Evidence map›Paper›PMID 20583220›Full record

ArticlePediatric pulmonology2010

Evaluation and management of pulmonary disease in ataxia-telangiectasia.

Sharon A McGrath-Morrow, W Adam Gower, Cynthia Rothblum-Oviatt, Alan S Brody, Claire Langston, Leland L Fan, Maureen A Lefton-Greif, Thomas O Crawford, Michelle Troche, John T Sandlund and 5 more

2 registry-linked trialsAbstract read
In one paragraph

Article in Pediatric pulmonology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 2 registered trials, which are not on this map. Cited by 36 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
36citing papers in PubMed, 1 pooled it
–field-weighted citation impact
1 · What the graph read from it

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.

2 · The registry

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.

NCT02345135 nacompletednot on this mapstarted 2012, after this paper: background citation

Susceptibility to Infections in Patients With Ataxia Telangiectasia : A Prospective Follow-up Study

TypeinterventionalSponsorJohann Wolfgang Goethe University HospitalRan2012 to 2016Enrolled41ConditionsAtaxia Telangiectasia, Infections, Immunodeficiency, Lung DiseaseArmsBlood collection, Lung function measurement, Symptom diary
NCT05692622 naunknown statusnot on this mapstarted 2023, after this paper: background citation

Exploring Effectiveness, Feasibility, and Acceptability of a Novel Home-based Complex Intervention for Children With Ataxia Telangiectasia: a Pilot Randomised Controlled Trial

TypeinterventionalSponsorUniversity of PlymouthRan2023 to 2024Enrolled40ConditionsAtaxia Telangiectasia in Children, Ataxia TelangiectasiaArmsWhole-body exercise and respiratory exercise
3 · Its place in the literature

Who cites it

36 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. ERS statement on the multidisciplinary respiratory management of ataxia telangiectasia.European respiratory review : an official journal of the European Respiratory Society · 2015
    Guideline
  2. Review
  3. MRI and Biomarkers in Early Detection of Pulmonary Changes in Ataxia-Telangiectasia.Medical science monitor : international medical journal of experimental and clinical research · 2025
    Article
  4. Review
  5. Review
  6. Article
  7. Observational
  8. Article
  9. Article
  10. Article
  11. Article
  12. Article
  13. Article
  14. Review
  15. Review
  16. Article
  17. Practical approach to respiratory emergencies in neurological diseases.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2020
    Review
  18. Article
  19. Chromosome instability syndromes.Nature reviews. Disease primers · 2019
    Review
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

15 authors.

Sharon A McGrath-MorrowDivision of Pediatric Pulmonary, Department of Pediatrics, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21287-2533, USA. smcgrath@jhmi.edu
W Adam Gower
Cynthia Rothblum-Oviatt
Alan S Brody
Claire Langston
Leland L Fan
Maureen A Lefton-Greif
Thomas O Crawford
Michelle Troche
John T Sandlund
Paul G Auwaerter
Blaine Easley
Gerald M Loughlin
John L Carroll
Howard M Lederman

Funding

ZINC AND CA EDTA THERAPY OF PLUMBISMM01RR000052 · NCRR · JOHNS HOPKINS UNIVERSITY · PI BELCHER, HAROLYN MILLICENT EDITH · 1985 to 2007
$37.3M
NCRR NIH HHS M01 RR000052
6 · The paper itself

Abstract

summaryAtaxia-telangiectasia (A-T) is a rare autosomal recessive disorder caused by mutations in the ATM gene, resulting in faulty repair of breakages in double-stranded DNA. The clinical phenotype is complex and is characterized by neurologic abnormalities, immunodeficiencies, susceptibility to malignancies, recurrent sinopulmonary infections, and cutaneous abnormalities. Lung disease is common in patients with A-T and often progresses with age and neurological decline. Diseases of the respiratory system cause significant morbidity and are a frequent cause of death in the A-T population. Lung disease in this population is thought to exhibit features of one or more of the following phenotypes: recurrent sinopulmonary infections with bronchiectasis, interstitial lung disease, and lung disease associated with neurological abnormalities. Here, we review available evidence and present expert opinion on the diagnosis, evaluation, and management of lung disease in A-T, as discussed in a recent multidisciplinary workshop. Although more data are emerging on this unique population, many recommendations are made based on similarities to other more well-studied diseases. Gaps in current knowledge and areas for future research in the field of pulmonary disease in A-T are also outlined.

Indexed as

Ataxia TelangiectasiaBronchiectasisDeglutition DisordersHumansLung Diseases, InterstitialRespiratory Function Tests

Identifiers

PMID20583220
PMCPMC4151879

What OpenQuestion holds

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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.