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Record W2900810914 · doi:10.1097/cnd.0000000000000204

Current Cardiac Imaging Approaches in Duchenne Muscular Dystrophy

2018· article· en· W2900810914 on OpenAlexaff
Sabrina Poonja, Alyssa Power, Jean K. Mah, Nowell M. Fine, Steven C. Greenway

Bibliographic record

VenueJournal of Clinical Neuromuscular Disease · 2018
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicMuscle Physiology and Disorders
Canadian institutionsLibin Cardiovascular Institute of AlbertaAlberta Children's HospitalUniversity of Calgary
Fundersnot available
KeywordsMedicineDuchenne muscular dystrophyCardiomyopathyDilated cardiomyopathyCardiologyInternal medicineSubclinical infectionMuscular dystrophyGold standard (test)Myocardial fibrosisHeart failureCardiac function curveDisease

Abstract

fetched live from OpenAlex

Duchenne muscular dystrophy (DMD) is an X-linked neuromuscular condition caused by mutations in the dystrophin gene leading to skeletal muscle weakness and dilated cardiomyopathy. The prevalence of DMD-related cardiomyopathy increases with age and is almost universal by the third decade of life. Myocardial fibrosis and progressive left ventricular dysfunction lead to the development of heart failure and premature death. With modern advances in medical and surgical management for patients with DMD increasing their life expectancy, cardiac dysfunction represents an increasing cause of morbidity and mortality in these patients. Early diagnosis of dilated cardiomyopathy before symptom development enables the initiation of potentially disease-modifying therapies, but requires regular dedicated imaging surveillance with sufficient sensitivity to detect subclinical changes in cardiac structure and function. Currently, transthoracic echocardiography (TTE) and cardiac magnetic resonance imaging (CMR) are commonly used and have complementary roles. TTE is rapid and readily available, whereas CMR is the gold standard for the quantification of ventricular structure and function and can detect the presence and extent of myocardial fibrosis, an increasingly appreciated marker for early disease. This review describes the clinical applications, advantages, and disadvantages of cardiac imaging screening and surveillance for the myocardial manifestations of DMD, with a particular focus on TTE and CMR.

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 distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.001
Version: codex-gemma-dda1882f352aValidation 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 score0.131
Threshold uncertainty score0.675

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.001
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

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 head0.049
GPT teacher head0.350
Teacher spread0.301 · 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 teacher head, 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

Citations20
Published2018
Admission routes1
Has abstractyes

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