Abstract: FR-PO1004
Hyperammonemia Requiring Hemodialysis as Salvage Therapy in Short Bowel Syndrome
Session Information
- Hemodialysis: Clinical Challenges, Patient-Centered Outcomes, and Quality of Life
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Dialysis
- 801 Dialysis: Hemodialysis and Frequent Dialysis
Authors
- Patel, Dhara D., Jefferson Health - Northeast, Philadelphia, Pennsylvania, United States
- Scheid, Zachary, Jefferson Health - Northeast, Philadelphia, Pennsylvania, United States
- Bernabei, Luca L., Jefferson Health - Northeast, Philadelphia, Pennsylvania, United States
- Abubaker, Azza, Jefferson Health - Northeast, Philadelphia, Pennsylvania, United States
- Mcclellan, Elijah, Jefferson Health - Northeast, Philadelphia, Pennsylvania, United States
- Kern, Rachael, Jefferson Health - Northeast, Philadelphia, Pennsylvania, United States
- Fontanilla, John R., Jefferson Health - Northeast, Philadelphia, Pennsylvania, United States
- Biery, Matthew R., Jefferson Health - Northeast, Philadelphia, Pennsylvania, United States
Introduction
Hyperammonemia (HA) encephalopathy is classically associated with cirrhosis, but it can occur in non-cirrhotic patients with short bowel syndrome (SBS) on parenteral nutrition (TPN). An altered gut flora impairs the urea cycle; susceptibility increases during transitions of on and off TPN. Severe HA causes seizures and cerebral edema, constituting a neurologic emergency with a narrow therapeutic margin; this leaves dialysis as the only treatment option.
Case Description
A 67 year old female pmhx of Crohn’s Disease, SBS on TPN, prior HA requiring HD x1 presented with AMS. She had nausea and vomiting for 3 weeks. She was off TPN for 3 months due to worsening diarrhea. TPN was restarted 1 day prior to arrival. Vitals on arrival were stable however the patient was unresponsive. Workup showed ammonia 242 µmol/L. Creatinine 0.8, at baseline. Aminotransferases showed a cholestatic pattern. There was no evidence of cirrhosis. She was intubated and admitted for HA encephalopathy. Despite lactulose, HA worsened. She developed seizures and was given hypertonic saline for osmotherapy. Nephrology was consulted for evaluation for dialysis. Patient received 1 session of HD at blood flow rate of 250mL/min. Serum ammonia fell to 33 µmol/L from 276 µmol/L. CT head showed concern for cerebral edema. The last electrographic seizure occurred shortly after. The course was complicated by septic shock. Prior to CRRT, family opted for comfort care.
Discussion
This case illustrates severe HA encephalopathy in the setting of SBS requiring acute HD. The lack of intestinal floral in SBS alters arginine synthesis due decreased conversion of glutamine to citrulline. Normally, citrulline is converted to arginine in the kidneys. Arginine plays a key role in the urea cycle, aiding in conversion of ammonia to urea for safe excretion (1). Dialysis, as an adjuvant therapy, is indicated in HA with ammonia levels 3-4 times greater than upper limit of normal and associated encephalopathy (2). Treatment goal is to optimize clearance with HD initially, followed by CRRT to inhibit rebound HA (3). Although urea cycle disorders are commonly recognized causes of HA apart from cirrhosis, there were no cases of HA from high protein loads in parenteral nutrition, requiring HD, from our literature review. It is worth exploring the utility of HD even earlier in the course of HA encephalopathy.