Abstract W MP120: Hemorrhage Rates from Brain Arteriovenous Malformations in HHT Patients
Bibliographic record
Abstract
Introduction: Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant disease caused by mutations in transforming growth factor-beta signaling genes (ENG, ALK1, or SMAD4), and is characterized by mucocutaneous telangiectasia and arteriovenous malformations (AVM) primarily in brain, lung and liver. In contrast to sporadic brain AVM, HHT brain AVMs are often multiple though small series to date have suggested that they may have a lower risk of intracranial hemorrhage (ICH). We sought to describe ICH rates and characteristics in HHT patients with brain arteriovenous malformations (HHT-BAVM). Methods: We collected data from the first 134 HHT-BAVM patients with follow-up enrolled in the BVMC HHT Project, including age, sex, race, HHT gene mutation, and ICH at diagnosis. We performed Kaplan-Meier survival analysis to estimate ICH rates after diagnosis censoring at date of first treatment, death or last follow-up, out to 15 years. Results: The majority of patients were female (57%) and Caucasian (93%). HHT gene mutation status was known in 57%, of which 74% had ENG mutations, 23% ALK1, and 3% SMAD4. The mean age at BAVM diagnosis was 30±19 years (range: 0-70), with over half of cases diagnosed based on asymptomatic screening (54%). Among symptomatic cases, 32% presented initially with ICH. During 482 person-years of follow-up, there were 5 ICH events corresponding to a rate of 1.04% per year (95% CI: 0.43 - 2.49%). ICH-free survival differed significantly by ICH presentation (P=0.003); ruptured cases had a higher rate (5.23%, 95% CI: 1.31 - 20.89%) than unruptured cases (0.51%, 95% CI: 0.13 - 2.04%). ICH-free survival did not differ by gender (P=0.285). Conclusions: HHT-BAVM patients who present with hemorrhage are at a higher risk for rehemorrhage compared to BAVMs detected presymptomatically in HHT patients. Correlation with unruptured sporadic BAVM cases will however need a larger sample and number of events in the HHT BAVM cohort. Ongoing recruitment of HHT-BAVM patients into the BVMC study will increase precision of ICH estimates and ability to examine additional risk factors such as angiographic features that may differ from sporadic BAVM.
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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 | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
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".