Urea Cycle Disorders

What is Urea Cycle Disorder?

Urea Cycle Disorders (UCD) are a group of inherited conditions, characterised by enzyme deficiencies of the urea cycle. The result is waste nitrogen accumulating as ammonia and glutamine, both of which are neurotoxic and may cause severe encephalopathy. The aim of dietary management is to restrict dietary protein by means of a low protein diet. The protein requirements are met by supplementing the diet with an essential amino acid mixture if necessary, and / or specific individual amino acids such as arginine and citrulline depending upon the type of Urea Cycle Defect. A high calorie diet is also an important aspect of dietary management
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Sodium benzoate and phenylbutyrate are used in conjunction with diet therapy to facilitate the removal of nitrogen.

Emergency regimes are required in periods of decompensation and catabolism.

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