Dr Hawley's comments bring to the forefront a potentially larger issue that we were not at liberty to share with the reader in our previous reports.1- 3 This issue concerns the unknown prevalence of hyperammonemic coma in the intensive care unit setting. Because of our familiarity with congenital urea cycle enzyme defects (UCEDs), we were able to recognize the complication of hyperammonemia in adults following solid organ transplantation.1- 3 At the Hospital of the University of Pennsylvania and the Children's Hospital of Philadelphia, 6 of 145 patients who underwent orthotopic lung transplantation developed hyperammonemia and only 1 survived.4 Most importantly, we 3 and others5 now realize that this type of patient can be treated successfully with the same modalities and type of medicines used for the congenital UCED.
Berry GT, Kaplan PB, Lichtenstein GR. Hyperammonia Possibly due to Corticosteroids—Reply. Arch Neurol. 2000;57(7):1085-1086. doi: