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Record W4414305430 · doi:10.1016/j.hctj.2025.100120

Transition services in 22q11 deletion syndrome: Hit or miss

2025· article· en· W4414305430 on OpenAlexafffundabout
Sophie Ayoub, Sandra Meier, Cheryl Cytrynbaum, Ann Swillen, Luzius A. Steiner, Holly Carbyn, Bernice S. Elger, Eva De Clercq

Bibliographic record

VenueHealth Care Transitions · 2025
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicCongenital heart defects research
Canadian institutionsUniversity of TorontoHospital for Sick ChildrenIzaak Walton Killam Health CentreDalhousie University
FundersCanadian Institutes of Health ResearchCanada Research ChairsSchweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung
KeywordsTransition (genetics)Transitional careDeletion syndromeLongitudinal dataMEDLINE

Abstract

fetched live from OpenAlex

Introduction: 22q11 deletion syndrome (22q11DS) is a rare genetic condition and the most common microdeletion in humans. The symptomatology is broad and variable between individuals. It is usually diagnosed in childhood and requires a multidisciplinary lifelong approach including a structured transitional care process. This study therefore seeks to enrich our understanding of transitional care for individuals with 22q11DS by exploring the insights from healthcare professionals (HCPs) from Europe and Canada with multidisciplinary backgrounds. Methods: This interview study was part of a larger mixed-methods research project, with the qualitative component concentrating on gathering insights from HCPs involved in patient care through semi-structured interviews. We conducted qualitative thematic analysis while focusing on transitional care within families caring for a child with 22q11DS from the perspective of HCPs. Results: The 20 HCPs interviewed came from diverse professional backgrounds and all had clinical experience with 22q11DS. Our analysis of the data identified three primary themes. Our participants emphasized the inadequacy of the transitional care system, despite its critical importance. HCPs tried their best to overcome the challenges by relying on longer follow-up with their patients, with some even merging children and adult services. There was a shared desire among HCPs for a structured transitional care plan, better access to adult services for 22q11DS, and a more integrated, collaborative approach. Conclusion: This study highlights the inadequacy of transitional care for individuals with 22q11DS and their families, showing a need to better align policies and clinical practice. Filling the gap between existing policies and the desired transitional care services is essential. The latter includes: an appropriate longitudinal transition plan, strong psychological support not only for the individual with 22q11DS but also for their family, a coordination system, and accessible adult services with expertise on 22q11DS.

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: Not applicable · Consensus signal: none
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.473
Threshold uncertainty score0.754

Codex and Gemma teacher scores by category

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

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 head0.010
GPT teacher head0.326
Teacher spread0.317 · 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 designNot applicable
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

Citations0
Published2025
Admission routes3
Has abstractyes

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