Transient idiopathic hyperammonaemia in adults.
Publication/Presentation Date
12-7-1985
Abstract
Transient severe hyperammonaemia developed in the absence of serious liver dysfunction in three patients being treated for acute leukaemia. The onset of the biochemical disturbance was abrupt and led rapidly to acute encephalopathy, fatal in two cases. In the third patient, prompt initiation of aggressive haemodialysis and intravenous sodium benzoate and sodium phenylacetate infusion successfully controlled plasma ammonium levels until they spontaneously resolved. The cause of the disorder remains to be determined, but urinary nitrogen partition studies suggest temporary impairment of ureagenesis in a catabolic setting as a major pathophysiological feature of this disorder. The absence of liver disease, the normal mitochondrial ultrastructure seen in two cases, and the plasma aminoacid profiles observed serve to distinguish this disorder from others such as Reye's syndrome.
Volume
2
Issue
8467
First Page
1271
Last Page
1274
ISSN
0140-6736
Published In/Presented At
Watson, A. J., Chambers, T., Karp, J. E., Risch, V. R., Walker, W. G., & Brusilow, S. W. (1985). Transient idiopathic hyperammonaemia in adults. Lancet (London, England), 2(8467), 1271–1274. https://doi.org/10.1016/s0140-6736(85)91554-5
Disciplines
Medicine and Health Sciences | Oncology
PubMedID
2866337
Department(s)
Department of Radiation Oncology
Document Type
Article