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Record W2964811316 · doi:10.1017/cjn.2019.240

Health Care for Mitochondrial Disorders in Canada: A Survey of Physicians

2019· article· en· W2964811316 on OpenAlexafffundvenueabout
Karen Paik, Matthew A. Lines, Pranesh Chakraborty, Sara D. Khangura, Maureen Latocki, Walla Al‐Hertani, Catherine Brunel‐Guitton, Aneal Khan, Blaine Penny, Cheryl R. Greenberg, C. Anthony Rupar, Neal Sondheimer, Mark A. Tarnopolsky, Kylie Tingley, Doug Coyle, Sarah Dyack, Annette Feigenbaum, Michael T. Geraghty, Jane Gillis, Clara van Karnebeek, Jonathan B. Kronick, Julian Little, Murray Potter, Komudi Siriwardena, Rebecca Sparkes, Lesley Turner, Kumanan Wilson, Daniela Buhaş, Beth K. Potter

Bibliographic record

VenueCanadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques · 2019
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicMitochondrial Function and Pathology
Canadian institutionsMemorial University of NewfoundlandUniversity of AlbertaMcMaster UniversityUniversity of British ColumbiaBC Children's HospitalStollery Children's HospitalIzaak Walton Killam Health CentreOttawa HospitalMcMaster University Medical CentreHospital for Sick ChildrenWestern UniversityMItoCanadaChildren's Hospital of WinnipegChildren's Hospital Research Institute of ManitobaLondon Health Sciences CentreDalhousie UniversityCentre Hospitalier Universitaire Sainte-JustineUniversity of ManitobaNewborn Screening OntarioUniversity of CalgaryChildren's Hospital of Eastern OntarioMcGill University Health CentreUniversity of Ottawa
FundersCanadian Institutes of Health Research
KeywordsSnowball samplingMedicineMitochondrial diseaseFamily medicineHealth careMEDLINEMitochondrial DNAPathology

Abstract

fetched live from OpenAlex

BACKGROUND: An improved understanding of diagnostic and treatment practices for patients with rare primary mitochondrial disorders can support benchmarking against guidelines and establish priorities for evaluative research. We aimed to describe physician care for patients with mitochondrial diseases in Canada, including variation in care. METHODS: We conducted a cross-sectional survey of Canadian physicians involved in the diagnosis and/or ongoing care of patients with mitochondrial diseases. We used snowball sampling to identify potentially eligible participants, who were contacted by mail up to five times and invited to complete a questionnaire by mail or internet. The questionnaire addressed: personal experience in providing care for mitochondrial disorders; diagnostic and treatment practices; challenges in accessing tests or treatments; and views regarding research priorities. RESULTS: We received 58 survey responses (52% response rate). Most respondents (83%) reported spending 20% or less of their clinical practice time caring for patients with mitochondrial disorders. We identified important variation in diagnostic care, although assessments frequently reported as diagnostically helpful (e.g., brain magnetic resonance imaging, MRI/MR spectroscopy) were also recommended in published guidelines. Approximately half (49%) of participants would recommend "mitochondrial cocktails" for all or most patients, but we identified variation in responses regarding specific vitamins and cofactors. A majority of physicians recommended studies on the development of effective therapies as the top research priority. CONCLUSIONS: While Canadian physicians' views about diagnostic care and disease management are aligned with published recommendations, important variations in care reflect persistent areas of uncertainty and a need for empirical evidence to support and update standard protocols.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.002
metaresearch head score (Gemma)0.007
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.037
Threshold uncertainty score0.268

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0020.007
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.005
Science and technology studies0.0040.001
Scholarly communication0.0010.001
Open science0.0010.001
Research integrity0.0010.001
Insufficient payload (model declined to judge)0.0020.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.018
GPT teacher head0.259
Teacher spread0.240 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations5
Published2019
Admission routes4
Has abstractyes

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Same venueCanadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques→Same topicMitochondrial Function and Pathology→French-language works237,207→