Anesthesia in Patients With Mitochondrial Disease
Mitochondrial Diseases
About this trial
This is an interventional other trial for Mitochondrial Diseases focused on measuring Mitochondrial Diseases, Sevoflurane, Dexmedetomidine (Precedex®), Propofol
Eligibility Criteria
Inclusion Criteria:
- Subjects must fulfill the following inclusion criteria:
- Subject is informed and given ample opportunity to consider his/her participation and has given his/her written consent.
- Subject is willing and able to comply with all study requirements.
- Subject is between 0 - 17 years of age.
- Subject has been diagnosed with mitochondrial dysfunction based on modified Walker criteria.
- Subject is scheduled to have a non-emergent diagnostic or therapeutic procedure for routine medical care requiring general anesthesia estimated to last at least one hour.
- Subject is classified ASA I - IV
Exclusion Criteria:
Subjects are not permitted to enroll in the study if any of the following criteria are met:
- Subject is older than 17 years
- Subject is pregnant
- Subject is a nursing female and
- Subject has participated in the same study within 48 hours
- Subject is allergic or has had any adverse effect to any of the study agents in the past
- Anesthesia time is less than one hour
- Subject is classified ASA V
Sites / Locations
- University of Texas Health Science Center at HoustonRecruiting
Arms of the Study
Arm 1
Arm 2
Arm 3
Active Comparator
Active Comparator
Active Comparator
Sevoflurane
Dexmedetomidine (Precedex®)
Propofol
In the Operating Room (OR) after applying routine monitors, anesthesia will be induced with a mask using Sevoflurane up to 8%. Sevoflurane will be used for maintenance for the rest of the procedure with dosage between 1.5% and 4%. Vitals will be monitored. Fentanyl will be used as needed. Rocuronium will be used as needed for muscle relaxation. At the end of the procedure twitches will be evaluated and muscle relaxation will be reversed. Sevoflurane will be turned off at the end of procedure.
These subjects will receive Dexmedetomidine intravenously. In the OR routine monitors will be placed. Vitals will be monitored. A loading dose of Precedex 1mcg/ kg will be given over 10 minutes. Infusion will be started using Precedex 1mcg/ kg/ hr and can be increased or decreased as needed. Fentanyl will be used as needed. Rocuronium will be used as needed for muscle relaxation. At the end of the procedure twitches will be evaluated and muscle relaxation will be reversed. Precedex infusion will be stopped at the end of the procedure.
These subjects will receive propofol for anesthesia maintenance. In the OR routine monitors will be placed. Vitals will be monitored. A loading dose of Propofol 2mg/ kg will be given over 2 minutes. Infusion will be started using Propofol 50 mcg/ kg/min and can be increased or decreased as needed (range 50-300mcg/kg/min). Fentanyl will be used as needed. Rocuronium will be used as needed for muscle relaxation. At the end of the procedure twitches will be evaluated and muscle relaxation will be reversed. Propofol infusion will be stopped at the end of the procedure.