title: Anesthetic management of a patient with isovaleric acidemia.
abstract: A 3-year-old male with isovaleric acidemia presented for dental rehabilitation under general anesthesia. In times of stress, such as in the perioperative period, patients with isovaleric acidemia are at greater risk for morbidity and mortality from disordered metabolism, including glucose disturbances, hyperammonemia, hypocalcemia, and non-anion gap metabolic acidosis. Communication between the anesthesiology, dental, and endocrine teams allowed for safe and successful care of the patient.
authors: From the Department of Pediatric Anesthesiology, Vanderbilt University Medical Center, Nashville, Tennessee.
authors: Humphrey
authors: H
authors: Lam
authors: Roy
authors: R
authors: Kiberenge
authors: Thanh
authors: T
authors: Nguyen
authors: Jenna Helmer
authors: JH
authors: Sobey
authors: Thomas
authors: T
authors: Austin
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy; D000768:Anesthesia, General / Q000009:adverse effects; D002675:Child, Preschool; D003731:Dental Caries / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D033183:Interdisciplinary Communication; D050769:Isovaleryl-CoA Dehydrogenase / Q000172:deficiency* / Q000378:metabolism; D008297:Male; D019647:Oral Surgical Procedures / Q000009:adverse effects; D010348:Patient Care Team; D011300:Preoperative Care; D018570:Risk Assessment; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D050769:Isovaleryl-CoA Dehydrogenase