Mitochondrial Diseases
Conditions
Keywords
Mitochondrial Diseases, Sevoflurane, Dexmedetomidine (Precedex®), Propofol
Brief summary
This pilot study is a prospective, randomized clinical trial to evaluate the effect of anesthesia in the mitochondrial dysfunction patient.
Detailed description
Study subjects undergoing a routine medical care non-emergent procedure will be randomized into three different groups to receive one of the following anesthetic agents; Sevoflurane, Propofol or Dexmedetomidine. The primary outcome of this pilot study is to evaluate and compare the incidence of adverse events in mitochondrial patients undergoing a diagnostic or therapeutic procedure up to 48 hours post anesthesia. The secondary outcome is to compare the metabolic derangements between three study groups by comparing changes in blood sugar, serum pH, serum bicarbonate, serum lactate and serum pyruvate levels before, during and after anesthesia in the groups.
Interventions
DRUGSevoflurane
Sevoflurane up to 8% will be administered to study subjects
DRUGDexmedetomidine
Dexmedetomidine (Precedex) infusion starting at 1mcg/ kg/ hr will be administered to study subjects.
DRUGPropofol
Propofol up to 50 mcg/ kg/min will be administered to study subjects.
Sponsors
The University of Texas Health Science Center, Houston
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
OTHER
Masking
TRIPLE (Subject, Caregiver, Investigator)
Eligibility
Sex/Gender
ALL
Age
1 Days to 17 Years
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Number of Participants Who Experienced an Adverse Event | Up to 48 hours post anesthesia. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Blood Glucose Level | Baseline | — |
| Serum pH | Baseline | The pH scale measures how acidic of alkaline a substance is and ranges from 0 to 14, where 7 is neutral, 14 is the most alkaline and 0 is the most acidic. |
| Serum Bicarbonate | Baseline | — |
| Serum Lactate | Baseline | — |
| Serum Pyruvate | Baseline | — |
Countries
United States
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Sevoflurane 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.
Sevoflurane: Sevoflurane up to 8% will be administered to study subjects | 6 |
| Dexmedetomidine (Precedex®) 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.
Dexmedetomidine: Dexmedetomidine (Precedex) infusion starting at 1mcg/ kg/ hr will be administered to study subjects. | 7 |
| Propofol 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.
Propofol: Propofol up to 50 mcg/ kg/min will be administered to study subjects. | 6 |
| Total | 19 |
Baseline characteristics
| Characteristic | Dexmedetomidine (Precedex®) | Propofol | Total | Sevoflurane |
|---|---|---|---|---|
| Age, Continuous | 6.57 years STANDARD_DEVIATION 4.15 | 4.33 years STANDARD_DEVIATION 2.33 | 5.84 years STANDARD_DEVIATION 3.67 | 6.5 years STANDARD_DEVIATION 4.22 |
| Race and Ethnicity Not Collected | — | — | 0 Participants | — |
| Region of Enrollment United States | 7 participants | 6 participants | 19 participants | 6 participants |
| Sex: Female, Male Female | 2 Participants | 0 Participants | 3 Participants | 1 Participants |
| Sex: Female, Male Male | 5 Participants | 6 Participants | 16 Participants | 5 Participants |
Adverse events
| Event type | EG000 affected / at risk | EG001 affected / at risk | EG002 affected / at risk |
|---|---|---|---|
| deaths Total, all-cause mortality | 0 / 6 | 0 / 7 | 0 / 6 |
| other Total, other adverse events | 3 / 6 | 2 / 7 | 2 / 6 |
| serious Total, serious adverse events | 0 / 6 | 0 / 7 | 0 / 6 |
Outcome results
Primary
Number of Participants Who Experienced an Adverse Event
Time frame: Up to 48 hours post anesthesia.
| Arm | Measure | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|
| Dexmedetomidine (Precedex®) | Number of Participants Who Experienced an Adverse Event | 2 Participants |
| Propofol | Number of Participants Who Experienced an Adverse Event | 2 Participants |
| Sevoflurane | Number of Participants Who Experienced an Adverse Event | 3 Participants |
Secondary
Blood Glucose Level
Time frame: Baseline
Population: Data were not collected for one participant in the Dexmedetomidine (Precedex®) arm. Data were not collected for one participant in the Propofol arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Blood Glucose Level | 88.66 milligrams per decilitre | Standard Deviation 15.29 |
| Propofol | Blood Glucose Level | 89.20 milligrams per decilitre | Standard Deviation 4.76 |
| Sevoflurane | Blood Glucose Level | 83.66 milligrams per decilitre | Standard Deviation 10.2 |
Secondary
Blood Glucose Level
Time frame: 24 hours post-operative
Population: Data were not collected for 4 participants in the Dexmedetomidine (Precedex®) arm. Data were not collected for 4 participants in the Propofol arm. Data were not collected for 2 participants in the Sevoflurane arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Blood Glucose Level | 87 milligrams per decilitre | Standard Deviation 7 |
| Propofol | Blood Glucose Level | 81.50 milligrams per decilitre | Standard Deviation 13.43 |
| Sevoflurane | Blood Glucose Level | 93.75 milligrams per decilitre | Standard Deviation 16.5 |
Secondary
Serum Bicarbonate
Time frame: Baseline
Population: Data were not collected for one participant in the Dexmedetomidine (Precedex®) arm. Data were not collected for one participant in the Propofol arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Serum Bicarbonate | 25.50 millimoles per liter | Standard Deviation 1.91 |
| Propofol | Serum Bicarbonate | 24.04 millimoles per liter | Standard Deviation 3.66 |
| Sevoflurane | Serum Bicarbonate | 25.23 millimoles per liter | Standard Deviation 2.51 |
Secondary
Serum Bicarbonate
Time frame: 24 hours post-operative
Population: Data were not collected for 4 participants in the Dexmedetomidine (Precedex®) arm. Data were not collected for 5 participants in the Propofol arm. Data were not collected for 4 participants in the Sevoflurane arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Serum Bicarbonate | 24.33 millimoles per liter | Standard Deviation 4.16 |
| Propofol | Serum Bicarbonate | 20 millimoles per liter | — |
| Sevoflurane | Serum Bicarbonate | 26.5 millimoles per liter | Standard Deviation 2.12 |
Secondary
Serum Lactate
Time frame: Baseline
Population: Data were not collected for one participant in the Dexmedetomidine (Precedex®) arm. Data were not collected for one participant in the Propofol arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Serum Lactate | 1.28 millimoles per liter | Standard Deviation 0.44 |
| Propofol | Serum Lactate | 0.82 millimoles per liter | Standard Deviation 0.25 |
| Sevoflurane | Serum Lactate | 1.36 millimoles per liter | Standard Deviation 0.58 |
Secondary
Serum Lactate
Time frame: 24 hours post-operative
Population: Data were not collected for 3 participants in the Dexmedetomidine (Precedex®) arm. Data were not collected for 3 participants in the Propofol arm. Data were not collected for 2 participants in the Sevoflurane arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Serum Lactate | 2.76 millimoles per liter | Standard Deviation 0.47 |
| Propofol | Serum Lactate | 1.15 millimoles per liter | Standard Deviation 0.36 |
| Sevoflurane | Serum Lactate | 2.50 millimoles per liter | Standard Deviation 0.65 |
Secondary
Serum pH
The pH scale measures how acidic of alkaline a substance is and ranges from 0 to 14, where 7 is neutral, 14 is the most alkaline and 0 is the most acidic.
Time frame: 24 hours post-operative
Population: Data were not collected for 4 participants in the Dexmedetomidine (Precedex®) arm. Data were not collected for 5 participants in the Propofol arm. Data were not collected for 4 participants in the Sevoflurane arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Serum pH | 7.36 score on a scale | Standard Deviation 0.05 |
| Propofol | Serum pH | 7.40 score on a scale | — |
| Sevoflurane | Serum pH | 7.32 score on a scale | Standard Deviation 0.04 |
Secondary
Serum pH
The pH scale measures how acidic of alkaline a substance is and ranges from 0 to 14, where 7 is neutral, 14 is the most alkaline and 0 is the most acidic.
Time frame: Baseline
Population: Data were not collected for one participant in the Dexmedetomidine (Precedex®) arm. Data were not collected for one participant in the Propofol arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Serum pH | 7.39 score on a scale | Standard Deviation 7.37 |
| Propofol | Serum pH | 7.36 score on a scale | Standard Deviation 0.05 |
| Sevoflurane | Serum pH | 7.37 score on a scale | Standard Deviation 0.02 |
Secondary
Serum Pyruvate
Time frame: Baseline
Population: Data were not collected for 3 participants in the Dexmedetomidine (Precedex®) arm. Data were not collected for 3 participants in the Propofol arm. Data were not collected for 2 participants in the Sevoflurane arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Serum Pyruvate | 0.72 milligrams per decilitre | Standard Deviation 0.47 |
| Propofol | Serum Pyruvate | 1.06 milligrams per decilitre | Standard Deviation 0.36 |
| Sevoflurane | Serum Pyruvate | 1.03 milligrams per decilitre | Standard Deviation 0.65 |
Secondary
Serum Pyruvate
Time frame: 24 hours post-operative
Population: Data were not collected for 5 participants in the Dexmedetomidine (Precedex®) arm. Data were not collected for 5 participants in the Propofol arm. Data were not collected for 3 participants in the Sevoflurane arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Dexmedetomidine (Precedex®) | Serum Pyruvate | 1.34 milligrams per decilitre | Standard Deviation 0.85 |
| Propofol | Serum Pyruvate | 1.32 milligrams per decilitre | — |
| Sevoflurane | Serum Pyruvate | 1.42 milligrams per decilitre | Standard Deviation 0.69 |