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Record W2535041295 · doi:10.1111/hae.13111

European retrospective study of real‐life haemophilia treatment

2016· article· en· W2535041295 on OpenAlexaff
Erik Berntorp, Gerard Dolan, C. R. M. Hay, Silvia Linari, Elena Santagostino, Alberto Tosetto, Giancarlo Castaman, MT. Álvarez‐Román, Rafael Parra López, Johannes Oldenburg, T. Albert, U. Scholz, Mats Holmström, Jean‐François Schved, Marc Trossaërt, Cédric Hermans, Ana Boban, Christopher A. Ludlam, Stefan Lethagen

Bibliographic record

VenueHaemophilia · 2016
Typearticle
Languageen
FieldMedicine
TopicHemophilia Treatment and Research
Canadian institutionsSt. Thomas Hospital
FundersSwedish Orphan Biovitrum
KeywordsMedicineHaemophiliaHaemophilia BHaemophilia ABleedPediatricsRetrospective cohort studySurgeryInternal medicine

Abstract

fetched live from OpenAlex

Introduction Haemophilia treatment varies significantly between individuals, countries and regions and details of bleed rates, factor consumption and injection frequency are often not available. Aim To provide an overview of the FVIII / FIX treatment practice and outcome for patients with haemophilia A ( HA ) or haemophilia B ( HB ) across Europe. Methods Non‐interventional, 12‐month retrospective study where anonymized data were retrieved from haemophilia centres/registers in Belgium, France, Germany, Italy, Spain, Sweden and the United Kingdom. Male patients (all ages) receiving coagulation factor treatment 24 months prior to the study, with basal FVIII / FIX levels ≤5 IU dL −1 , without inhibitors, were included. Data were summarized descriptively. Results In total, 1346 patients with HA and 312 with HB were included in the analysis; 75% and 57% had severe disease ( FVIII / FIX < 1 IU dL −1 ) respectively. Prophylaxis was most common for severe haemophilia, especially for children, whereas on‐demand treatment was more common for moderate haemophilia in most countries. The mean ( SD ) prescribed prophylactic treatment ranged from 67.9 (30.4) to 108.4 (78.1) ( HA ) and 32.3 (10.2) to 97.7 (32.1) ( HB ) IU kg −1 per week, across countries. Most patients on prophylaxis were treated ≥3 times/week ( HA ) or two times/week ( HB ). The median annual bleeding rate ( ABR ) for patients on prophylaxis ranged from 1.0 to 4.0 for severe HA , and from 1.0 to 6.0 for severe HB , while those with moderate haemophilia generally had slightly higher ABR s. Median ABR s for on‐demand‐treated severe HA ranged from 4.5 to 18.0, and for HB , 1.5 to 14.0. Conclusion Treatment practice varied greatly between centres and countries and patients treated on‐demand and prophylactically both experienced bleeds, emphasizing the need for further optimization of care.

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)0.000
metaresearch head score (Gemma)0.000
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 score0.047
Threshold uncertainty score0.882

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
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)0.0010.001

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 head0.052
GPT teacher head0.325
Teacher spread0.273 · 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

Citations77
Published2016
Admission routes1
Has abstractyes

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