title: Anesthesia for children with mitochondrial disorders: a national survey and review.
abstract: OBJECTIVE
  Mitochondrial diseases are a heterogeneous group of disorders. Patients with such diseases often need general anesthesia for diagnostic procedures and surgery; guidelines are lacking for the anesthetic care of these patients.
  
  
  METHODS
  We conducted a survey to investigate the current practices of pediatric anesthesiologists in the US in order to determine and document current practice. The survey consisted of twenty questions, including two demographic questions. A link to the survey was sent via email to members of the Society for Pediatric Anesthesia (2440), and was available online for 14 weeks.
  
  
  RESULTS
  Only 503 completed the survey: a response rate of 20.61 %. Among the responders, 93.2 % had children with mitochondrial disorders among their patients, but only 11 % had institutional guidelines for such cases in place. Among the responders, 80.3 % used the standard nil per os (NPO) status guidelines, while the rest give intravenous dextrose solution once NPO was in effect. Only 18.3 % took precautions for malignant hyperthermia during treatment. The majority of the practitioners chose sevoflurane as the safest inhaled agent for induction and maintenance (89.7 and 78.5 %, respectively). Regional anesthesia was deemed safe by 97.3 % of the responders. Lactated Ringer's solution was considered safe for these children by 49 %; only 47.8 % used dextrose-containing fluids for fluid replacement. The blood glucose was monitored by 72.7 %, and the majority (85 %) of this monitoring was done in a postanesthesia care unit.
  
  
  CONCLUSIONS
  Although the response rate was low, the majority of the responders provide care to these children routinely, so it can be inferred that the results of this survey are the closest published results to the true trend.
authors.affiliation: Department of Anesthesiology, University of Texas-Medical School at Houston, 6431 Fannin St., MSB 5.020, Houston, TX 77030, USA. Muhammad.Rafique@uth.tmc.edu
authors.forename: Muhammad B
authors.initials: MB
authors.lastname: Rafique
authors.forename: Staci D
authors.initials: SD
authors.lastname: Cameron
authors.forename: Qaiser
authors.initials: Q
authors.lastname: Khan
authors.forename: Suur
authors.initials: S
authors.lastname: Biliciler
authors.forename: Salman
authors.initials: S
authors.lastname: Zubair
mesh_terms: D000758:Anesthesia; D000765:Anesthesia, Conduction / Q000592:standards; D000769:Anesthesia, Inhalation / Q000592:standards; D001786:Blood Glucose / Q000378:metabolism; D002648:Child; D020927:Dexmedetomidine; D017408:Guidelines as Topic; D019538:Health Care Surveys; D006801:Humans; D006993:Hypnotics and Sedatives; D007552:Isotonic Solutions; D028361:Mitochondrial Diseases / Q000150:complications* / Q000201:enzymology; D061214:Patient Safety; D000077331:Ringer's Solution; D011795:Surveys and Questionnaires; D014481:United States
chemical_list: D001786:Blood Glucose; D006993:Hypnotics and Sedatives; D007552:Isotonic Solutions; D020927:Dexmedetomidine; D000077331:Ringer's Solution