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Record W4240218095 · doi:10.1097/cpm.0b013e31818cbafb

Lymphangioleiomyomatosis

2008· article· en· W4240218095 on OpenAlexaff
Cory Yamashita, David A. Lynch, Gregory P. Downey

Bibliographic record

VenueClinical Pulmonary Medicine · 2008
Typearticle
Languageen
FieldMedicine
TopicTuberous Sclerosis Complex Research
Canadian institutionsWestern University
Fundersnot available
KeywordsLymphangioleiomyomatosisTuberous sclerosisMedicineTSC1TSC2PathogenesisProgressive diseaseDiseasePathologySirolimusBioinformaticsCancer researchInternal medicinePI3K/AKT/mTOR pathwayBiologySignal transductionGenetics

Abstract

fetched live from OpenAlex

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 distilled prediction

Teacher imitation

Not 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.

metaresearch head score (Codex)0.001
metaresearch head score (Gemma)0.001
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesInsufficient payload (model declined to judge)
Consensus categoriesInsufficient payload (model declined to judge)
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.111
Threshold uncertainty score1.000

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0010.001
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
Bibliometrics0.0000.001
Science and technology studies0.0000.002
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0020.001

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.

Opus teacher head0.297
GPT teacher head0.441
Teacher spread0.144 · 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; both teacher heads agree on what is shown here.

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

Citations2
Published2008
Admission routes1
Has abstractyes

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