A 72-year-old woman with end-stage kidney disease related to recurrent urinary tract infections, history of recurrent nephrolithiasis, and obstructive uropathy of a solitary kidney presented to our hospital for renal transplantation. Her past medical history was notable for a prior cerebrovascular accident without residual deficits, and oligometastatic colon cancer over 30 years ago, for which she underwent chemotherapy and extensive surgical resection, including a right colectomy, right nephrectomy, and partial hepatectomy.Subsequent to this surgery, she developed recurrent urinary tract infections and renal calculi requiring multiple procedural interventions. She had progressive deterioration of her remaining left kidney function and she eventually initiated hemodialysis 18 months prior to presentation. She had normal baseline functional status and was cleared by the transplantation committee at our institution to be listed as active on the United Network for Organ Sharing kidney waiting list. On the day of admission, a deceased donor organ with a Kidney Donor Profile Index of 85% had become available from a 55-year-old man with a 5-year history of hypertension who died of a hemorrhagic stroke. There was a 3 out of 6 human leukocyte antigen mismatch between donor and recipient. Pretransplant workups are as follows. Preoperative laboratory tests, including liver function tests, were normal with the exception of a creatinine of 4.7 mg/dL, blood urea nitrogen (BUN) of 15 mg/ 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.
K E Y W O R D Sclinical research/practice, encephalopathy, genetics, genetics, infection and infectious agents -bacterial, infectious disease, kidney disease, kidney transplantation/nephrology, metabolism/metabolite