Propofol administration in patients with methylmalonic acidemia and intracellular cobalamin metabolism disorders: a review of theoretical concerns and clinical experiences in 28 patients.

ABSTRACT:

Background

Methylmalonic acidemia and intracellular cobalamin metabolism disorders represent a heterogeneous group of inborn errors of metabolism. Most patients will require diagnostic and/or therapeutic procedures frequently requiring sedation or anesthetic management due to neurological and neurocognitive impairments. It has been stated that propofol is contraindicated in this population. We report our experience with propofol administration in a large series of patients.

Methods

Twenty eight patients (14 mut, seven cblC, three cblA, three cblB, one cblG) aged 2-35.6 years enrolled in a natural history study (ClinicalTrials.gov identifier: NCT00078078) and required anesthetics for 39 diagnostic or therapeutic procedures. Data were collected on the anesthetic technique, per

SUBMITTER: Ktena YP 

PROVIDER: S-EPMC5577977 | biostudies-literature | 2015 Sep

REPOSITORIES: biostudies-literature