabolism. Therefore, the PN solution should be high in branched-chain amino acids. Aromatic amino acids, such as phenylalanine and tyrosine, are typically restricted in the diet of patients with urea cycle disorders because they are converted to tyrosine, which can further increase ammonia production. Omega-3 fatty acids, while beneficial for other health conditions, do not have a specific role in the management of hyperammonemia in patients with urea cycle disorders.

Case Study GF Is a 10-Month-Old Infant with a Urea Cycle Disorder.GF