Abnormalities of Ammonia Metabolism

Abstract

The body metabolizes ammonia in the Krebs-Henseleit urea cycle. In hyperammonemia, either congenital or secondary to cirrhosis, this cycle does not function efficiently. Children with cycle defects have abnormal ammonia tolerance tests, amino-aciduria and atypical electroencephalograms. Amino acid chromatography of urine will detect congenital hyperammonemia.