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Record W4413946393 · doi:10.1186/s13023-025-03961-4

Measurement of fatigue in sickle cell disease: a systematic review of fatigue measures

2025· review· en· W4413946393 on OpenAlexfundno aff
A Gourdin, Damien Oudin Doglioni, Michalina Dannoune, Mélanie Astié, S. Monnier, Caroline Makowski, Marie‐Claire Gay

Bibliographic record

VenueOrphanet Journal of Rare Diseases · 2025
Typereview
Languageen
FieldMedicine
TopicHemoglobinopathies and Related Disorders
Canadian institutionsnot available
FundersNovo NordiskFondation Maladies RaresRare Disease Foundation
KeywordsDiseaseMedicineQuality of life (healthcare)Physical therapySystematic reviewCognitionChronic fatigue syndromeMEDLINEInclusion and exclusion criteriaClinical psychologyPsychiatryAlternative medicineInternal medicinePathology

Abstract

fetched live from OpenAlex

BACKGROUND: Sickle cell disease (SCD) is a chronic inherited blood disorder caused by abnormal haemoglobin production, affecting over seven million people worldwide. Although pain-particularly acute bone pain-is the hallmark symptom of this disease, fatigue is also a commonly observed manifestation. Fatigue is a debilitating symptom in Sickle Cell Disease (SCD) that significantly impacts quality of life. Accurate assessment of fatigue is crucial for effective disease management. However, a comprehensive analysis of fatigue assessment tools in SCD research is lacking. OBJECTIVE: This systematic literature review aims to identify and evaluate self-reported psychometric measures of fatigue used in SCD research with children, adolescents, young adults and adults. METHODS: A systematic search was conducted across six databases from 2010 to March 2024. The main inclusion criteria included peer-reviewed journal articles, patients with all SCD genotypes, studies evaluating fatigue using a self-reported psychometric measure, and studies published in English or French. The PRISMA guidelines were followed for study selection and data extraction. RESULTS: Twenty-eight studies met the inclusion criteria, reporting on 16 psychometric measures of fatigue. The most frequently used tool was the PROMIS system. Nine dimensions of fatigue were identified, including general, physical, mental, cognitive, emotional fatigue, and its impact on motivation, activity, vigour, and sleep/rest. However, the definitions of these dimensions were often unclear. Reported fatigue scores are not directly comparable due to methodological issues and variability in the assessment used. These methodological issues limit our knowledge on the prevalence of fatigue in SCD. CONCLUSION: The lack of a standardised fatigue assessment tool in SCD research hinders direct comparison of fatigue scores across studies. Future research should prioritise the development of a tailored assessment tool for SCD, considering the specific dimensions of fatigue relevant to this population. In the interim, clinicians and researchers can employ a combination of multidimensional and unidimensional tools to gain a more comprehensive understanding of patients' fatigue experiences.

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)0.017
metaresearch head score (Gemma)0.069
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesMetaresearch
Consensus categoriesnone
DomainCandidate signal: Methods · Consensus signal: none
Study designCandidate signal: Systematic review · Consensus signal: Systematic review
GenreCandidate signal: Review · Consensus signal: Review
Teacher disagreement score0.983
Threshold uncertainty score0.088

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0170.069
Meta-epidemiology (narrow)0.0020.001
Meta-epidemiology (broad)0.0080.010
Bibliometrics0.0120.010
Science and technology studies0.0010.001
Scholarly communication0.0020.003
Open science0.0020.002
Research integrity0.0020.001
Insufficient payload (model declined to judge)0.0030.000

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 head0.037
GPT teacher head0.312
Teacher spread0.275 · 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.

Study designSystematic review
DomainMethods
GenreReview

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

Citations0
Published2025
Admission routes1
Has abstractyes

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