Anesthesia in Patients With Mitochondrial Disease

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.

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