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Updated: Sep 27, 2025

Author Spotlight: Advancing Cellular and Protein Engineering to Control Biological Functions and Develop Novel Therapies
Published on: September 27, 2024
Anesthetic Management of a Patient With S-Adenosylhomocysteine Hydrolase Deficiency: A Case Report
Cori R Van Gorkom1, Emily D Black2,3, Joelle B Karlik1,3
1From the Departments of Anesthesiology and Pain Medicine.
Abstract:
S-adenosylhomocysteine hydrolase (AHCY) deficiency is a rare congenital disorder in methionine metabolism with minimal guidelines regarding anesthetic management. This case report describes a 19-year-old man presenting for a liver biopsy in interventional radiology due to a history of elevated aminotransferases and creatine kinase. He received dextrose-containing fluids and a total intravenous anesthetic to avoid rhabdomyolysis and hyperkalemia. Anesthetic goals for patients with AHCY deficiency should focus on avoiding rhabdomyolysis, minimizing postoperative ventilatory compromise, monitoring for potential coagulopathy, and providing anxiolysis.
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