ArticlePediatric pulmonology2014
Pulmonary function in children and young adults with ataxia telangiectasia.
Article in Pediatric pulmonology, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 3 of them syntheses that pooled it.
What it found
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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.
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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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
15 citing papers in PubMed, 3 syntheses or guidelines pooled it.
- Biomarkers in Ataxia-Telangiectasia: a Systematic Review.Journal of neurology · 2025Pooled it
- Pooled it
- ERS statement on the multidisciplinary respiratory management of ataxia telangiectasia.European respiratory review : an official journal of the European Respiratory Society · 2015Guideline
- MRI and Biomarkers in Early Detection of Pulmonary Changes in Ataxia-Telangiectasia.Medical science monitor : international medical journal of experimental and clinical research · 2025Article
- My A-T pack: a qualitative study of the utility, acceptability, design, and content of a family-designed and owned information pack relevant to the lives of children and young people living with ataxia telangiectasia.Orphanet journal of rare diseases · 2025Article
- Pioglitazone as a Possible Treatment forBiomolecules · 2024Review
- An overview of proactive monitoring and management of respiratory issues in ataxia-telangiectasia in a specialist and shared care pediatric clinic.Frontiers in pediatrics · 2024Review
- Ataxia telangiectasia mutated is required for efficient proximal airway epithelial cell regeneration following influenza A virus infection.American journal of physiology. Lung cellular and molecular physiology · 2022Article
- Imaging in children with ataxia-telangiectasia-The radiologist's approach.Frontiers in pediatrics · 2022Article
- Infections and immune dysregulation in ataxia-telangiectasia children with hyper-IgM and non-hyper-IgM phenotypes: A single-center experience.Frontiers in pediatrics · 2022Article
- Chromosome instability syndromes.Nature reviews. Disease primers · 2019Review
- Oxidative stress-driven pulmonary inflammation and fibrosis in a mouse model of human ataxia-telangiectasia.Redox biology · 2018Article
- Ataxia telangiectasia: a review.Orphanet journal of rare diseases · 2016Review
- Altered mucosal immune response after acute lung injury in a murine model of Ataxia Telangiectasia.BMC pulmonary medicine · 2014Article
- Non invasive assessment of lung disease in ataxia telangiectasia by high-field magnetic resonance imaging.Journal of clinical immunology · 2013Article
Corrections and comments
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Authors and funding
8 authors.
Funding
Abstract
backgroundPulmonary disease contributes to significant morbidity and mortality in people with ataxia telangiectasia (A-T). To determine the association between age and lung function in children and young adults with A-T and to identify factors associated with decreased lung function, pulmonary function tests were performed in 100 consecutive people with A-T.
methodsChildren and adults ranging from 6 to 29 years of age and with the diagnosis of A-T were recruited, and underwent pulmonary function tests.
resultsThe mean forced vital capacity % predicted (FVC %) in the population was 56.6 ± 20.0. Males and females between 6 and 10 years of age had similar pulmonary function. Older females were found to have significantly lower FVCs % than both older males (P < 0.02) and younger females (P < 0.001). The use of supplemental gamma globulin was associated with significantly lower FVC %. A modest correlation was found between higher radiation-induced chromosomal breakage and lower FVC % in males. No significant change in FVC % was found in a subset of subjects (n = 25) who underwent pulmonary function testing on two or more occasions over an average of 2 years.
conclusionIn children and young adults with A-T, older females and people who required supplemental gamma globulin had significantly lower lung function by cross-sectional analysis. Stable lung function is possible over a 2-year period. Recognition of groups who are at higher risk for lower pulmonary function may help direct care and improve clinical outcomes in people with A-T.
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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.