Liver Ultrastructure in Mitochondrial Urea Cycle Enzyme Deficiencies and Comparison with Reye's Syndrome
Patricia S. Latham, Douglas R. Labrecque, John W. Mcreynolds, Gerald Klatskin – 1 May 1984 – The liver ultrastructural findings in two girls with partial carbamyl phosphate synthetase I (CPS) deficiency and their heterozygote parents and two siblings with ornithine transcarbamylase (OTC) deficiency are described. Liver ultrastructure in the four patients with inherited deficiences of urea cycle enzymes showed minimal alterations with essentially normal mitochondria when biopsy was performed during periods of good control of their hyperammonemia.