Clinical burden of hyperphagia, obesity and health outcomes in paediatric individuals with Bardet‐Biedl syndrome: A <scp>CRIBBS</scp> data analysis
Bibliographic record
Abstract
BACKGROUND: To date, research on the natural history of hyperphagia, weight gain and health outcomes in individuals with Bardet-Biedl syndrome (BBS) has been limited. Understanding these characteristics is important for disease burden management. OBJECTIVES: The international Clinical Registry Investigating BBS (CRIBBS) is a registry of individuals with BBS to investigate the severity of hyperphagia, the natural history of weight gain and the frequency of obesity-associated health outcomes in paediatric individuals with BBS. METHODS: Paediatric participants (<18 years of age) enrolled in CRIBBS were evaluated to assess weight and weight loss interventions, hyperphagia and the frequency of cardiac, endocrine/diabetic and renal health outcomes. RESULTS: Of 331 children, 81.0% had obesity at baseline and 22.7% reported use of weight loss diet or medication at baseline or later. Among participants with ≥2 weight measurements ≥2 years apart (n = 186), 17.2% had a higher weight classification from the first to the last assessment. Increasing hyperphagia severity was associated with increasing obesity. The prevalence of cardiac, diabetic, endocrine and renal health outcomes increased with obesity class. CONCLUSION: Hyperphagia and obesity are highly prevalent in children with BBS. Many children with BBS also experience adverse health outcomes. Timely diagnosis and targeted treatment of hyperphagia and obesity are needed.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot 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.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
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.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
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".