Anesthesia in Patients With Mitochondrial Disease

This pilot study is a prospective, randomized clinical trial to evaluate the effect of anesthesia in the mitochondrial dysfunction patient.

Study Overview

• Status: Terminated
• Conditions: Mitochondrial Diseases
• Intervention / Treatment: Drug: Sevoflurane; Drug: Dexmedetomidine; Drug: Propofol

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.

• Study Type: Interventional
• Enrollment (Actual): 19
• Phase: Not Applicable

Contacts and Locations

Study Locations

• United States
  • Texas
    • Houston, Texas, United States, 77030
      • University of Texas Health Science Center at Houston

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 3 years to 13 years (Child)
• Accepts Healthy Volunteers: No

Description

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

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Other
• Allocation: Randomized
• Interventional Model: Parallel Assignment
• Masking: Triple

Number of Arms

3

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Active Comparator: 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.	Drug: Sevoflurane; Sevoflurane up to 8% will be administered to study subjects; Other Names: Ultane
Active Comparator: 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.	Drug: Dexmedetomidine; Dexmedetomidine (Precedex) infusion starting at 1mcg/ kg/ hr will be administered to study subjects.; Other Names: Precedex
Active Comparator: 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.	Drug: Propofol; Propofol up to 50 mcg/ kg/min will be administered to study subjects.; Other Names: Diprivan

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Number of Participants Who Experienced an Adverse Event	Up to 48 hours post anesthesia.

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Blood Glucose Level		Baseline
Blood Glucose Level		24 hours post-operative
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.	Baseline
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.	24 hours post-operative
Serum Bicarbonate		Baseline
Serum Bicarbonate		24 hours post-operative
Serum Lactate		Baseline
Serum Lactate		24 hours post-operative
Serum Pyruvate		Baseline
Serum Pyruvate		24 hours post-operative

Collaborators and Investigators

• Sponsor: The University of Texas Health Science Center, Houston
• Investigators: Principal Investigator: Maria Matuszczak, MD, The University of Texas Health Science Center, Houston

Study record dates

Study Major Dates

• Study Start (Actual): January 10, 2014
• Primary Completion (Actual): October 1, 2020
• Study Completion (Actual): October 1, 2020

Study Registration Dates

• First Submitted: January 31, 2014
• First Submitted That Met QC Criteria: January 31, 2014
• First Posted (Estimated): February 4, 2014

Study Record Updates

• Last Update Posted (Actual): August 28, 2024
• Last Update Submitted That Met QC Criteria: August 6, 2024
• Last Verified: August 1, 2024

More Information

Terms related to this study

• Keywords: Propofol; Sevoflurane; Mitochondrial Diseases; Dexmedetomidine (Precedex®)
• Additional Relevant MeSH Terms: Metabolic Diseases; Mitochondrial Diseases; Physiological Effects of Drugs; Adrenergic Agents; Neurotransmitter Agents; Molecular Mechanisms of Pharmacological Action; Central Nervous System Depressants; Peripheral Nervous System Agents; Analgesics; Sensory System Agents; Anesthetics, Intravenous; Anesthetics, General; Anesthetics; Analgesics, Non-Narcotic; Platelet Aggregation Inhibitors; Adrenergic alpha-2 Receptor Agonists; Adrenergic alpha-Agonists; Adrenergic Agonists; Hypnotics and Sedatives; Anesthetics, Inhalation; Propofol; Dexmedetomidine; Sevoflurane
• Other Study ID Numbers: HSC-MS-13-0600

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO