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July 1984

Detection of Urea Cycle Enzymopathies in Childhood

Author Affiliations

From the Departments of Neurosciences (Dr Trauner) and Pediatrics (Drs Trauner and Self), University of California Medical Center, and the Department of Pediatrics, Kaiser Permanente Medical Foundation Hospital (Dr Self), San Diego.

Arch Neurol. 1984;41(7):758-760. doi:10.1001/archneur.1984.04050180080023

• Inborn errors of ureagenesis must be considered in the differential diagnosis of recurrent vomiting and lethargy in childhood. Elevations of liver enzyme levels are often present during these episodes and may lead to an erroneous diagnosis of hepatic encephalopathy. We studied two cases of urea cycle defects.