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Record W4390921073 · doi:10.1016/j.jcf.2024.01.005

Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues

2024· article· en· W4390921073 on OpenAlexaff
Pierre‐Régis Burgel, Kevin W Southern, C. Addy, Alberto Battezzati, C.A. Berry, Jean‐Philippe Bouchara, Edwin J. Brokaar, Anne Brown, Pilar Azevedo, I. Durieu, Miquel B. Ekkelenkamp, F. Finlayson, Julian Forton, Johanna Gardecki, P. Hodkova, Gina Hong, J. Lowdon, Su Madge, Clémence Martin, Edward F. McKone, À. Munck, Chee Y. Ooi, Lucy Perrem, Amanda J. Piper, Andrew Prayle, Félix Ratjen, Margaret Rosenfeld, Don B. Sanders, C. Schwarz, Giovanni Taccetti, Claire Wainwright, Natalie E. West, Michael Wilschanski, Amanda Bevan, Carlo Castellani, Pavel Dřevı́nek, Silvia Gärtner, Andrea Gramegna, Elise Lammertyn, E. Landau, Barry J. Plant, Alan R Smyth, Silke van Koningsbruggen‐Rietschel, Peter G. Middleton

Bibliographic record

VenueJournal of Cystic Fibrosis · 2024
Typearticle
Languageen
FieldMedicine
TopicCystic Fibrosis Research Advances
Canadian institutionsHospital for Sick Children
FundersEuropean Cystic Fibrosis SocietyNational Institute for Health and Care Research
KeywordsCystic fibrosisMedicineIntensive care medicineFamily medicineInternal medicine

Abstract

fetched live from OpenAlex

This is the third in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family. CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey.

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)0.080
metaresearch head score (Gemma)0.150
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Not applicable · Consensus signal: Not applicable
GenreCandidate signal: Methods · Consensus signal: none
Teacher disagreement score0.080
Threshold uncertainty score0.423

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0800.150
Meta-epidemiology (narrow)0.0010.001
Meta-epidemiology (broad)0.0010.002
Bibliometrics0.0050.003
Science and technology studies0.0040.004
Scholarly communication0.0070.006
Open science0.0040.010
Research integrity0.0090.011
Insufficient payload (model declined to judge)0.0040.003

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 head0.024
GPT teacher head0.371
Teacher spread0.346 · 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 designNot applicable
Domainnot available
GenreMethods

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
Published2024
Admission routes1
Has abstractyes

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