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Pharmacologic amino acid acylation in the acute hyperammonemia of propionic acidemia

 

作者: PetrowskiSuzanne,   NyhanWilliam L.,   ReznikVivian,   SweetmanLawrence,   KulovichStanko,   WolffJon,   JonesGilbert,  

 

期刊: Journal of Neurogenetics  (Taylor Available online 1987)
卷期: Volume 4, issue 1  

页码: 87-96

 

ISSN:0167-7063

 

年代: 1987

 

DOI:10.3109/01677068709102336

 

出版商: Taylor&Francis

 

关键词: Propionic acidemia;Hyperammonemic coma;Hemodialysis;Phenyl acetate;Pharmacologic acylation

 

数据来源: Taylor

 

摘要:

An infant with propionic acidemia presented at one month of age with hyperammonemic coma. Treatment by two double-volume exchange transfusions did not have an appreciable effect, but hemodialysis led to a substantial reduction of the serum concentration of ammonia on two occasions. Nevertheless, continued therapy with sodium benzoate, arginine-HCl, carnitine and lactulose did not have any observable effect on the blood concentration of ammonia. Treatment with sodium phenylacetate was followed by a reduction in serum concentrations of ammonia to normal levels which were maintained. These observations demonstrate the dramatic reduction in serum concentrations of ammonia that may be obtained in patients with organic acidemia by hemodialysis. They suggest that pharmacologic acylation therapy with phenylacetate may be of lasting benefit in the management of this complication.

 

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