Noncirrhotic hyperammonemia after deceased donor kidney transplantation: A case report

• Published in: American journal of transplantation Vol. 19; no. 11; pp. 3197 - 3201
• Main Authors: Li, George Z., Tio, Maria C., Pak, Linda M., Krier, Joel, Seifter, Julian L., Tullius, Stefan G., Riella, Leonardo V., Malek, Sayeed K., Stergachis, Andrew B.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Limited 01.11.2019
• Subjects: Aged; Ammonia; Antibiotics; Case reports; clinical research/practice; Death; Dialysis; encephalopathy; Female; genetics; Humans; Hyperammonemia; Hyperammonemia - diagnosis; Hyperammonemia - drug therapy; Hyperammonemia - etiology; infection and infectious agents ‐ bacterial; infectious disease; kidney disease; Kidney diseases; Kidney Failure, Chronic - pathology; Kidney Failure, Chronic - surgery; Kidney transplantation; Kidney Transplantation - adverse effects; kidney transplantation/nephrology; Kidney transplants; metabolism/metabolite; Morbidity; Patients; Prognosis; Renal function; Tissue Donors - supply & distribution; Transplantation, Homologous; Urea; Urinary tract; Urinary tract infections; infection and infectious agents - bacterial; genetics; infectious disease; metabolism/metabolite; clinical research/practice; kidney transplantation/nephrology; kidney disease; encephalopathy
• ISSN: 1600-6135; 1600-6143
• DOI: 10.1111/ajt.15545

A 72‐year‐old woman with end‐stage kidney disease due to recurrent urinary tract infections and obstructive uropathy of a solitary kidney presented to our hospital for renal transplantation. She underwent successful transplantation of a deceased donor allograft, but developed acute mental status deterioration on the fifth postoperative day. Her serum ammonia was found to be markedly elevated to 447 μmol/L in the setting of normal hepatic function. She was treated with emergent dialysis and empiric antibiotics targeting urea‐splitting organisms, and ultimately made a full neurologic recovery with stable renal allograft function. Noncirrhotic hyperammonemia (NCH) is an exceedingly rare clinical entity but seems to have a predilection for patients who have undergone solid organ transplantation. This report emphasizes the importance of rapid diagnosis and initiation of treatment for NCH, which is associated with a high rate of mortality and irreversible neurological morbidity. We outline the successful workup and management approach for this patient. The authors describe the successful work‐up and management of a patient who developed noncirrhotic hyperammonemia after deceased donor kidney transplantation, an exceedingly rare clinical entity with high rates of mortality and irreversible neurologic morbidity.