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 mcchanism 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;

Phlebotomy Essentials
6th Edition
ISBN:9781451194524
Author:Ruth McCall, Cathee M. Tankersley MT(ASCP)
Publisher:Ruth McCall, Cathee M. Tankersley MT(ASCP)
Chapter1: Phlebotomy: Past And Present And The Healthcare Setting
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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 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 effect of amino acids keto-analogs? For the answer:
a) write down the scheme of ammonia detoxification in the liver;
b) explain the causc of argininosuccinic aciduria;
c) make up the scheme of present amino acid keto-analogs usage for the
reduction of hyperammoniemia.
Transcribed Image Text: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 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 effect of amino acids keto-analogs? For the answer: a) write down the scheme of ammonia detoxification in the liver; b) explain the causc of argininosuccinic aciduria; c) make up the scheme of present amino acid keto-analogs usage for the reduction of hyperammoniemia.
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