title: Propofol administration in patients with methylmalonic acidemia and intracellular cobalamin metabolism disorders: a review of theoretical concerns and clinical experiences in 28 patients.
abstract: BACKGROUND
  Methylmalonic acidemia and intracellular cobalamin metabolism disorders represent a heterogeneous group of inborn errors of metabolism. Most patients will require diagnostic and/or therapeutic procedures frequently requiring sedation or anesthetic management due to neurological and neurocognitive impairments. It has been stated that propofol is contraindicated in this population. We report our experience with propofol administration in a large series of patients.
  
  
  METHODS
  Twenty eight patients (14 mut, seven cblC, three cblA, three cblB, one cblG) aged 2-35.6 years enrolled in a natural history study (ClinicalTrials.gov identifier: NCT00078078) and required anesthetics for 39 diagnostic or therapeutic procedures. Data were collected on the anesthetic technique, perianesthetic course, and adverse events related to propofol.
  
  
  RESULTS
  Propofol was used as the sole induction agent in most cases (36/39) and as the primary maintenance agent in all cases. Infusion rates were 100-400 mcg kg(-1) min(-1) (mean = 214). Infusion duration was 60-325 min (mean = 158) and total doses ranged between 270-3610 mg (mean = 1217). Adverse events were recorded in two cases; neither appeared to be related to propofol administration.
  
  
  CONCLUSIONS
  Propofol is an effective, safe induction and maintenance agent for elective short procedures requiring anesthesia in patients with MMA and cobalamin metabolism disorders. Despite multiple comorbidities and propensity toward instability, those affected can receive anesthesia with an acceptable safety profile, if metabolically and hemodynamically stabilized prior to the event.
  
  
  CONCLUSIONS
  A review of the perianesthetic records of 28 patients with isolated MMA and intracellular cobalamin metabolism disorders suggests that propofol anesthesia can be administered safely to these patients, in the setting of metabolic stability.
authors.affiliation: Organic Acid Research Section, Genetics and Molecular Biology Branch, National Human Genome Research Institute, National Institutes of Health, 49 Convent Drive, Building 49, Room 4A18, Bethesda, MD, USA.
authors.forename: Yiouli P
authors.initials: YP
authors.lastname: Ktena
authors.forename: Trygg
authors.initials: T
authors.lastname: Ramstad
authors.forename: Eva H
authors.initials: EH
authors.lastname: Baker
authors.forename: Jennifer L
authors.initials: JL
authors.lastname: Sloan
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Mannes
authors.forename: Irini
authors.initials: I
authors.lastname: Manoli
authors.forename: Charles P
authors.initials: CP
authors.lastname: Venditti
mesh_terms: D000293:Adolescent; D000328:Adult; D000592:Amino Acid Metabolism, Inborn Errors / Q000188:drug therapy* / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D042541:Intracellular Space / Q000378:metabolism; D008297:Male; D008659:Metabolic Diseases / Q000378:metabolism* / Q000473:pathology; D008962:Models, Theoretical; D015742:Propofol / Q000008:administration & dosage*; D012189:Retrospective Studies; D014805:Vitamin B 12 / Q000378:metabolism*; D055815:Young Adult
chemical_list: D014805:Vitamin B 12; D015742:Propofol