Evidence map›Paper›PMID 30023305›Full record

ArticleMolecular genetics and metabolism reports2018

Marked elevation in plasma trimethylamine-N-oxide (TMAO) in patients with mitochondrial disorders treated with oral l-carnitine.

H D Vallance, A Koochin, J Branov, A Rosen-Heath, T Bosdet, Z Wang, S L Hazen, G Horvath

Open access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Molecular genetics and metabolism reports, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
25citing papers in PubMed, 1 pooled it
4.1field-weighted citation impact, top 6% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

25 citing papers in PubMed, 1 synthesis or guideline pooled it, 42 citations in OpenAlex.

  1. The bright and the dark sides of L-carnitine supplementation: a systematic review.Journal of the International Society of Sports Nutrition · 2020
    Pooled it
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  20. Carnitine Inborn Errors of Metabolism.Molecules (Basel, Switzerland) · 2019
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors at 2 institutions in 2 countries.

H D VallanceBiochemical Genetics laboratory, Department of Pathology and Laboratory Medicine, BC Children's Hospital, 4480 Oak Street, Vancouver, BC, Canada.
A KoochinAdult Metabolic Diseases Clinic, Level 4 - 2775 Laurel Street, V5Z 1M0 Vancouver, BC, Canada.
J BranovAdult Metabolic Diseases Clinic, Level 4 - 2775 Laurel Street, V5Z 1M0 Vancouver, BC, Canada.
A Rosen-HeathAdult Metabolic Diseases Clinic, Level 4 - 2775 Laurel Street, V5Z 1M0 Vancouver, BC, Canada.
T BosdetAdult Metabolic Diseases Clinic, Level 4 - 2775 Laurel Street, V5Z 1M0 Vancouver, BC, Canada.
Z WangDepartment of Cellular and Molecular Medicine, Center for Microbiome & Human Health, Lerner Research Institute, Cleveland Clinic, OH 44195, USA.
S L HazenDepartment of Cellular and Molecular Medicine, Center for Microbiome & Human Health, Lerner Research Institute, Cleveland Clinic, OH 44195, USA.
G HorvathAdult Metabolic Diseases Clinic, Level 4 - 2775 Laurel Street, V5Z 1M0 Vancouver, BC, Canada.
Cleveland Clinic Lerner College of Medicine · USBritish Columbia Children's Hospital · CA

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Oral supplementation with l-carnitine is a common therapeutic modality for mitochondrial disorders despite limited evidence of efficacy. Recently, a number of studies have demonstrated that a gut microbiota-dependent metabolite of l-carnitine, trimethylamine oxide (TMAO), is an independent and dose-dependent risk factor for cardiovascular disease (CVD). Given the limited data demonstrating efficacy with oral l-carnitine therapy and the newly raised questions of potential harm, we assessed plasma TMAO levels in patients with mitochondrial disease with and without oral l-carnitine supplementation. Nine subjects were recruited and completed the study. Eight out of 9 subjects at baseline had plasma TMAO concentrations <97.5th percentile (<15.5 μM). One subject with stage 3 renal disease, had marked elevation in plasma TMAO (pre 33.98 μm versus post 101.6 μm). Following at least 3 months of l-carnitine supplementation (1000 mg per day), plasma TMAO levels were markedly increased in 7out of 9 subjects; overall, plasma TMAO significantly increased 11.8-fold (p < 0.001) from a baseline median level of 3.54 μm (interquartile range (IQR) 2.55-8.72) to 43.26 (IQR 23.99-56.04) post supplementation. The results of this study demonstrate that chronic oral l-carnitine supplementation markedly increases plasma TMAO levels in subjects with mitochondrial disorders. Further studies to evaluate both the efficacy and long term safety of oral l-carnitine supplementation for the treatment of mitochondrial disorders are warranted.

Indexed as

l-carnitineMitochondrial disordersTrimethylamine N-oxide

Identifiers

PMID30023305
PMCPMC6047224
OpenAlexW2800244745

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.