ArticleChest2011
Natural history of pulmonary fibrosis in two subjects with the same telomerase mutation.
Article in Chest, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00071045 (Collection of Blood, Bone Marrow, Urine, and/or Tissue Samples From Patients With Solid Tumors, Hematological Malignancies or Non-Malignant Hematologic Disorders or HLA Compatible Family Members), which is not on this map. Cited by 26 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Collection of Blood, Bone Marrow, Urine, and/or Tissue Samples From Patients With Solid Tumors, Hematological Malignancies or Non-Malignant Hematologic Disorders or HLA Compatible Family Members
Who cites it
26 citing papers in PubMed, 45 citations in OpenAlex.
- Hematological complications in solid organ transplant recipients with telomere biology disorders: a narrative review.Frontiers in immunology · 2025Review
- The Plastic Interplay between Lung Regeneration Phenomena and Fibrotic Evolution: Current Challenges and Novel Therapeutic Perspectives.International journal of molecular sciences · 2023Review
- FK506 induces lung lymphatic endothelial cell senescence and downregulates LYVE-1 expression, with associated decreased hyaluronan uptake.Molecular medicine (Cambridge, Mass.) · 2020Article
- Interstitial Lung Disease in Relatives of Patients with Pulmonary Fibrosis.American journal of respiratory and critical care medicine · 2020Article
- The Role of Telomerase and Telomeres in Interstitial Lung Diseases: From Molecules to Clinical Implications.International journal of molecular sciences · 2019Review
- Interstitial lung abnormalities: erecting fences in the path towards advanced pulmonary fibrosis.Thorax · 2019Review
- Telomeres in Interstitial Lung Disease: The Short and the Long of It.Annals of the American Thoracic Society · 2019Review
- The Immunome in Two Inherited Forms of Pulmonary Fibrosis.Frontiers in immunology · 2018Article
- Treatment of inherited bone marrow failure syndromes beyond transplantation.Hematology. American Society of Hematology. Education Program · 2017Review
- Management of suspected monogenic lung fibrosis in a specialised centre.European respiratory review : an official journal of the European Respiratory Society · 2017Review
- Review
- Relationship between survival and age in patients with idiopathic pulmonary fibrosis.Journal of thoracic disease · 2016Article
- Telomere Dysfunction Triggers Palindrome Formation Independently of Double-Strand Break Repair Mechanisms.Genetics · 2016Article
- Potential Metabolic Biomarkers to Identify Interstitial Lung Abnormalities.International journal of molecular sciences · 2016Article
- Incorporating genetics into the identification and treatment of Idiopathic Pulmonary Fibrosis.BMC medicine · 2015Article
- Article
- Extensive phenotyping of individuals at risk for familial interstitial pneumonia reveals clues to the pathogenesis of interstitial lung disease.American journal of respiratory and critical care medicine · 2015Article
- Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations.Clinical medicine insights. Circulatory, respiratory and pulmonary medicine · 2015Review
- Modeling pulmonary fibrosis by abnormal expression of telomerase/apoptosis/collagen V in experimental usual interstitial pneumonia.Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologica · 2014Article
- MUC5B promoter polymorphism and interstitial lung abnormalities.The New England journal of medicine · 2013Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
11 authors at 3 institutions in 1 country.
Funding
Abstract
unlabelledPrevious studies have identified subclinical lung disease in family members of probands with familial pulmonary fibrosis, but the natural history of preclinical pulmonary fibrosis is uncertain. The purpose of this study was to determine whether individuals with preclinical lung disease will develop pulmonary fibrosis. After a 27-year interval, two subjects with manifestations of preclinical familial pulmonary fibrosis, including asymptomatic alveolar inflammation and alveolar macrophage activation, were reevaluated for lung disease. CT scans of the chest, pulmonary function tests, and BAL were performed, and genomic DNA was analyzed for mutations in candidate genes associated with familial pulmonary fibrosis. One subject developed symptomatic familial pulmonary fibrosis and was treated with oxygen; her sister remained asymptomatic but had findings of pulmonary fibrosis on high-resolution CT scan of the chest. High concentrations of lymphocytes were found in BAL fluid from both subjects. Genetic sequencing and analyses identified a novel heterozygous mutation in telomerase reverse transcriptase (TERT, R1084P), resulting in telomerase dysfunction and short telomeres in both subjects. In familial pulmonary fibrosis, asymptomatic preclinical alveolar inflammation associated with mutation in TERT and telomerase insufficiency can progress to fibrotic lung disease over 2 to 3 decades. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT00071045; URL: www.clinicaltrials.gov.
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What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.