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Record W4400912864 · doi:10.1016/j.gimo.2024.101873

ARID1B-related disorder in 87 adults: Natural history and self-sustainability

2024· article· en· W4400912864 on OpenAlex
Pleuntje J. van der Sluijs, M. Gösgens, Alexander J.M. Dingemans, P. Striano, Antonella Riva, C. Mignot, A. Faudet, Georgia Vasileiou, Michael Walther, Samantha A. Schrier Vergano, Mariëlle Alders, Fowzan S. Alkuraya, Ibrahim A. Alorainy, Hessa S. Alsaif, BM Anderlid, Ian Bache, Ingrid van Beek, Maud Blanluet, Bregje W.M. van Bon, T. Brunet, H. Brunner, Miriam Lucia Carriero, P. Charles, N. Chatron, Emanuele Coccia, C. Dubourg, Rachel K. Earl, Evan E. Eichler, L. Faivre, Nicola Foulds, Claudio Graziano, A.-M. Guerrot, Solveig Heide, Delphine Héron, Scott E. Hickey, Saskia Hopman, Anja A. Kattentidt‐Mouravieva, Jennifer Kerkhof, Jolien S. Klein Wassink‐Ruiter, Evangeline C. Kurtz‐Nelson, K. Kušíková, Malin Kvarnung, François Lecoquierre, Gloria Leszinski, L. Loberti, Pilar Magoulas, Francesca Mari, Isabelle Maystadt, Giuseppe Merla, Jeff M. Milunsky, S. Moortgat, Gaël Nicolas, Mal Leary, S. Odent, Jillian R. Ozmore, Kareesma Parbhoo, Rolph Pfundt, Maria Piccione, Anna Maria Pinto, Bernt Popp, Audrey Putoux, Heidi L. Rehm, A. Heitor Reis, Alessandra Renieri, Jill A. Rosenfeld, Michele Marta Rossi, Emanuela Salzano, P. Saugier-Veber, Marco Seri, Gianluca Severi, F.M. Sonmez, G Strobl‐Wildemann, K.E. Stuurman, Eyyüp Üçtepe, Hilde Van Esch, G. Vitetta, Bert B.A. de Vries, David B. Wahl, T. Wang, Pia Zacher, Karin R. Heitink, Fabienne G. Ropers, Duco Steenbeek, T Rybak, Gijs W.E. Santen

Why this work is in the frame

A frame that forgets how it found something cannot be audited. These are the routes that admitted this work.

affAt least one author lists a Canadian institution in the pinned OpenAlex snapshot.
fundA Canadian funder is recorded on the work.

Bibliographic record

VenueGenetics in Medicine Open · 2024
Typearticle
Languageen
FieldBiochemistry, Genetics and Molecular Biology
TopicChromatin Remodeling and Cancer
Canadian institutionsLondon Health Sciences Centre
FundersNational Human Genome Research InstituteNational Institute of Mental HealthPeking University Health Science CenterFundamental Research Funds for the Central UniversitiesNational Institutes of HealthHospices Civils de LyonRadboud Universitair Medisch CentrumUniversità degli Studi di PalermoDartmouth CollegeKaradeniz Teknik ÜniversitesiCentre National de la Recherche ScientifiqueOhio State UniversityRadboud UniversiteitPeking University's Heeren LooGentofte HospitalInstitut National de la Santé et de la Recherche MédicaleUniversità degli Studi di SienaUniversità di BolognaLeids Universitair Medisch CentrumBerlin Institute of HealthUniversitair Medisch Centrum GroningenMinistry of Education of the People's Republic of ChinaUniversiteit LeidenNational Health Commission of the People's Republic of ChinaUniversiteit MaastrichtUniversity Hospital Southampton NHS Foundation TrustUniversità degli Studi di Napoli Federico IIRégion NormandieRigshospitaletUniversity of WashingtonBroad InstituteLondon Health Sciences CentreTechnische Universität MünchenNationwide Children's HospitalUniverzita Komenského v BratislaveSchool of Medicine, Indiana UniversityMaastricht Universitair Medisch CentrumRijksuniversiteit Groningen
KeywordsSustainabilityNatural historyNatural (archaeology)PsychologyMedicineGeographyEcologyBiologyInternal medicineArchaeology

Abstract

fetched live from OpenAlex

Purpose: -related disorder have been described, which limits our understanding of the disease's natural history and our ability to counsel patients and their families. Methods: -related disorder were collected through an online questionnaire completed by clinicians and parents. Results: were included. Cognitive functioning ranged from borderline to severe intellectual disability. Patients identified through the genetic workup of their child were either mosaic or had a variant in exon 1. New clinical features identified in this population are loss of skill (16/64, 25%) and recurrent patella luxation (12/45, 32%). Self-sustainability data showed that 88% (45/51) could eat independently, and 16% (7/45) could travel alone by public transport. Facial photo analysis showed that patients' photographs taken at different ages clustered consistently, separate from matched controls. Conclusion: spectrum is broad, and as patients age, there is a significant shift in the medical aspects requiring attention. To address the changing medical needs with increasing age, we have formulated recommendations to promote timely intervention in an attempt to mitigate disease progression.

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.

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 score0.729
Threshold uncertainty score0.477

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 head0.006
GPT teacher head0.286
Teacher spread0.280 · 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