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Urea Cycle Disorders

How is it treated?

Management aims to control ammonia concentrations and prevent episodes of decompensation, as these are potentially serious. General measures include a protein restricted diet, arginine supplementation (as this becomes an essential amino acid in these disorders with the exception of hyperargininaemia), and the use of alternate pathway medicines which help rid the body of excess nitrogen bypassing the urea cycle. During periods of infection, protein intake is stopped, and glucose is given either by mouth or intravenously.

View What are the causes? What are the causes?  |  Inheritance patterns and prenatal diagnosis View Inheritance patterns and prenatal diagnosis

Medical text written October 2001 by Dr M Champion. Last updated January 2006 by Dr M Champion, Consultant in Paediatric Metabolic Medicine, Evelina Children's Hospital, London, UK.

 

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