Mark Shlomovich, MD
Director of Extracorporeal Liver Support
Children's Hospital At Montefiore, United States
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Title: MARS as Bridge to Recovery in an Adolescent with Idiopathic Acute Liver Failure
Case Report Body:
Introduction:
Pediatric acute liver failure (ALF) carries significant morbidity; Molecular Adsorbent Recirculating System (MARS) therapy is a useful but infrequently used tool to aid in bridging to recovery or liver transplantation, specifically for the management of hepatic encephalopathy (HE).
Description:
A 17 year old male with obesity and history of elevated liver enzymes (suspected non-alcoholic steatohepatitis) presented to an outside emergency room with 3 days of emesis, progressive fatigue and then altered mental status, and was transferred to our institution for liver transplant evaluation. Initial labs showed AST of 4377, ALT of 6212, total bilirubin of 5.2, INR of 5.6 (post-fresh frozen plasma), and ammonia of 270. N-acetyl cysteine was started, and he was emergently listed for liver transplantation. On hospital day 1, he was placed on continuous veno-venous hemodiafiltration (CVVHDF) for ammonia clearance. Ammonia level decreased to 150 after 12 hours of CVVHDF, with HE scores improving from 3 to 2. On hospital day 2, MARS was initiated, with resultant decrease in ammonia level to 90 and significant improvement in mental status, out of proportion to change in serum ammonia. He remained fatigued but became alert, oriented, and fully conversant after 1 MARS cycle, with HE scores improving to 1 on hospital day 3, and 0 by hospital day 4. Due to his improving mental status, MARS was discontinued on hospital day 4 (after 3 runs of 12-24 hours each). Around this time, his native liver synthetic function improved, and coagulopathy resolved. Pathology from trans-venous liver biopsy was consistent with toxic or drug-induced liver injury; all infectious and toxic workup was negative, and no definitive cause for the ALF was identified. The patient was removed from the transplant list, discharged home, and has continued to recover with close clinical follow-up.
Discussion:
Treating HE is a challenging and important component to managing pediatric acute liver failure, especially in the peri-transplant period. We report excellent results with the use of combined renal replacement therapy and MARS for the treatment of HE in a teenage male with ALF, a first at our institution.