3. A 2-year-old child was taken to the hospital. His mother said that he vomited frequently, cspecially after feedings. The child's weight and physical development were below normal. His hair, although dark, containcd patches of white. A urine sample treated with ferric chloride (FeCl,) gave a green color characteristic of the presence of phenylpyruvate. Quantitative analysis of urine samples revealcd the presence of phenylalanine 7.0 uMol/L (0.01 in health), phenylpyruvate 8.7. Decarboxylation of Some Amino Acids, Synthesis of Neurotransmitters and Hormones 457 4.8 uMol/L (0 in health) and phenyllactate (0 in health). Suggest, which enzyme might be deficient in this child? Why does the abnormal pathway come into play under these conditions? b) write down the reaction of damaged pathway; explain the appearance of phenylalanine in the urine in the large amounts; c) write down the scheme of an alternative metabolic pathway of the substrate;
3. A 2-year-old child was taken to the hospital. His mother said that he vomited frequently, cspecially after feedings. The child's weight and physical development were below normal. His hair, although dark, containcd patches of white. A urine sample treated with ferric chloride (FeCl,) gave a green color characteristic of the presence of phenylpyruvate. Quantitative analysis of urine samples revealcd the presence of phenylalanine 7.0 uMol/L (0.01 in health), phenylpyruvate 8.7. Decarboxylation of Some Amino Acids, Synthesis of Neurotransmitters and Hormones 457 4.8 uMol/L (0 in health) and phenyllactate (0 in health). Suggest, which enzyme might be deficient in this child? Why does the abnormal pathway come into play under these conditions? b) write down the reaction of damaged pathway; explain the appearance of phenylalanine in the urine in the large amounts; c) write down the scheme of an alternative metabolic pathway of the substrate;
Chapter16: Specialized Nutrition Support: Enteral And Parenteral Nutrition
Section: Chapter Questions
Problem 8SC
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Transcribed Image Text:3. A 2-year-old child was taken to the hospital. His mother said that he vomited
frequently, especially after feedings. The child's weight and physical development
were below normal. His hair, although dark, containcd patches of white. A urine
sample treated with ferric chloride (FeCl,) gave a green color characteristic of
the presence of phenylpyruvate. Quantitative analysis of urine samples revealed
the presence of phenylalanine 7.0 uMol/L (0.01 in health), phenylpyruvate
8.7. Decarboxylation of Some Amino Acids, Synthesis of Neurotransmitters and Hormones
457
4.8 uMol/L (0 in health) and phenyllactate (0 in health). Suggest, which enzyme
might be deficient in this child? Why does the abnormal pathway come into play
under these conditions?
b) write down the reaction of damaged pathway; explain the appearance of
phenylalanine in the urine in the large amounts;
c) write down the scheme of an alternative metabolic pathway of the substrate;
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