7. A 22-year-old patient suffering argininosuccinic aciduria from little was prescribed the treatment by administration of keto-analogs of valine, leucine, isoleucine and phenylalanine against the background of reduced protein in the diet during two wceks. The doctor noted the decrease ofammonia concentration in plasma from 0.09 mg/dL to 0.04 mg/dL as a result, and argininc succinate excretion was reduced from 2 to 0.8 g/24h. What is the molecular mechanism of the curative effeet of amino acids keto-analogs? For the answer: a) write down the scheme of ammonia detoxification in the liver; b) explain the cause of argininosuccinic aciduria; c) make up the scheme of present amino acid keto-analogs usage for the reduction of hyperammoniemia.
7. A 22-year-old patient suffering argininosuccinic aciduria from little was prescribed the treatment by administration of keto-analogs of valine, leucine, isoleucine and phenylalanine against the background of reduced protein in the diet during two wceks. The doctor noted the decrease ofammonia concentration in plasma from 0.09 mg/dL to 0.04 mg/dL as a result, and argininc succinate excretion was reduced from 2 to 0.8 g/24h. What is the molecular mechanism of the curative effeet of amino acids keto-analogs? For the answer: a) write down the scheme of ammonia detoxification in the liver; b) explain the cause of argininosuccinic aciduria; c) make up the scheme of present amino acid keto-analogs usage for the reduction of hyperammoniemia.
Cardiopulmonary Anatomy & Physiology
7th Edition
ISBN:9781337794909
Author:Des Jardins, Terry.
Publisher:Des Jardins, Terry.
Chapter7: Acid–base Balance And Regulation
Section: Chapter Questions
Problem 11RQ
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Transcribed Image Text:7. A 22-ycar-old patient suffering argininosuccinic aciduria from little was
prescribed the treatment by administration of keto-analogs of valine, leucine,
isoleucine and phenylalanine against the background of reduced protein in the
diet during two weeks. The doctor noted the decrease of ammonia concentration
in plasma from 0.09 mg/dL to 0.04 mg/dL as a result, and arginine succinate
excretion was reduced from 2 to 0.8 g/24h. What is the molecular mechanism of
the curative effcet of amino acids keto-analogs? For the answer:
a) write down the scheme of ammonia detoxification in the liver;
b) explain the cause of argininosuccinic aciduria;
c) make up the scheme of present amino acid keto-analogs usage for the
reduction of hyperammoniemia.
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