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Record W4415112400 · doi:10.1002/ajmg.a.64273

Prevalence of Dysautonomic Symptoms in <scp>CHARGE</scp> Syndrome: A Pilot Study of 25 Individuals With <scp>CHARGE</scp> Syndrome

2025· article· en· W4415112400 on OpenAlexaff
Kim Blake, J. Kaitlin Morrison, Laura Burge, Alexandra Hudson, Angela Arra, Rita Jabbour

Bibliographic record

VenueAmerican Journal of Medical Genetics Part A · 2025
Typearticle
Languageen
FieldMedicine
TopicCongenital Ear and Nasal Anomalies
Canadian institutionsCarleton UniversityUniversity of AlbertaNova Scotia Health AuthorityUniversity of OttawaDalhousie University
FundersCHARGE Syndrome Foundation
KeywordsDysautonomiaAnhidrosisCHARGE syndromePsychological interventionYoung adultBalance (ability)

Abstract

fetched live from OpenAlex

CHARGE syndrome is a heterogeneous genetic disorder with an evolving phenotype, particularly in adults. This study examined whether dysautonomic symptoms are common in adolescents and adults with CHARGE syndrome. Adolescents and adults with CHARGE syndrome completed a CHARGE-specific checklist and modified items from COMPASS-31 with the cardiovascular items from the ASP to assess dysautonomic symptoms. Participants were 25 individuals (20 female, 5 male), aged 12-33 years (mean = 21.3 years), with clinically confirmed diagnoses, 16 of whom had a CHD7 mutation. Dysautonomic symptoms were common: 50%-60% reported fatigue, heat intolerance, constipation, nausea, and headaches; 40%-50% reported postural tachycardia, tachycardia, dyspnea, weakness, exercise intolerance, and anxiety. These rates appear at a high frequency. A detailed case study of one participant is included in the supplemental material. Findings suggest dysautonomia may be prevalent in individuals with CHARGE syndrome and highlight the need for greater awareness of symptoms and triggers. Interventions such as vagal nerve stimulation may help restore autonomic balance and improve quality of life.

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)0.001
metaresearch head score (Gemma)0.002
Version: metacan-v3-hybrid-931329e0061cValidation 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 score0.004
Threshold uncertainty score0.008

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0010.002
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.000
Science and technology studies0.0010.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0010.000

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 head0.013
GPT teacher head0.271
Teacher spread0.258 · 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 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

Citations0
Published2025
Admission routes1
Has abstractyes

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Same venueAmerican Journal of Medical Genetics Part ASame topicCongenital Ear and Nasal AnomaliesFrench-language works237,207