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Record W2343647993 · doi:10.3899/jrheum.151357

Heart Involvement in a Woman Treated with Hydroxychloroquine for Systemic Lupus Erythematosus Revealing Fabry Disease

2016· letter· en· W2343647993 on OpenAlexvenueno aff
C. Chatre, Nathalie Filippi, François Roubille, Yves‐Marie Pers

Bibliographic record

VenueThe Journal of Rheumatology · 2016
Typeletter
Languageen
FieldMedicine
TopicLysosomal Storage Disorders Research
Canadian institutionsnot available
FundersUniversité de MontpellierCentre National de la Recherche ScientifiqueInstitut National de la Santé et de la Recherche Médicale
KeywordsHydroxychloroquineMedicineFabry diseaseAnti-nuclear antibodyCardiomyopathyRashConnective tissue diseaseInternal medicineDermatologyDiseaseChloroquineHeart failurePathologyAutoimmune diseaseImmunologyAntibodyAutoantibody

Abstract

fetched live from OpenAlex

To the Editor: Chloroquine (CQ) and hydroxychloroquine (HCQ) are widely used in the longterm treatment of connective tissue disease (CTD) and are usually considered safe1. However, these therapies may cause serious adverse events2, including cardiac toxicity3. The cardiomyopathy induced by an antimalarial drug might mimic the cardiac involvement of Fabry disease (FD), a genetic storage disorder that causes a deficiency of a lysosomal enzyme, alpha galactosidase A (GLA). Indeed, CQ and HCQ provoke a dysfunction in the lysosomal enzymes, leading to the impairment of intracellular degradation processes in conjunction with the accumulation of pathological metabolic products4,5. The cardiac presentation of these 2 diseases is close regarding the clinical symptoms as well as the imaging and pathological findings. We report the case of a patient, treated longterm with HCQ, with cardiac disorders revealing FD. A 61-year-old woman was diagnosed 20 years ago for a CTD with dermatological disorders: alopecia, skin rash, and joint pain. The antinuclear antibodies (ANA) were positive at 1/100. Anti-SSa/Ro and anti-dsDNA antibodies were negative. The … Address correspondence to Dr. Y.M. Pers, 371 Avenue du Doyen Gaston Giraud, 34295 Montpellier, France. E-mail: ympers2000{at}yahoo.fr

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)0.001
metaresearch head score (Gemma)0.004
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Case report · Consensus signal: Case report
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.006
Threshold uncertainty score0.009

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0010.004
Meta-epidemiology (narrow)0.0020.001
Meta-epidemiology (broad)0.0020.001
Bibliometrics0.0020.001
Science and technology studies0.0010.001
Scholarly communication0.0010.002
Open science0.0020.001
Research integrity0.0060.003
Insufficient payload (model declined to judge)0.0030.001

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 head0.022
GPT teacher head0.278
Teacher spread0.257 · 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 designCase report
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

Citations22
Published2016
Admission routes1
Has abstractyes

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