Adherence to clinical surveillance guidelines in von Hippel-Lindau (vHL) disease: Evaluating the impact of a coordinated care centre implementation in Canada.
Bibliographic record
Abstract
460 Background: VHL is a rare hereditary cancer syndrome, predisposing to the development of multiorgan tumour growth, including bilateral and recurrent clear cell renal cell carcinoma. While guidelines for the screening and management of these patients exist, adherence to guidelines is variable. The development of coordinated clinical care centres with multidisciplinary expertise in rare diseases has been demonstrated to improve outcomes. Methods: In this study, we reviewed adherence to surveillance guidelines prior to and after development of a Canadian provincial vHL clinic at our institution, British Columbia (BC) Cancer. Care in this clinic is provided both in-person and through telehealth. We sought to evaluate whether patient geographic distance from the clinic impacted time to being seen for consultation, and whether there was a change in management plan after being seen in the clinic. Results: Between June 2022, and April 2024, 57 patients were seen in the vHL clinic (median age 34, 58% female, and 42% male). Of these, 13 lived within 50km from the clinic, 7 within 50-100km, 8 within 100-500km, and 29 lived more than 500km away. We found significant improvement in adherence to nearly all surveillance guidelines after the development of the vHL Clinic (table). 10.5% of patients had never had germline confirmation of vHL and were referred for expedited testing after being seen. We did not find any difference in time from referral to consultation based on patient geographic proximity to the clinic, F(3,53)=1.10, p =0.35 nor the modality of initial consultation (t(55)=1.07, p =0.29; in person vs telehealth). Finally, after initial consultation, 87% of patients had a change in their management plans, most commonly involving referral for further subspecialty evaluation (n=41) or initiation of pharmaceutical treatment (n=24). Conclusions: Our results add to existing evidence that coordinated care centres with multidisciplinary expertise in vHL are feasible in the Canadian public healthcare system and can result in improvements in quality of care. Our clinic model has a robust multidisciplinary care team that utilizes technology to reach patients for equitable access to care. Future work will evaluate potential cost savings to the healthcare system with this model, as well as patient satisfaction with their care pre and post participation in the vHL clinic. Adherence to clinical screening guidelines for patients with vHL before and after consultation with the BC cancer provincial vHL clinic. Screening Guideline Parameter Pre connection with vHL clinic Post connection with vHL clinic Central nervous system imaging 51.8% 100%* Abdomen/Pelvis imaging 44.6% 100%* Metanephrines 8.9% 64.3%* Ophthalmologic 75.0% 85.7% Audiology 21.4% 85.7%* *Statistically significant difference ( p <0.05).
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot 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.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.012 | 0.039 |
| Meta-epidemiology (narrow) | 0.000 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.004 |
| Science and technology studies | 0.004 | 0.001 |
| Scholarly communication | 0.002 | 0.001 |
| Open science | 0.002 | 0.003 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.001 | 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 source (direct Gemma or distilled Codex), 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".