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case_report·pediatrics, gastroenterology, neurology, clinical nutrition, pediatric ophthalmology·PMC10158269

Secondary Ammonia Scavenge With Glycerol Phenylbutyrate Improves Hyperammonemia Following Portosystemic Shunting

JPGN reports · 4 authors, 3 centres

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TL;DR

The treatment resulted in a significant reduction in serum ammonia levels and sustained clinical improvement in behavior and neurocognitive function.

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674 CHARS

After failing conventional therapies including lactulose, rifaximin, diet, and probiotics, he was started on glycerol phenylbutyrate. The primary outcome was a sustained stabilization of serum ammonia levels, with a pretreatment median of 64.0 µmol/L compared to a post-treatment median of 55.0 µmol/L. This biochemical improvement corresponded to marked behavioral and neurocognitive improvement, with no further episodes of hyperammonemic encephalopathy over 24 months. The case illustrates a potential therapeutic role for glycerol phenylbutyrate in noncirrhotic patients with portosystemic shunts, though the evidence is limited to a single case without a control group.

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case_report

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pediatrics

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100% · A

ORIGINAL PAPER

PMC10158269

Secondary Ammonia Scavenge With Glycerol Phenylbutyrate Improves Hyperammonemia Following Portosystemic Shunting

JPGN reports

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ABSTRACT

Abstract Portosystemic shunts are used to treat portal hypertension arising from extrahepatic portal venous obstruction. They decompress the portal system by allowing intestinal blood to bypass the liver and enter directly into the systemic circulation. These shunts increase the risk of minimal hepatic encephalopathy and altered neurodevelopmental outcomes in affected children. Treatment options are limited and have been extrapolated from those used in cirrhosis. We describe the use of glycerol phenylbutyrate as an alternate management strategy. A 3-year-old boy underwent distal splenorenal shunt for refractory variceal bleeding secondary to portal hypertension. He had neurologic deterioration and hyperammonemia refractory to traditional management strategies. Glycerol phenylbutyrate was initiated and resulted in a sustained improvement in ammonia levels, behavior, and school performance. This case illustrates the successful use of glycerol phenylbutyrate in a noncirrhotic patient with Portosystemic shunt and minimal hepatic encephalopathy refractory to conventional management strategies. Keywords: hepatic encephalopathy, minimal hepatic encephalopathy

CITATION EXPORT

Simone Kortbeek, Christy Gilkes, Aneal Khan et al.. Secondary Ammonia Scavenge With Glycerol Phenylbutyrate Improves Hyperammonemia Following Portosystemic Shunting. JPGN reports. (2022). https://doi.org/10.1097/PG9.0000000000000210

AUTHORS

4 · 3 CENTRES

Simone Kortbeek, Christy Gilkes, Aneal Khan, Alfred K Yeung

From the *Section of Pediatric Gastroenterology, Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada
†Section of Medical Genetics, Department of Pediatrics, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada
‡Pediatrician and Medical Geneticist M.A.G.I.C. Clinic Ltd. in Calgary and Vancouver, Adjunct Professor of Pediatrics, University of Calgary, Calgary, AB, Canada, Metabolic Consultant, Department of Pediatrics, Saskatchewan Health Authority