Bibliographic record
Abstract
In Brief Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease occurring almost exclusively in women of childbearing age. Genetic analysis has revealed common mutations in both the TSC1 and TSC2 genes that lead to both sporadic cases of LAM (S-LAM) and those associated with the tuberous sclerosis complex (TSC-LAM). S-LAM is characterized by multiple pulmonary and nonpulmonary complications including progressive airflow obstruction, recurrent pneumothoraces, chylothoraces, and renal angiomyolipomas. Despite the putative role of estrogen in the pathogenesis of this disease, antiestrogen therapies have not shown benefit in prospective clinical trials. Recent insights into the molecular pathogenesis of LAM have offered hope for newer therapies targeting specific signaling pathways that are believed to drive LAM cell proliferation. Patients should be encouraged to participate in clinical trials that will help determine the efficacy of these novel therapies. However, until these therapies are proven to be beneficial, lung transplantation remains the only viable option for patients with progressive end-stage lung disease. Lymphangioleiomyomatosis is a rare cystic lung disease resulting in progressive obstructive lung disease and recurrent pneumothoraces that affects women of childbearing age. Genetic studies have revealed that mutations in the TSC1 and TSC2 genes are responsible for lymphangioleiomyomatosis, and that this disorder may represent a type of neoplastic process involving myofibroblast-like lymphangioleiomyomatosis cells. Recent insights into the molecular pathogenesis of lymphangioleiomyomatosis have spurred the development of specific therapies that target signaling known to drive lymphangioleiomyomatosis cell proliferation.
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 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 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.016 | 0.007 |
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".