ReviewJournal of inherited metabolic disease2015
Propofol administration in patients with methylmalonic acidemia and intracellular cobalamin metabolism disorders: a review of theoretical concerns and clinical experiences in 28 patients.
Review in Journal of inherited metabolic disease, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00078078 (Clinical and Basic Investigations of Methylmalonic Acidemia), which is not on this map. Cited by 9 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Clinical and Basic Investigations of Methylmalonic Acidemia (MMA) and Related Disorders
Who cites it
9 citing papers in PubMed, 14 citations in OpenAlex.
- General Anesthesia for a Child With Methylmalonic Acidemia Undergoing Oral Care and Dental Treatment Before Kidney Transplantation.Anesthesia progress · 2024Article
- Versatile enzymology and heterogeneous phenotypes in cobalamin complementation type C disease.iScience · 2022Review
- Ketamine and dexmedetomidine sedation for brain magnetic resonance imaging in methylmalonic acidemia.Indian journal of anaesthesia · 2021Article
- Anaesthetic considerations in a child with methylmalonic acidemia and its literature review.BMJ case reports · 2020Review
- Metabolic Disorders and Anesthesia.Current anesthesiology reports · 2019Article
- Successful perioperative management of living-donor liver transplantation for a patient with severe methylmalonic acidemia: a case report.JA clinical reports · 2018Article
- Anesthetic management of a patient with methylmalonic acidemia: a case report.JA clinical reports · 2018Article
- Anaesthetic considerations in a patient with methylmalonyl-coenzyme A mutase deficiency.Indian journal of anaesthesia · 2017Article
- Guidelines for diagnosis and management of the cobalamin-related remethylation disorders cblC, cblD, cblE, cblF, cblG, cblJ and MTHFR deficiency.Journal of inherited metabolic disease · 2017Review
Corrections and comments
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundMethylmalonic 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.
methodsTwenty 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, perianesthetic course, and adverse events related to propofol.
resultsPropofol was used as the sole induction agent in most cases (36/39) and as the primary maintenance agent in all cases. Infusion rates were 100-400 mcg kg(-1) min(-1) (mean = 214). Infusion duration was 60-325 min (mean = 158) and total doses ranged between 270-3610 mg (mean = 1217). Adverse events were recorded in two cases; neither appeared to be related to propofol administration.
conclusionsPropofol is an effective, safe induction and maintenance agent for elective short procedures requiring anesthesia in patients with MMA and cobalamin metabolism disorders. Despite multiple comorbidities and propensity toward instability, those affected can receive anesthesia with an acceptable safety profile, if metabolically and hemodynamically stabilized prior to the event. SYNOPSIS: A review of the perianesthetic records of 28 patients with isolated MMA and intracellular cobalamin metabolism disorders suggests that propofol anesthesia can be administered safely to these patients, in the setting of metabolic stability.
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Registered trials
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